NR 603 Week 1 APEA Predictor Exam Review Questions & Answers Updated 2023-2024, Exams of Nursing

Download NR 603 Week 1 APEA Predictor Exam Review Questions & Answers Updated 2023-2024 and more Exams Nursing in PDF only on Docsity! NR 603 Week 1 APEA Predictor Exam Review A 75-year-old man is involved in a motor vehicle accident and strikes his forehead on the windshield. He complains of neck pain and severe burning in his shoulders and arms. His physical examination reveals weakness of his upper extremities. What type of spinal cord injurydoes this patient have? A anterior cord syndrome B central cord syndrome C Brown-Séquard syndrome D complete cord transection E cauda equina syndrome ANS: B Central Cord Syndrome the central cord syndrome involves loss of motor function that is more severe in the upper extremities than in the lower extremities, and is more severe in the hands. There is typically hyperesthesia over the shoulders and arms. Anterior cord syndrome presents with paraplegia or quadriplegia, loss of lateral spinothalamic function with preservation of posterior column function. Brown-Séquard syndrome consists of weakness and loss of posterior column function on one side of the body distal to the lesion with contralateral loss of lateral spinothalamic function one to two levels below the lesion. Complete cord transection would affect motor and sensory function distal to the lesion. Cauda equina syndrome typically presents as low back pain with radiculopathy. A 37-year-old man fell from a ladder as he finished hanging the Christmas lights on his house. The right side of his head hit the alley cement, and he lost consciousness for about 1 minute; he woke up with a headache, but he had no other complaints. A few hours later, the patient is brought to the emergency room by his neighbor because of an intense headache, confusion, and left hand hemiparesis. On examination, the patient has a bruise located over the right temporal region, mydriasis, and right deviation of the right eye, papilledema, and left extensor plantar response. An emergency CT scan of the head without contrast reveals a lens-shaped hyper- density under the right temporal bone with mass effect and edema. What is the most likely diagnosis? Answer Choices 1 Epidural hematoma 2 Subdural hematoma 3 Subarachnoid hemorrhage 4 Intracerebral parenchymal hemorrhage 5 Acute meningitis ANS: 1 Epidural Hematoma Epidural hematoma most often results from a traumatic tear of the middle meningeal artery. Although a lucid interval ranging from minutes to hours followed by altered mental status and focal deficits is typical for epidural hematoma, this clinical picture is only encountered in up to 1/3 of the patients. The collection of blood between the skull and dura mater causes an evident mass effect with ophthalmic nerve palsy and the contralateral hemiparesis. Surgical evacuation of the clot via burr holes is the treatment of choice. Subdural hematoma results from a traumatic rupture of the bridging veins that connect the cerebrum to the venous sinuses within the dura. This venous hemorrhage will result in a gradual increase of the hematoma, with a progressive clinical picture over days or weeks. The CT scan will show a concave, crescent-shaped hyper-density compared to the convex, lens-shaped hyper- density in epidural hematoma. Subarachnoid hemorrhage is the result of an aneurysm rupture; the most common is the congenital berry aneurysm. The clinical picture is of a sudden, severe headache with meningeal irritation. A CT scan will show blood in the subarachnoid space, and a lumbar puncture will reveal xanthochromia CSF. Intracerebral parenchymal hemorrhage is most likely caused by hypertension complicated with Charcot-Bouchard aneurysms. The blood accumulates into the brain substance and most commonly involves the basal ganglia. Acute meningitis is not associated with trauma. Fever and signs of meningeal irritation dominate the clinical picture. Lumbar puncture, indicated if there are no focal neurological signs on clinical examination, will be the diagnostic procedure. The CT scan of the patient presented in this case is characteristic for epidural hematoma, and there is no indication for a lumbar puncture. A 31-year-old woman presents with a purpural rash covering her arms, legs, and abdomen. She also has fever, chills, nausea, abdominal tenderness, tachycardia, and generalized myalgias. Prior to the development of the rash, the patient noted that she had a headache, cough, and sore throat. Laboratory studies were positive for Gram-negative diplococci in the blood, along with thrombocytopenia and an elevation in PMNs. Urinalysis showed blood, protein, and casts. Vital signs are as follows: PB 92/66, P 96, RR 14, T 39. The patient denies any foreign travel and does not have any sick contacts. However, she does work part time as a nurse in a local hospital. Question The patient is diagnosed with Meningococcemia; she is admitted to the hospital and placed in respiratory isolation. What major course of therapy should this patient receive? Answer Choices 1 Steroids 2 Supportive care 3 Antibiotics 4 Transfusion 5 Bactericidal/permeability-increasing protein ANS:3 Antibiotics Antibiotics are the treatment of choice for meningococcemia. The preferred drug for active infection is penicillin G. For those allergic to penicillin, chloramphenicol and cephalosporins (ie, cefotaxime, cefuroxime) may be used as alternatives. Patients will also receive supportive care, but antibiotic therapy must be initiated quickly if the patient is to survive. Intensive care placement may be necessary if organ failure is imminent. Ventilatory support, inotropic support, and IV fluids are necessary in some. If adrenal insufficiency occurs, corticosteroid replacement may be considered. A central venous line helps Awareness of self and the surrounding environment or consciousness may be altered into different abnormal states of consciousness. Consciousness can shift from loss of clear thinking or confusion, usually accompanied by disorientation, to delirium, a succession of confused and unconnected ideas manifested in children as extreme mental and motor excitement, to lethargy, a profound type of slumber where movement or speech is limited, to stupor or deep sleep where arousal is achieved only by repeated vigorous stimuli, finally to coma, unresponsiveness to even painful stimuli. Non-traumatic coma is most common in infants and toddlers with another smaller peak of occurrence in adolescence. The most common cause of non-traumatic altered level of consciousness in children is infection of either the brain (encephalitis), meninges (meningitis), or both; infections account for more than 1/3 of cases. Prolonged seizures, anticonvulsive therapy, and postictal states can also lead to altered levels of consciousness. The most common metabolic cause of alteration of consciousness is diabetic ketoacidosis, which can occur at any age, but is most common in adolescence. Caused by severe insulin deficiency, hyperglycemia and ketogenesis lead initially to polyuria, polydipsia, hyperpnea, vomiting, and abdominal pain. As the process progresses, hyperosmolar dehydration and acid/base and electrolyte disturbances occur. Advanced stages alter level of consciousness and can lead to coma. Alterations of consciousness due to inborn errors of metabolism that present with electrolyte and glucose abnormalities typically present in infancy. The availability of gluconeogenic precursors or the functions of the enzymes required for production of hepatic glucose are affected. Metabolic defects causing hypoglycemia include glycogen storage disease, galactosemia, fatty acid oxidation defects, carnitine deficiency, several of the amino acidemias, hereditary fructose intolerance, and defects of other gluconeogenic enzymes. Toxic ingestion and exposure are very common in toddlers and adolescents, with a toddler's ability to explore his environment filled with often brightly colored medications and intentional ingestion by adolescents typically involving over-the-counter medications or psychotropic drugs such as antidepressants. A 44-year-old man starts to notice that his eyelids are drooping. Some time afterwards, his jaw becomes weak. He has difficulty swallowing and also experiences weakness in his limbs. He is quite embarrassed when he eats because he must use his hand to help support his jaw. His weakness gets progressively worse. Finally, he seeks medical attention. His physical examination demonstrates the weakness in his limbs; however, no sensory defects are present. A Tensilon test is done and is positive. His doctor is concerned about an associated malignancy. What is the underlying pathology of this disease? Answer Choices 1 Inhibition of acetylcholine release 2 Blockage of the sodium channels 3 Demyelination 4 Subacute combined degeneration of the spinal cord 5 Antibodies to the acetylcholine receptor ANS: 5 5 Antibodies to the acetylcholine receptor Antibodies directed towards the acetylcholine receptor at the neuromuscular junction are seen with myasthenia gravis. This man has myasthenia gravis. Ocular muscle weakness, ptosis, dysphagia, and limb weakness can all be seen with myasthenia gravis. When the initial symptom is ocular weakness, Eaton Lambert Syndrome is extremely unlikely. Eaton Lambert Syndrome tends to not involve the extra-ocular muscles or the muscles involving chewing, swallowing, or speech. The Tensilon test is used in the diagnosis of myasthenia gravis. The Tensilon test consists of the administration of edrophonium. Edrophonium is a quick acting anticholinesterase. Thymic tumors are associated with myasthenia gravis. Thymic tumors are also referred to as thymomas. Approximately 10 - 15% of patients with myasthenia gravis have an associated thymoma. The majority of patients with myasthenia gravis have hyperplasia of their thymus. Botulinum toxin inhibits acetylcholine release. The site of action is at the neuromuscular junction. Botulinum toxin is an enterotoxin produced by Clostridium botulinum. Botulism can result from incorrectly canned foods. Tetrodotoxin is a toxin produced by puffer fish. The sodium channels are blocked by tetrodotoxin. The blockage of the sodium channels interferes with the inflow of sodium. As a result, the propagation of nerve and muscle action potentials is affected. Demyelination refers to the loss of myelin around the axon. Several disorders result in demyelination. An example of a demyelinating disease is multiple sclerosis. Subacute combined degeneration of the spinal cord is also called combined systems disease. Subacute combined degeneration of the spinal cord is a neuropathy secondary to B12 deficiency. It is seen in patients with pernicious anemia, especially pernicious anemia that has been present for quite some time. Symptoms include paresthesias and a loss of proprioception. A 74-year-old man presents after his wife witnessed him grab his head in pain and fall to the floor. He has not regained consciousness. His current blood pressure is 150/96 mm Hg, and his heart rate is 65 bpm. Emergent head CT shows a subarachnoid hemorrhage. Question In addition to life saving interventions, what prescription medication will most benefit this patient at this time? Answer Choices 1 Furosemide (loop diuretic) 2 Prednisone (glucocorticoid) 3 Tranexamic acid (antifibrinolytic agent) 4 Labetolol (beta blocker) 5 Nimodipine (calcium channel blocker) ANS: 5 Nimodipine (CCB) Nimodipine, a calcium channel blocker, has been shown to improve outcomes in patients following aneurysmal SAH. The mechanism of action is thought to be prevention of ischemia. Glucocorticoids (e.g., prednisone) are often utilized in patients with SAH because they offer symptomatic relief of headache and neck pain, but they have not been proved to decrease cerebral edema. Antifibrinolytic agents (e.g., Tranexamic acid) can be utilized in patients with a diagnosed aneurysm who cannot undergo directed treatment, but they are not routinely used following aneurysmal rupture. Labetalol (a beta blocker) may be utilized to treat elevated BP, but it must be used with caution because it can also decrease cerebral perfusion. Diuretics (e.g., furosemide) have no identified role in the treatment of aneurysmal SAH. A 37-year-old woman presents to her GP surgery with a history of right-sided facial weakness and peri-auricular discomfort since she awoke this morning. She is afebrile. Question What is the most likely diagnosis? Answer Choices 1 Trigeminal neuralgia 2 Bell's Palsy 3 Multiple sclerosis 4 Myasthenia gravis 5 Primary lateral sclerosis ANS: 2 Bell's Palsy Explanation The correct answer is Bell's palsy, which is a condition typically with sudden onset that affects the facial nerve, causing unilateral facial weakness. Trigeminal neuralgia presents with sharp pain on one side of the mouth that radiates to the ipsilateral ear, eye, or nostril. Multiple sclerosis is a demyelinating disorder, causing a multitude of symptoms that typically include diplopia or blurred vision early on, then an insidious onset of progressive weakness, numbness, and/or tingling in the extremities. Myasthenia gravis commonly presents with ptosis and diplopia, as well as difficulty swallowing, fatigue, and muscle weakness. Primary lateral sclerosis is an upper motor neuron disease that causes limb weakness, stiffness, and fasciculations. A 62-year-old man presents with vision problems and difficulty swallowing. Over the last week, he has had a constellation of symptoms; they began with numbness and tingling in his feet and progressed to weakness that now affects both lower and upper extremities. Within the last day, he has started to notice difficulty swallowing and double vision. He also feels it is difficult for him to take a big breath. His past medical history is noncontributory, and he takes no medications. Exam reveals bilateral absence of patellar and ulnar reflexes. A lumbar puncture is performed to confirm the diagnosis. Intravenous glucose is necessary if the patient is found to be hypoglycemic; bedside serum glucose is mandatory in any patient that presents with a seizure. Intravenous phenytoin and an MRI of the head might also be necessary for a patient such as the one in the vignette, but would not emergently precede antibiotics. The diagnosis of bacterial meningitis rests on CSF examination performed after lumbar puncture. However, LP is deferred in patients with evidence of increased intracranial pressure, new onset seizure, cardiorespiratory compromise, or focal neurological deficits. Antibiotics should be given, and CT scan of the head should be performed. If CT scan is negative, LP can be performed. A 12-year-old girl presents with a 3-day history of progressive dysarthria, dysphagia, and weakness. The patient was well until 3 days prior to admission to the hospital; at that time, she developed the onset and subsequent gradual worsening of dysarthria. She attributed the dysarthria to a sore throat that she had had about 2 weeks earlier. 3 days prior to admission, she also had the onset of mild dysphagia; it mostly occurred with liquids. 24 hours prior to admission, she developed weakness in both upper extremities, which increased and began to involve the lower extremities. This limb weakness was neither worsened by activity nor improved by rest. She also developed tingling in her toes 24 hours prior to presentation. When she became unable to walk without assistance on the day of admission, she decided to seek medical attention and was admitted to the hospital. Past medical history is significant for measles and mumps. Because of family religious beliefs, she has not had any immunizations. She is very athletic, and frequently plays soccer with friends and siblings in the fields on her grandfather's horse farm. Physical examination reveals a well- developed, well-nourished girl. She is awake, alert, cooperative, and in no acute distress. Temperature is 98.7 F by mouth, blood pressure of 140/80 mm Hg, heart rate is 84/min and regular, and respirations are 22/min and unlabored. There are multiple scratches and abrasions in varying stages of healing over most of her extremities. Her speech is moderately dysarthric. She experiences some mild choking when she tries to drink a glass of water. She can smile weakly, but she cannot raise her eyebrows against resistance. She shows mild bilateral weakness of eye adduction. Pupillary responses are normal. There is mild to moderate upper extremity and mild lower extremity weakness, greater distally than proximally. Her motor strength is sustained over at least 30 seconds without fatigue. Her gait is ataxic, and she cannot walk without assistance. Reflexes are hypoactive to absent, and the response to plantar stimulation is downgoing bilaterally. Sensation is intact, except for mildly impaired position and vibratory sensation in both feet. A complete blood count, chemistry profile, chest X-ray, and EKG are all normal. Computed tomography of the brain, with and without contrast, is negative. A nerve conduction study reveals a moderate degree of mostly motor demyelinating peripheral neuropathy, highly suggestive of Guillain-Barre. Question What statement best describes the patient's prognosis? Answer Choices 1 With a predominantly demyelinating rather than axonal neuropathy, her prognosis is especially bad 2 Mortality is 25-30% 3 Full functional recovery is expected in 45% in several months to a year 4 Whether recovery is full or partial, relapses do not occur during the recovery phase 5 Her rapidly evolving clinical course indicates a poor prognosis ANS: 5 Her rapidly evolving clinical course indicates a poor prognosis This patient must be watched very closely for the very real possibility of respiratory failure and the need for ventilatory support (2). Mortality is expected to be less than 5% with good medical support (1). With a demyelinating pattern on EMG, her prognosis is better. A consistent indicator of residual muscle weakness is an EMG pattern of axonal damage, with the more severe degrees of damage suggesting the worse prognosis (1). About 85% of patients with GBS have a full functional recovery within a year; however, some may be left with minor residuals such as areflexia on exam (2). Between 5-10% of patients with Guillain-Barre have 1 or more relapses; these cases are referred to as chronic inflammatory demyelinating peripheral neuropathy (CIDP) (1). A 45-year-old African-American man with no significant past medical history presents with a 1- hour history of left retroorbital headache. The headache was of a sudden onset and began upon waking that morning. It is described as excruciating, stabbing, sharp, and lancinating; it is rated as severe in intensity. He denies any preceding infections, nausea, vomiting, photophobia, or osmophobia; he also denies fever, chills, stiff neck, focal weakness, numbness, tingling, vision, hearing, gait, or speech changes. He recalls a similar episode several months ago; it lasted about a week, and it dissipated without complications. His physical exam is remarkable for painful distress, lacrimation with conjunctival injection, nasal congestion, rhinorrhea, left ocular miosis, and left forehead diaphoretic flushing. Question What pharmacologic agent is the most beneficial for this patient at this time? Answer Choices 1 Sumatriptan 2 Verapamil 3 Lithium 4 Topiramate 5 Prednisone ANS: 1 Sumatriptan The correct response is sumatriptan. This patient's most likely diagnosis is most likely a cluster headache. Pharmacologic management of cluster headache may be divided into abortive/symptomatic and preventive/prophylactic strategies. Abortive agents are used to stop or reduce the severity of an acute attack, and include oxygen, triptans, ergot alkaloids, and anesthetics. Inhalation of high- flow concentrated oxygen is extremely effective for aborting attacks. 5-Hydroxytryptamine-1 (5- HT1) receptor agonists, such as triptans or ergot alkaloids with metoclopramide, are often the first line of treatment. Stimulation of 5-HT1 receptors produces a direct vasoconstrictive effect and may abort the attack. Subcutaneous injection of sumatriptan can be effective, in large part because of the rapidity of onset. Studies have indicated that intranasal administration is more effective than placebo but not as effective as injections. Prophylactic agents are used to reduce the frequency and intensity of individual headache exacerbations. Preventive and prophylactic medications include calcium channel blockers, mood stabilizers, and anticonvulsants. Verpamil is the most effective calcium channel blocker for prophylaxis. It inhibits calcium ions from entering slow channels, select voltage-sensitive areas, or vascular smooth muscle, thereby producing vasodilation and preventing the initial vasoconstrictive phase of cluster headaches. It can be combined with ergotamine or lithium. Preliminary evidence suggests that prophylactic lithium may interfere with substance P and vasoactive intestinal peptide (VIP)-induced arterial relaxation. Anticonvulsants such as Divalproex and Topiramate are preventative medications whose mechanism of action may involve regulation of central sensitization. Prednisone is very effective for aborting the cluster headache cycle or providing intermediate prophylaxis as bridging therapy between acute and prophylactic agents. It is effective for treatment that does not respond to lithium. A 48-year-old woman presents after a seizure. Prior to the seizure, she experienced confusion and disorientation preceded by nausea, vomiting, and blurred vision. Symptoms appeared after working for several hours in the garden under the sun. Her medical history is significant for the presence of schizophrenia, for which she takes chlorpromazine at bedtime. Her temperature is 41 C; BUN and creatinine are elevated; and there is neutrophilia, hemoconcentration, and lactic acidosis. You think that the event is possibly drug-related. Question What is the most likely diagnosis? Answer Choices 1 Heat cramps 2 Neuroleptic malignant syndrome 3 Heat stroke 4 Malignant hyperthermia 5 Heat exhaustion ANS:5 Heat stroke Heat disorders can be exertional and nonexertional. Both can be drug-related. Neuroleptics (e.g., phenothiazines, thioxanthenes) may impair thermoregulation due to both anticholinergic and antidopaminergics effects. Anticholinergics inhibit sweating, therefore disturbing thermoregulation during exercise or under conditions of environmental heat stress. Antidopaminergics elevate the set point of the temperature regulation center in hypothalamus. Your patient most probably suffered heat stroke. It is a life-threatening condition characterized by elevated body temperature with nausea, blurred vision, confusion, disorientation, and Methadone, a longer-acting opioid medication, can be used for chronic pain. However, for several reasons, this is not the most appropriate medication to use in CRPS. Opiates carry addiction potential and increase fall risk in the elderly. CRPS is often categorized as early (<6 months' duration) versus late (>6 months). For early CRPS, opiates should be avoided and even with late CRPS, other medications options should be utilized before initiating opiates. Probenecid is a medication which inhibits urate resorption. It is used for gout, a painful inflammatory condition which may cause acute pain, erythema and swelling in the affected joint(s). Gout tends to be more acute and affect joints (not the forearm, as with this patient). Vancomycin is an IV glycopeptide antibiotic; it is used for severe bacterial infections, including cellulitis. Cellulitis, which commonly presents with erythema, edema and tenderness in an extremity, may be considered on the differential for this patient. Cellulitis tends to occur more acutely. This patient had a normal 8 week exam with symptoms of CRPS, but no findings of cellulitis at that time. A 46-year-old man recently recovered from a bout of influenza, and he now presents due to an 8- hour history of right-sided facial paralysis. He is having trouble closing his right eye, cannot raise his right eyebrow, and cannot smile with the right side of his mouth. Question What medication(s) should this patient be started on? Answer Choices 1 Ibuprofen 800mg PO q 6 hours plus acyclovir 400mg PO 5 times daily 2 Prednisone 60mg PO daily alone 3 Prednisone 60 mg PO daily plus acyclovir 400mg PO 5 times daily 4 Acyclovir 400mg PO 5 times daily alone 5 Prednisone 60mg PO daily plus augmentin 500mg PO 2 times daily ANS: 3 3 Prednisone 60 mg PO daily plus acyclovir 400mg PO 5 times daily The correct answer is prednisone 60mg PO daily plus acyclovir 400mg PO 5 times daily. Either medication alone can be used with fairly good results, but the most recent research has shown that patients treated within 3 days of onset with both a glucocorticoid and an antiviral have a better outcome than patients treated with either prednisone or acyclovir alone. Ibuprofen 800mg PO q 6 hours plus acyclovir 400mg PO 5 times daily is not the correct answer. Acyclovir is an appropriate choice for treatment, as it is an antiviral, and herpes simplex virus is the likely etiologic agent in the pathogenesis of Bell's palsy. However, a nonsteroidal anti- inflammatory is not the correct type of anti-inflammatory to treat Bell's palsy, so ibuprofen is an incorrect choice. Prednisone 60mg PO daily alone is not the correct answer. Prednisone is an appropriate treatment choice for Bell's palsy. It is typically given 1mg/kg for 5-7 days and then tapered over the next 5-7 days. This appears to shorten the duration of symptoms and improve the functional outcome. It should be started as early as possible, but not at all if it has been 7 days or more since the onset of symptoms. However, outcomes are even better if this treatment is used in conjunction with an antiviral, such as acyclovir. Acyclovir 400mg PO 5 times daily alone is not the correct answer. Acyclovir 400mg PO 5 times per day for 10 days is a good treatment option for Bell's palsy. It is best if started within 72 hours of symptoms, but can be started up to 7 days after the onset. However, outcomes are even better if this treatment is used in conjunction with a glucocorticoid, such as prednisone. Prednisone 60mg PO daily plus augmentin 500mg PO 2 times daily is not the correct answer. Prednisone is an appropriate treatment choice for Bell's palsy. It is typically given 1mg/kg for 5-7 days and then tapered over the next 5-7 days. This appears to shorten the duration of symptoms and improve the functional outcome. However, because it is an antibiotic used to treat bacterial infections, augmentin would not be used to treat Bell's palsy. Bell's palsy is caused by a virus, not a bacterium; therefore, an antibiotic would not help the outcome. A 4-month-old febrile infant presents with loss of appetite, irritability, seizures, focal sensory and motor deficits, and an acute petechial rash. On physical examination, a bulging fontanelle is noted; rectal temperature is 102.8°F. What study would be most important in this child's evaluation? Answer Choices 1 CBC 2 Urinalysis 3 Chemistry panel 4 Serum glucose 5 CSF analysis ANS: 5 CSF analysis The clinical picture is suggestive of meningitis, which is diagnosed after obtaining and evaluating the cerebrospinal fluid (CSF). Although a bulging fontanelle is an indication of increased intracranial pressure (ICP), it is not an automatic contraindication for a lumbar puncture (LP) unless the patient displays additional signs of increased ICP. Tonic seizures would be an example of an indication of increased ICP. Even though this child has had seizures, an LP to run a CSF evaluation is still the best answer. This will be the most important study and diagnostic tool for this child. It is likely that a CT or MRI would be done to look at the level of swelling in the brain, and possibly determine the level of ICP. It would be likely for either of these tests to be done prior to a lumbar puncture. Normally, meningitis causes fever, inactivity, and mental status changes; however, these symptoms are often hard to detect in young children. In infants, signs and symptoms may include appearing to be slow or inactive (lack of alertness), irritable, vomiting, or feeding poorly. When CSF infections are suspected, blood should be obtained for a CBC, general chemistry panel, and culture. However, obtaining CSF analysis is the most important. A 46-year-old man presents with severe insomnia and anxiety. While hospitalized, an overnight polysomnogram was performed over 2 consecutive nights. Sleep latency was 60 minutes; REM latency was 45 minutes. He reports feeling paresthesias deep within his legs while lying in bed, especially while falling asleep. He denies recent illness or illicit drug use. His physical exam and lab work were within normal limits. Question What is the most likely cause of the patient's symptoms? Answer Choices 1 Deep vein thrombosis 2 Peripheral neuropathy 3 Restless legs syndrome 4 Alcoholic peripheral neuropathy 5 Periodic limb movements in sleep ANS:3 Restless leg syndrome Restless legs syndrome (RLS) is a neurological disorder with symptoms of an unpleasant sensations in the legs, such as insects crawling inside the legs, burning, tugging, or creeping. There is an uncontrollable urge to move the limb when at rest (lying down and trying to relax activates the symptoms). Most people with RLS have difficulty falling asleep and staying asleep. Women may be slightly more affected than men. Symptoms may begin at any stage of life, although the disorder is more common with increasing age. The severity of the disorder appears to increase with age. In some patients, symptoms will improve over a period of weeks or months. In most cases, the cause of RLS is idiopathic. A family history of the condition is seen in many cases, suggesting a genetic component. People with familial RLS tend to be younger when symptoms start, and they have a slower progression of the condition. Hypoglycemia can worsen the condition. The disorder is diagnosed clinically by evaluating the patient's history and symptoms. Needle electromyography and nerve conduction studies should be considered if polyneuropathy is suspected on clinical grounds, even if results of neurologic examination are apparently normal. Ropinirole is approved for the treatment of moderate-to-severe RLS. Benzodiazepines may be prescribed for patients who have mild or intermittent symptoms. These drugs help patients obtain a more restful sleep, but they do not fully alleviate the symptoms and can cause daytime drowsiness. For more severe symptoms, opioids may be prescribed since they can cause relaxation and decrease pain. Anticonvulsants, such as carbamazepine and gabapentin, are also useful for some patients; they can decrease the creeping and crawling sensations. There may be no symptoms associated with deep vein thrombosis (DVT), but the classical symptoms of DVT include pain, swelling, redness of the leg, and dilation of the surface veins. Peripheral neuropathy, of which alcoholic peripheral neuropathy is a type, causes tingling or burning pain in the feet. At times, it may be so severe that it interferes with walking, which is a result of injury to sensory fibers. As the condition worsens, the pain typically decreases and numbness increases. Periodic limb movements in sleep are associated with periodic episodes of highly repetitive limb movements during sleep. These repetitive episodes of muscle contractions are usually grouped into series. A 32-year-old man presents with a severe headache; he has had 2 similar headaches within the past week. He describes a burning, 'hot poker'-type of pain located primarily behind his right eye. headaches. Other clues to a post-concussion headache may be memory problems, "fogginess," and nausea, and/or vomiting. The eye symptoms and signs this patient displayed are not consistent with a prior head injury. Periodic episodes of aphasia and gait disturbances would be concerning for a severe disorder, ranging from vascular, infectious, neurodegenerative, and neoplastic in etiology. Patients with cluster headaches would not be expected to have these types of neurologic symptoms. Recent exposure to outdoor pollens would be expected in a patient experiencing allergy-related headaches. A patient with allergy-related headaches would be expected to have much less intense severity and bilateral eye and nasal symptoms. 2 to 3-day duration of each headache can be seen with tension-type headaches and migraines. However, cluster headaches are noted to last 15-180 minutes of duration. A 66-year-old English teacher is in the hospital. Her neurologist gives her a magazine to read, and she is unable to do so. What is this phenomenon called? Answer Choices 1 Paraphasia 2 Echolalia 3 Alexia 4 Apraxia 5 Agraphia ANS:3 Alexia When a patient is able to hear things and repeat them, it is called echolalia. With echolalia, the patient does not understand what he has heard. This is also referred to as echophrasia. Paraphasia is a type of aphasia. The substitution of a similar sounding word for another word is called paraphasia. With paraphasia, the words can also be jumbled. Alexia is a type of aphasia. An aphasia where there is a problem with reading is called alexia. Alexia is word blindness or text blindness. Alexia is also called optical aphasia or visual aphasia. Apraxia refers to the condition where a patient has difficulty performing motor acts, despite having the muscular capacity and coordination to do so. A patient with apraxia cannot execute the intended movement. A writing disturbance is called agraphia. There are various forms of agraphia. With absolute agraphia, even simple letters cannot be written. This is also referred to as literal agraphia. A 35-year-old man presents with unusual sensations in his legs. He states that his symptoms began in college and were mild, but they have been steadily worsening over the last 4 years. His symptoms begin whenever he is lying down attempting to go to sleep. He describes the sensation as an uncomfortable crawly sensation deep within his legs. If he tries to ignore them, the feeling worsens. He has brief relief with movement, but the feelings return as soon as he is still again. He was given a trial of a benzodiazepine to take at bedtime, but it did not really help. In addition, he complains of being sleepy during the day as a result of the medication. He also tried leg massages and exercise without much relief. His past medical history is significant for depression and anxiety. The patient denies snoring, vivid dreams, or cataplexy. He states that he has a depressed mood and reduced energy and concentration, but he denies suicidal ideation. His physical exam is normal. Complete blood cell count and chemistry panels are normal. Question What is true regarding the patient's condition? Answer Choices 1 Men are affected more than women 2 Opioids are the drug of choice for this condition 3 The condition is caused by a deficiency in calcium 4 Symptoms usually resolve when underlying depression is successfully treated 5 An abnormal electrical impulse conduction study is frequently seen ANS:5 5 An abnormal electrical impulse conduction study is frequently seen An abnormal electrical impulse conduction study is frequently seen in restless legs syndrome (RLS), which is a neurological disorder characterized by unpleasant sensations in the legs and an uncontrollable urge to move when at rest in an effort to relieve these feelings. RLS sensations are often described by people as burning, creeping, tugging, or like insects crawling inside the legs. Often called paresthesias or dysesthesias, the sensations range in severity from uncomfortable to painful. Needle electromyography and nerve conduction studies should be considered if polyneuropathy is suspected on clinical grounds, even if results of the neurologic examination are apparently normal. The most distinctive aspect of the condition is that lying down and trying to relax activates the symptoms. Many patients with RLS have difficulty falling asleep and staying asleep. The disorder is diagnosed clinically by evaluating the patient's history and symptoms. Women may be slightly more affected than men. Calcium deficiency causes mainly muscle spasm and twitching, and is usually associated with other symptoms of arm and leg numbness, numbness around the mouth, nervousness, heart palpitations, and an inability to sleep, which are all not found in this case. In most cases, the cause of RLS is not known; in some cases, it may be caused by iron deficiency, folic acid deficiency, vitamin B12 deficiency, or arthritis. Sometimes, RLS can be associated with the use of antidepressant medications, necessitating the use of an alternative treatment. A 70-year-old man presents with memory loss that has been worsening over the past few months. He also has history of slurred speech and walking difficulty. There is a history of getting lost in familiar surroundings. His history is also significant for a stroke 2 years ago. On examination, there are focal neurological signs including plantar response, gait abnormalities, exaggeration of deep tendon reflexes, and weakness of the right lower limb. Brain imaging studies show multiple large-vessel infarcts and extensive periventricular white matter lesion. What is indicated by evidence of relevant cerebrovascular disease on brain imaging and by focal signs, along with temporal relationship of stroke and dementia, or abrupt cognitive deterioration and stepwise progression? Answer Choices 1 Lewy body dementia 2 Vascular Dementia 3 Dementia of the Alzheimer's Type 4 Dementia Due to Parkinson's disease 5 Delirium ANS;2 Vascular dementia Dementia is defined as a multifaceted decline in cognitive functioning that causes impaired functioning in daily life. Memory impairment is generally regarded as a necessary aspect, but decline in one or more other cognitive domains (such as language, praxis, gnosis, visuoconstructive function, frontal-executive functions) also should be demonstrated. Vascular dementia is the second most common cause of dementia after Alzheimer's. Multi-infarct dementia is the most common type of vascular dementia. The criteria for the diagnosis of vascular dementia includes evidence of relevant cerebrovascular disease on brain imaging (brain imaging must show evidence of multiple large-vessel infarcts, a single strategically placed infarct, multiple basal ganglia, white-matter lacunae's or hemorrhages, extensive periventricular white-matter lesions, or combinations of these), by focal signs along with temporal relationship of stroke and dementia, or abrupt cognitive deterioration and stepwise progression. The course must come on gradually and be accompanied by continuing cognitive decline, and there must be no evidence that the dementia is due to a physical condition. Frequently, laboratory studies, MRI, or CT scans will confirm the diagnosis. Lewy body dementia is caused by the abnormal microscopic deposition of protein in nerve cells, called Lewy bodies, which cause destruction of the nerve cells over time. These deposits can cause symptoms typical of Parkinson disease, such as tremor and muscle rigidity, as well as dementia similar to that of Alzheimer's disease. Delirium is a state of transient global disorder of cognition. White matter underlying the cortex can aid in determining the etiology of this disorder. Alzheimer's dementia is the most common type of dementia that is caused by a loss of brain cells. It is a form of degenerative brain disease resulting in progressive mental deterioration with disorientation, memory disturbance, and confusion. Parkinson's disease is a progressive neurological disorder in which there is limb stiffness, speech problems, and tremor. Dementia may develop late in the disease, but not everyone with Parkinson's disease has dementia. A 60-year-old man presents for evaluation of loss of recent and remote memory, psychomotor slowing, and difficulty with complex tasks. On exam, the patient also shows some tremor tics, dystonia, and impaired coordination. Family history reveals he had 2 brothers who both died of a strange "movement" disorder. What is the provisional diagnosis? Answer Choices Adenovirus is a rare cause of encephalitis. Adenovirus more commonly causes respiratory and eye infections. Encephalitis is a rare complication of these conditions. Influenza A is a rare cause of encephalitis. Influenza A typically causes respiratory infection, which is infrequently complicated by encephalitis. West Nile Virus is a rare cause of encephalitis. While about half of all patients who are diagnosed with West Nile Virus have meningitis and encephalitis, the overall rate of West Nile Virus is low. Epstein-Barr Virus is incorrect. Epstein-Barr virus causes infectious mononucleosis. It is estimated that only 1-5% of cases of infectious mononucleosis have nervous system involvement. A 44-year-old man presents after an episode what is described as "passing out". He has a past medical history of hypertension, diabetes mellitus type II, and osteoarthritis. Earlier in the day, the patient visited his primary care physician for routine blood work. During his blood draw, the patient had an acute onset of syncope. The patient never had such an episode before. Vital signs upon presentation are as follows: BP 124/82 mmHg, P 88 beats/min, R14/min. EKG is within normal limits. Cardiovascular examination reveals a normal S1 and S2, with no rubs, murmurs, or gallops. The patient is being treated with atenolol and hydrochlorothiazide for his hypertension. His blood sugar is 96 mg/dl. Question What is the most likely cause of the patient's syncope? Answer Choices 1 Hypoglycemic episode 2 Arrhythmia 3 Neurogenic shock 4 Vasovagal episode 5 Orthostatic hypotension ANS:4 vasovagal episode This patient is having syncope due to a vasovagal episode. This reaction is common in patients who are exposed to blood or are having their blood drawn. In fact, vasovagal episodes are the most common cause of syncope. These individuals typically present with loss of consciousness when exposed to a certain trigger. The trigger leads to an activation of the nucleus tract solitarius of the brainstem, which results in a surge of parasympathetic response, leading to the cardioinhibitory and vasodepressor effects. Although this patient has a history of diabetes mellitus, is not currently hypoglycemic. Hypoglycemia, if severe, can potentially lead to a loss of consciousness. However, diabetics who are hypoglycemic typically have a sympathetic response. Arrhythmia is not the correct answer choice. This patient does not have any cardiac history of arrhythmia. As the patient had the syncopal episode during the blood draw, and there were no ECG changes, this answer choice is unlikely. Orthostatic hypotension is a reasonable answer choice. However, this patient's acute syncopal episode in response to the blood draw makes a vasovagal reaction more likely. In addition, this patient has no history of dehydration or any other cause that would lead to this condition. A 6-year-old girl presents because her teacher is concerned about behaviors at school. The teacher has noticed the girl "staring off into space" frequently throughout the day, and the teacher is able to get her attention only occasionally. There are also periods when she appears to be talking to herself, but there is no sound coming from her mouth. The girl's mother states that there are times when the patient does not seem to be paying attention to what the mother is saying. Shortly after these "episodes", the child engages in conversation without any problem; therefore, the mother did not think the episodes were an issue. There is no concern about other abnormal behavior or discipline issues at home or at school. Question What is the most likely diagnosis? Answer Choices 1 Attention-deficit disorder (ADD) 2 Syncopal episodes 3 Petit mal (absence seizures) 4 Narcolepsy 5 Childhood disintegration disorder (CDD) ANS:3 Petit mal (absence) seizures The patient is probably experiencing petit mal (absence) seizures. Absence seizures are a form of generalized seizures seen in children, and they usually cease by age 20. They are characterized by abrupt onset of impaired consciousness and can be associated with enuresis or automatisms. Patients often appear as though they are "staring off into space" during the seizure, and the episodes often terminate as quickly as they came. These children are often categorized as "being in their own world", leading to a delay in diagnosis or a misdiagnosis of attention deficit disorder. These types of seizures are diagnosed using an electroencephalogram (EEG), showing crusts of bilaterally synchronous and symmetric 3-Hz spike-and-wave activity. Attention-deficit disorder (ADD) is a form of Attention-deficit hyperactivity disorder (ADHD) that is characterized more by inattentiveness without the presence of restless or impulsive behavior. This is the most common emotional, cognitive, and behavioral disorder treated in children/adolescents. The diagnosis is made by careful clinical history, revealing a significant level of inattentiveness and distractibility, plus-or-minus impulsivity, and hyperactivity that is inappropriate for the developmental stage of the child. These patients often change activities frequently, have a hard time with organizational skills, and are commonly caught daydreaming. The symptoms of ADD are usually pervasive, even though they may not occur in all settings. ADD/ADHD also does not involve any impairment of consciousness. Syncopal episodes are better known as "fainting spells." These are episodes of diminished or complete loss of consciousness accompanied by flaccidity that usually occur in relation to postural change, emotional stress, instrumentation, pain, straining, cardiac arrhythmias, or other physiologic disturbance. Associated signs/symptoms include pallor, sweating, nausea, and malaise. While this could be in the differential diagnosis for our patient, the cause of the syncopal episode would still need to be determined by conducting a battery of tests. In addition, our patient is not noted to lose postural tone during her episodes; this can distinguish her presentation from true syncope. Narcolepsy is a condition that is associated with "sleep attacks" that are uncontrollable and occur in inappropriate and occasionally dangerous situations. Most of these attacks are brief; however, this is not always true. Associated symptoms can include cataplexy (a sudden loss of muscle tone), hypnagogic hallucinations (dream-like experiences while falling asleep), and sleep paralysis. Childhood disintegration disorder (CDD) is a rare syndrome on the autism spectrum. It is characterized by normal development until the age of 2 that is followed by a major loss of skills, both social and verbal. Motor skills are often preserved. The presentation is often very abrupt and very dramatic. This differs greatly from the child presented above; she has had a more gradual onset of symptoms that have almost gone "unnoticed". A 45-year-old secretary presents with a 1-month history of paresthesias on the medial aspect of her right hand. She has no neck pain or shoulder pain, but she has had some difficulty typing with the right hand. Neurological exam reveals diminished sensation to pain and light touch on the medial aspect of the palmar and dorsal surfaces of the hand to the wrist, the 5th finger, and the medial aspect of the ring finger. Mild weakness of the right abductor minimi digiti is present, but the other intrinsic hand muscles are of normal strength. The remainder of the neurological exam is normal. Question What condition does patient have? Answer Choices 1 Compressive ulnar neuropathy at the elbow 2 Compressive median neuropathy at the wrist 3 Compressive radial neuropathy at the mid-humerus 4 Compressive C8-radiculopathy 5 Compression of axillary nerve ANS:1 Compressive ulnar neuropathy at the elbow This patient has a right ulnar neuropathy, which is most likely due to nerve compression at the elbow. The abnormalities on neurologic exam are limited to weakness of the right abductor digiti minimi, which is innervated by the ulnar nerve, and a sensory disturbance in the distribution of the ulnar nerve, which includes splitting of the 4th finger. The ulnar nerve exits the brachial plexus as the terminal branch of the medial cord. The nerve then descends through the axilla and upper arm, after which it traverses the groove ("ulnar groove") between the medial epicondyle of the lower humerus and the olecranon of the ulnar bone. The nerve runs through the forearm deep to the flexor carpi ulnaris muscle; it exits the wrist through an anatomic space distinct from the carpal tunnel, which is where the median nerve is located. Ulnar nerve compression can occur in the axilla, elbow, or wrist. Occupational injury is most often located in the elbow because of the nerve's proximity to the bony surfaces; therefore, it is susceptible to external compression (e.g., in persons who lean their elbow against a hard surface). In addition, work-related repetitive flexion and extension of the elbow causes narrowing of the ulnar groove, and it results in ulnar nerve entrapment. Answer Choices 1 Traumatic Brain Injury 2 Dysthymia 3 Complex partial seizures 4 Post Concussion Syndrome 5 Subdural hemorrhage ANS:4 Post concussion syndrome This adolescent displays the characteristic features of post-concussion syndrome, which is usually diagnosed on the basis of presence of 3 out of the following 8 features after a significant injury event: 1) headache; 2) dizziness; 3) fatigue; 4) irritability; 5) insomnia; 6) concentration or 7) memory dif?culty; and 8) intolerance of stress, emotion, or alcohol. It is unlikely to be a traumatic brain injury because he had a short duration of loss of consciousness, normal head CT, and his current neurologic exam is normal. Symptoms of dysthymia or depression can seem to overlap with those of PCS, but he had no such symptoms prior to the traumatic event. Complex partial seizures are unlikely; they are usually associated with focal seizure activity in the form of tonic/tonic-clonic movements. Subdural hemorrhage is usually evident on head CT, but delayed subdural hemorrhages are rarely seen. In those situations, they are associated with abnormalities on neurologic examination. A 22-year-old man presents after being hit on the right temple by a baseball 1 hour ago. He lost consciousness for 2 minutes after the impact, but he did not seek immediate medical attention. He has no other significant medical problems and family history is unremarkable. On presentation, he is comatose, with no response to painful stimuli. There is anisocoria with fixed dilatation of the right pupil. Vital signs are as follows: BP 100 / 66 mm Hg, RR 14 rpm, HR 62 bpm, temperature 36.6 °C (97.9 F). What is the most appropriate next step to confirm the suspected diagnosis? Answer Choices 1 MRI of the brain 2 Perform a lumbar puncture 3 Burr hole craniotomy 4 Computed tomography of the brain 5 Observation only ANS:4 CT The signs and symptoms described in this clinical scenario are consistent with an acute epidural hematoma. This lesion's pathophysiology is a dural tear with laceration of an artery or, less commonly, a vein. The most commonly injured vessel is the middle meningeal artery (66% of cases), but lesions of the anterior ethmoidal artery and the dural sinuses may also occur. About 85-95% of these cases are associated with a fracture of the skull. However, there is little injury to the underlying intracranial components; because of this, the prognosis is good. Other findings, such as intracranial air or vascular malformations, may also be present. Due to the progressiveness of this pathology if not treated, immediate diagnosis and surgical intervention is required. The most commonly used method to make this diagnosis to date is a non-contrast CT of the head. Not only will this help locate the hematoma, this will also help visualize any other abnormalities that may be present, such as skull fractures. A CT scan would also enable the patient to be observed during the entirety of the procedure to ensure his stability and safety. MRI of the brain may be considered appropriate, however should not be used in any patients who are unstable. Due to the patient being comatosed with no response to painful stimuli, this would not be an acceptable choice. Burr hole craniotomy is used primarily in the treatment approach in a rapidly deteriorating patients with signs of imminent herniation. Although the patient is in a comatose state, his vitals are acceptable. Knowing if there is indeed an epidural hematoma as well as where it is located will both be accomplished via a CT of the head. A lumbar puncture would not be an acceptable choice in this patient scenario and in fact may result in detrimental results. Observation only would also most likely have devastating circumstances A 74-year-old woman presents for management of an ischemic stroke. She reports difficulty seeing objects on her right side. You perform confrontational visual field testing as part of your neurological examination, and you discover she has a right inferior homonymous quadrantanopsia. This lesion localizes to what part of the body? Answer Choices 1 Left optic tract 2 Optic chiasm 3 Left optic nerve 4 Left parietal lobe 5 Right optic tract ANS:4 Left parietal lobe Quadrantanopsia (visual loss in a quadrant of the visual field) may result from several causes, of which stroke is the most common. Accurate visual field testing can often localize the region of ischemia. An inferior homonymous quadrantanopsia localizes to the contralateral parietal lobe where the superior optic radiation fibers run. Other symptoms such as hemiplegia or hemisensory loss may also be seen. An optic tract lesion would cause a contralateral homonymous hemianopsia. An optic chiasmal lesion would cause a temporal non-homonymous hemianopsia. A left optic nerve lesion would cause unilateral, not bilateral visual field loss. Ethosuximide is a primary treatment for what type of epilepsy? Answer Choices 1 Febrile seizures 2 Absence seizures 3 Generalized tonic-clonic seizures 4 Status epilepticus 5 Simple partial seizures ANS:2 Absence seizures The term epilepsy refers to a group of central nervous system disorders. These disorders are characterized by repeated occurrence of sudden and transitory seizures, which may be of motor, sensory, autonomic, or psychic origin. Seizures are almost always accompanied by abnormal and excessive discharges in the brain. The many types of seizures are classified into 10 different categories. Partial seizures, also known as focal or local seizures, are broken down into 3 groups: simple partial seizures, complex partial seizures, and partial seizures secondarily generalized. The generalized seizures (convulsive and nonconvulsive) are grouped into absence seizures, atypical absence seizures, myoclonic seizures, clonic seizures, tonic seizures, tonic-clonic seizures, and atonic seizures. Ethosuximide and valproic acid are the primary agents for the treatment of absence seizures. These drugs are generally ineffective against generalized tonic-clonic seizures and simple partial seizures, which are best treated with carbamazepine or phenytoin. Febrile seizures, if treated at all, are best treated with phenobarbital. Status epilepticus is a life-threatening disorder that must be treated promptly. Intravenous diazepam and/or phenytoin is indicated for control of this disorder. A 33-year-old African-American woman presents with a new onset of left facial droop. The droop was present when she awoke this morning, and it has worsened since then. She reports that she had been feeling fine. She denies headaches, visual changes, and weakness or tingling in the extremities. Exam reveals an anxious, overweight woman. She is afebrile. Cranial nerve exam reveals weakness of the entire left side of the face, including the forehead. The left eye does not close entirely, and there is a widened palpebral fissure. Sensation is intact. All cranial nerves, other than VII, appear intact. Her tympanic membranes are clear, and there are no skin lesions around the face or head. The rest of her exam is unremarkable. Labs reveal normal CBC, serum electrolytes, thyroid function tests, and liver transaminases. A chest X-ray is clear. Question What is the most likely cause of this patient's facial droop? Answer Choices 1 Bell's palsy 2 Sarcoidosis 3 Ramsay-Hunt syndrome 4 Lyme disease 5 Cerebrovascular accident (CVA) ANS:1 bell's palsy This patient has a classic presentation of Bell's palsy. Bell's palsy is a sudden, unilateral facial weakness and affects about 1 out of every 60 or 70 persons in their lifetime. The etiology is unknown at this time, but herpes simplex virus type 1 has been associated with some cases. The presumed pathogenesis of the palsy is that inflammation of the facial nerve causes swelling then secondary compression, and ischemia occurs where the nerve courses through the temporal bone. Pain behind the ear may precede the paralysis by a day or 2. Hyperacusis and loss of taste may occur, if the lesion is proximal enough; lacrimation may be affected as well. Sensation is not affected, although patients may experience numbness or heaviness in the face. In some cases, there is a mild lymphocytosis of the CSF. MRI may reveal swelling and uniform enhancement of the geniculate ganglion and facial nerve. Also, in some cases, MRI may reveal a swollen facial nerve entrapped within the temporal bone. Prognosis depends upon the degree of paralysis. Approximately 80% of patients will recover within a few weeks to a month. Of those with an incomplete paralysis in the 1st week, nearly all will experience complete recovery within several months. Of those with a more complete paralysis, like this patient, nerve conduction studies and electromyography can indicate prognosis. The likelihood of completely recovery is 90% if the nerve branches in the face retain Answer Choices 1 Stat electroencephalogram (EEG) 2 Stat serum prolactin level 3 Stat creatine kinase (CK) 4 Stat referral to a comprehensive epilepsy center 5 Stat psychiatric referral ANS:1 stat EEG If an electroencephalogram can be obtained on a stat basis, it can be of immeasurable help in differentiating pseudoseizures from true seizures. During a spell suggesting generalized tonic- clonic seizure, the electroencephalogram should show generalized spike and wave activity; after the spell, it should show generalized slowing and disorganization lasting 30-90 minutes. Even if the active phase of the spell were over before the electroencephalogram, if done in this 30-90 minutes post-event time frame, it could be compared with a later electroencephalogram, which would probably be normal in case of pseudoseizure (5). If an electroencephalogram is normal during or immediately after this event, it is major evidence against epilepsy and for pseudoseizures. In view of the above, and with a history of 2 previous normal electroencephalograms, obtaining an outpatient electroencephalogram or one during subsequent admission would most likely be a waste of time and money. If a video camera were available, video recording the patient during a spell, especially during electroencephalography, may provide excellent evidence or even lead to a definitive diagnosis. A serum prolactin level is a very good test in differentiating seizure from pseudoseizure; most generalized seizures, and many complex partial seizures, result in elevated levels during this initial 30-minute period following the event (1). It should be done but the results may not be back for several days. In a study by Holtkamp et al., serum creatine kinase (CK) in psychogenic status had a mean of 39 U/L (range 16-90), but in true epileptic status the mean was 699 with a range of 57-2,625 (1). In this study, creatine kinase began to rise after a delay of 3 hours and peaked after more than 36 hours. Therefore, it will not help in an emergency room setting, but it should be done and repeated (e.g., at 12, 24, and 36 hours after the event), as it can provide good evidence later on for true seizures (levels will rise) or against seizures, and for pseudoseizures if levels do not rise or rise only minimally. Through the use of 24-hour electroencephalography and video monitoring, comprehensive epilepsy centers can be extremely valuable and even definitive in differentiating pseudoseizures from true epileptic seizures. However, they are tertiary centers for evaluation of patients who have not received definitive diagnoses or have not responded to treatment for either. Also, these centers are not that common, and referral can be prolonged and complicated. As valuable as psychiatric referral could be if the patient is having pseudoseizures, a consult at this time would not be helpful. The patient's condition would preclude any meaningful interview. Also, pseudoseizure is a diagnosis of exclusion, and a full evaluation for other causes of her spells has not yet been done. Furthermore, premature referral could negatively impact cooperation from the patient and family. Psychiatric referral should be considered only after a firm diagnosis of pseudoseizures has been made, or where it becomes apparent that behavioral input is necessary to help in this differential. Summary of objective diagnostic testing that can be done to differentiate seizures from pseudoseizures: Electroencephalograms, if available, can be very helpful during the acute phase; with tonic and/or clonic movements, one would expect to see epileptiform abnormalities. Psychomotor seizures may show in the electroencephalogram, but not necessarily. A negative electroencephalogram is strong evidence of pseudoseizures, but not proof. An encephalogram done within 90 minutes after an epileptic seizure frequently shows transient postictal changes that will not be seen after a psychogenic seizure (5). Prolactin levels, done within 30 minutes of a seizure, may be helpful since most generalized seizures, and many complex partial seizures, result in elevated levels (1). Serum creatine kinase (CK) in psychologic status had a mean of 38 U/L (range 16-90), but in true epileptic status, the mean was 699 with a range of 57-2,625. In the study cited in (1), with true epileptic seizures, creatine kinase begins to rise after a delay of 3 hours, and it peaks after more than 36 hours. EEGs, if they can be obtained on a stat basis, can be most valuable in picking up the presence or absence of epileptiform abnormalities during the spells or the presence or absence of postictal changes in the 30-90 minute period following true seizures.Video recording of the patient, if a video camera is available, can be most helpful in later efforts at diagnosis. A combination of EEG and video recording could be of major importance. At a later time, continuous video and electroencephalography (vEEG) may be arranged if necessary, and it constitutes the gold standard for diagnosis. A 21-year-old woman presents with double vision that occurs only when she looks to her right side. The double vision began when she woke up in the morning. She had an episode of left leg numbness while at summer camp 6 years ago, but it resolved over 3 days and she never told anyone. Also, 3 years ago she saw her private physician after a 7-day episode of right eye pain and visual blurring. He attributed her symptoms to an ocular migraine. There is no history of head trauma. She hasn't had any infections, fevers, or immunizations recently. She is awake, alert, and in no acute distress. Positive findings include mild pallor and atrophy of the right optic disc. Bedside visual fields and acuity are normal. Testing external ocular motion in both eyes together reveals that there is no left eye movement beyond midline when attempting to look to her right, accompanied by right eye lateral nystagmus. When the left eye is tested with the right eye closed, eye movements are full. No other motor signs are found. No skin, sensory, or hearing findings are found. A magnetic resonance imaging scan (MRI) of the head with gadolinium enhancement reveals a 2 x 3 cm lucency in the region of the right parietal white matter without swelling or enhancement. Multiple sclerosis (MS) is suspected. Question Given this history, how would this patient's condition best be subtyped? Answer Choices 1 Relapsing Remitting MS (RRMS) 2 Secondary Progressive MS (SPMS) 3 Primary Progressive MS (PPMS) 4 Progressive Relapsing MS (PRMS) 5 There is insufficient data at this time to subclassify ANS:1 Relapsing remitting MS The patient has had 3 discrete clinical exacerbations (right parietal white matter/left leg numbness, right optic nerve/ visual blurring, eye pain with residual disc pallor, and median longitudinal fasciculus/resolved diplopia), with full clinical resolution. It should be kept in mind that this is a clinical descriptive and disability rating. Therefore, residual abnormalities on exam and ancillary lab testing do not define the subtype. The patient is best subtyped at this time as Relapsing Remitting MS. Subtyping may change over time with new clinical events. This patient might later develop persistent clinical disability (e.g., severe ataxia, persistent urinary incontinence, extremity weakness, diplopia, etc.). She would then best be reclassified as Secondary Progressive MS, whether these occurred as residua of discrete relapses or as an independent progressive deterioration independent of relapses. Unlike the classic Relapsing/Remitting presentation of MS, some patients present with a progression of neurological disability with no clear relapses, per se. These are subtyped as Primary Progressive MS. Some patients may initially rate a Primary Progressive MS rating, yet later develop episodes of superimposed relapses and remissions. These patients are then reclassified as Progressive Relapsing MS. A 31-year-old man with a known generalized seizure disorder is brought to your emergency department. His friends tell you that the patient had a seizure and did not wake up. When he did not wake up after 30 minutes, his friends called 911. On examination, he is breathing and his heart is beating. He is warm, dry, and pink. His basic laboratory values are within normal limits, and the computerized axial tomography (C.A.T.) scan of his brain is unremarkable. An emergency electroencephalogram (E.E.G.) is not available. His only medications are phenytoin and phenobarbital. What is the best initial treatment in this case? Answer Choices 1 Glucose 2 Lorazepam (Ativan) 3 Phenytoin (Dilantin) 4 Phenobarbital 5 Craniotomy with burr hole placement by a neurosurgeon ANS:2 lorazepam (Ativan) According to consulting neurologist Wade S. Smith, Audio-Digest Family Practice, Vol. 49, 2001, "Consciousness is usually regained in a few minutes following a generalized seizure. When consciousness is not promptly regained following a seizure, then the diagnosis of status epilepticus becomes a reasonable consideration." When the emergency electroencephalogram (E.E.G.) is not readily available on a 24-hour basis, Professor Smith recommends that empiric pharmacotherapy for a working diagnosis of status epilepticus be provided. The first pharmacotherapy recommended for status epilepticus is lorazepam (Ativan). The second line pharmacotherapy for status epilepticus is phenytoin (Dilantin). The third line pharmacotherapy for status epilepticus is phenobarbital. frequently the event that precipitates admission. The computed tomogram is classic. The cerebrospinal fluid is typical, but it can be normal early in the clinical course. This is the most common and serious of the acute encephalitides. This is a very grave illness, and it must be recognized and treated with antiviral agents (acyclovir) as soon as possible. Acute hemorrhagic leukoencephalitis is seen mostly in children, but it can also be seen in young adults. However, it is almost always preceded by symptoms of respiratory infection of 1 - 14 days duration. These patients may also develop headache and confusion, but neck pain and signs of meningeal irritation are more common and pronounced. Anosmia and temporal lobe seizures would be less common or pronounced. In this condition, white blood cell counts as high as 20,000 - 30.000 can be an important point of differential. Cerebrospinal fluid may be identical, but pleocytosis of up to 3,000 can help to differentiate it from herpes simplex encephalitis. The most helpful information at this point in differentiating this condition is the computed tomogram and magnetic resonance imaging scan, which would be expected to show diffuse asymmetric, large, and confluent edematous lesions in the white matter, with multiple small hemorrhages in both white and gray matter. Subdural empyema is a suppurative condition between the inner surface of the dura and the outer surface of the arachnoid. Since it occurs in a preformed space, it is properly termed an empyema, although it is frequently and erroneously called subdural abscess. It most commonly follows a worsening of chronic sinusitis, in which cases it most commonly involves adolescent and young men. There is usually a history of worsening of brow or mastoid pain and nose or outer ear canal purulent discharge. Fever, malaise, and headache appear. The headache pain worsens, and then becomes generalized; vomiting is frequent. Focal neurological signs may appear, and stupor and coma may ensue. Fever, leukocytosis, and stiff neck are almost universal. Cerebrospinal fluid is rather similar to that seen in herpes simplex encephalopathy. However, computed tomography scan of the head shows ear or sinus lesions and possible bone erosion. Enhanced meninges around the pus may be seen. Magnetic resonance imaging is more likely to visualize the empyema. About 40% of cerebral abscesses are related to diseases of the paranasal sinuses, middle ear, and mastoid air cells. Other conditions include bronchiectasis, lung abscess, acute bacterial endocarditis, congenital heart disease, pulmonary arterial venous malformation (AVM), abscessed teeth, osteomyelitis, or other sites of bacterial infection. The clinical course may be as seen in this patient, but onset and progression are much more variable depending on where the abscess is and the stage of development it has reached. The main concern here would be in differentiating herpes simplex encephalopathy from temporal or inferior frontal abscess. Spinal fluid is usually quite similar, and while finding bacteria might resolve the issue, this is rarely the case. Here, contrast studies are most helpful. Computed tomography would show a central hypodensity, a surrounding ring of enhancement, and hypodensity beyond the enhancing ring. MRI T1-weighted images show capsular enhancement with central hypodensity. T2-weighted images show capsular hypodensity, and surrounding edema is obvious. A septic embolism usually produces sudden neurological deficit, with the clinical picture depending on the site of ischemia. It might be mild and brief, its history being obtained only later when the patient presents with a clinical picture of expanding intracranial lesion (abscess), or it may present as a major acute typical appearing ischemic cerebrovascular accident. If it formed a temporal or frontal abscess, magnetic resonance imaging and computed tomography would probably be definitive. They may also form mycotic aneurysms of the more distal cerebral arteries; in turn, they may rupture, producing a picture of acute subarachnoid hemorrhage with or without intracerebral hemorrhage. Elevated sedimentation rate, leukocytosis, fever, malaise, fatigue, and known heart disease should prompt a thorough search for a septic embolic source. Ruptured saccular aneurysms are not usually a problem. There should be no prodrome (preceding headache, fever, stiff neck, malaise, abnormal behavior, etc.). The patient would be expected to have sudden severe headache, nausea, vomiting, and possibly loss of consciousness. There would be no fever or leukocytosis (except for a possible stress pattern). Focal neurological deficits are the exception, at least initially. Optic disc hemorrhages may be seen. Computed tomography generally shows blood (90%) in the subarachnoid spaces, which can be dramatic, or show only a subtle shadow along the tentorium or in the Sylvian or adjacent fissures. Rarely, the bleed may extend into brain tissue, producing an intracerebral hematoma. In the 5 - 10% of cases where no blood is seen on computed tomography or magnetic resonance imaging, a lumbar puncture generally reveals red blood cells in the thousands to a million, frequently with xanthochromia after several hours. Parents of a 6-month-old infant are very worried about a severe reaction that occurred in the infant after the first dose of DTaP. The infant had a high fever (up to 105ºF), was irritable, and was crying incessantly for a few hours following the injection. Parents do not want to give any more doses of DTaP to their child. Question What adverse reaction is a contraindication for giving the next dose of DTaP in this infant? Answer Choices 1 Persistent inconsolable crying for 3 or more hours 2 Encephalopathy (coma or longer or multiple seizures) within 7 days of dose 3 Fever of more than 105º F within 48 hours of dose 4 Seizure within 3 days after dose 5 Collapse or shock like state within 48 hours of dose ANS;2 2 Encephalopathy (coma or longer or multiple seizures) within 7 days of dose If the infant develops signs of encephalopathy (coma or diminished level of consciousness, prolonged or multiple seizures not attributable to another identifiable cause, within 7 days of giving a dose of DTaP or DTP), it is a contraindication for subsequent doses of DTaP. Acute neurological illness after receiving DPT can have chronic neurological sequelae. The National Childhood Encephalopathy Study (NCES) data also are consistent with a possibility that some children with underlying brain or metabolic abnormality can have acute neurologic illness triggered by DPT, which might go to chronic nervous system dysfunction, which further affects the physical, social, behavioral, and educational outcome of children. Therefore, encephalopathy is a contraindication for subsequent doses of DTaP. If the infant has progressive or unstable neurologic disorder, including infantile spasms, uncontrolled seizures, or progressive encephalopathy, defer vaccination with DTaP or TdaP until the condition is stabilized. If the infant develops a fever of 105º or above following a dose of DTaP, it is not a contraindication for subsequent dose of DTaP. Risk and benefits of the vaccine should be carefully considered. Consider giving acetaminophen before and every 4 hours thereafter for 24 hours after giving the vaccine. Collapse or shock-like state, i.e., hypotonic hyporesponsive episode, within 48 hours after receiving a previous dose of DTP or DTaP is not a contraindication, but it is important to consider the benefits and the risks. If the benefits are believed to outweigh the risk (e.g., during an outbreak or foreign travel), give the vaccine under careful observation in a facility where anaphylaxis can be recognized and managed. Seizure within 3 days of a dose of DTaP is not a contraindication, but needs precaution and a consideration of the risks and the benefits. If the benefits outweigh the risk (e.g., during an outbreak or foreign travel), the vaccine should be given under observation. Persistent inconsolable crying lasting for 3 or more hours occurring within 48 hours of dose is not a contraindication, but it is important consider the benefits and the risks of this vaccine under the circumstances. If the benefits outweigh the risk (e.g., during an outbreak or foreign travel), give the vaccine. A 40-year-old man presents with drooping of eyelids and difficulty getting up from a sitting position. He also gives a history of impotence and frequent dryness of his mouth. On examination, he is found to have ptosis, depressed reflexes, and loss of power in the proximal muscle groups of the lower extremity. On electrodiagnostic testing, he is found to have incremental response on repetitive nerve stimulation. What is the likely diagnosis in this patient? Answer Choices 1 Neurasthenia 2 Botulism 3 Lambert Eaton syndrome 4 Progressive external ophthalmoplegia 5 Myasthenia gravis ANS:3 Lambert eaton syndrome The above history and findings are suggestive of Lambert-Eaton syndrome. Lambert-Eaton myasthenic syndrome (LEMS) is a presynaptic neuromuscular disorder that causes weakness of the muscles. LEMS is caused by antibodies directed against the calcium channel on the motor nerve terminal, leading to the decreased release of acetylcholine. 1/2 of the patients have an autoimmune form, the other half have a tumor-associated form (usually small-cell lung cancer) of the disease. Hence a thorough investigation has to be done to detect occult malignancy. Clinical features include: Weakness of proximal muscle groups initially (especially the lower limb) and later other muscles. Ptosis and diplopia. Depressed or absent reflexes. Autonomic changes such as dry mouth, impotence, etc. Worsening of symptoms in the morning and improvement with exercise, as opposed to myasthenia gravis Depending on the characteristic of the movements that are seen, it is sometimes modified and called choreoathetosis or athetotic dystonia. Athetosis can be seen with cerebral palsy and some other conditions. Ballism is a flailing, jerky movement of the limbs. It affects the proximal muscles. If the abnormal movement is on one side, it is called hemiballism. Asterixis is also called "liver flap". It is a flapping movement of the hands or arms. During the physical examination to test for asterixis, have the patient hold up his hands (dorsiflex) and spread his fingers. Asterixis is seen with metabolic encephalopathies. Dystonia is an abnormal movement where there is abnormal contraction of muscles. Dystonia can be divided into primary and secondary. Spasmodic torticollis is a form of dystonia. A colleague asks you to provide a neurosurgical consultation on a 75-year-old man. The consultation request form informs you that, "This man is having difficulty tolerating his medications as well as difficulty with his day-to-day activities". Your colleague wants to know if neurosurgery could help the patient's neurologic disorder. As a prudent provider, you plan to visit this man prior to recommending or denying definitive surgery. For what disorder may neurosurgery become a reasonable therapeutic option? Answer Choices 1 Alzheimer's 2 Parkinson's 3 Amyotrophic lateral sclerosis 4 Down's syndrome 5 Multiple sclerosis ANS:2 Parkinson's As per Svennilson E, Torvik A, Lowe R, and Leskell L, "Treatment of Parkinsonism by Stereotactic Thermo-lesions in the Pallidal Region," Acta Psychiatry Neurology Scandinavia, 35:358-377, 1960, neurosurgery was provided for patients afflicted with Parkinson's disorder (PD) 50 years ago. Providers now know that dopamine replacement, carbidopa-levodopa (Sinemet) for example, is not a cure for PD. Eskandar, EN, Cosgrove GR, and Shinobu LA, "Surgical Treatment of Parkinson's Disease," The Journal of the American Medical Association, Volume 286, Number 24, pages 3056 - 3059, December 26, 2001, endorse that thalamotomy and pallidotomy can improve tremor and rigidity in selected patients with PD, sparing patients the risk of the adverse effects of the dopamine receptors. Neurosurgery has not been reasonably considered for patients with Alzheimer's disorder, ametropic lateral sclerosis, multiple sclerosis, or Down's syndrome, all of which represent a diffuse involvement of the brain and central nervous system in contrast to PD, which represents a focal involvement of the brain and central nervous system. In addition, the reduced intellectual functioning commonly associated with Down's syndrome would prevent achieving scholastic and academic rank. A 5-year-old girl was hit by a slow moving car when she ran into the street while playing tag. She hit her head and had a brief period of loss of consciousness along with a few superficial scrapes and bruises. She was placed in cervical spine precautions and brought to the ED where cervical spine radiographs were read as negative. What transient symptoms immediately after injury suggest the presence of spinal cord injury without radiographic abnormality? Answer Choices 1 Burning hands syndrome 2 A see-saw respiratory pattern 3 Weakness in the arms greater than that in the legs 4 Lightning sensation down the spine with neck movement 5 Complete paralysis with loss of pain sensation ANS:4 4 Lightning sensation down the spine with neck movement Spinal cord injury without radiographic abnormality (SCIWORA) occurs almost exclusively among children younger than 8 years of age. Increased elasticity of the pediatric spine allows injury to the cord without obvious bony disruption. Up to 27% of these children may experience delayed onset of neurological signs. This delayed period may range from 30 minutes to 4 days, with a mean of 1.4 days. Clues to this condition may be the presence of certain transient symptoms immediately after the injury. These include, in addition to a lightning sensation down the spine associated with neck movement, paresthesia in the hands and legs or a subjective feeling of generalized weakness. The chief measures to improve outcome include injury prevention, prompt recognition through examination, search for such transient symptoms, use of MRI with electrophysiological verification, and timely bracing of SCIWORA patients. A burning sensation in the hands, fingertips, and occasionally feet after an injury, also known as burning hands syndrome, is probably due to hyperextension of the cervical cord with contusion of the spinothalamic tract. It is more commonly seen in football players. A see-saw respiratory pattern where the chest retracts and the abdomen distends may indicate paralyzed intercostal muscles due to cervical spine injury. Incomplete injuries include the central cord syndrome in which there is weakness in the arms that is greater than that in the legs, as well as variable bladder involvement. Another incomplete injury is the anterior cord syndrome that leads to complete paralysis below the level of injury, as the voluntary motor impulses carried by the corticospinal tracts are anteriorly located. There is also loss of pain sensation and /or temperature sensation. Preservation of proprioception through light touch remains intact. A 22-year-old woman presents with an 8-hour history of headache and vomiting. She resides in a college dormitory; she has no remarkable travel history, and reports eating food from a street vendor 24 hours before becoming ill. The patient is lethargic and disoriented. On examination, she is found to have a temperature of 38.5° C and a petechial rash on her legs. Gram stain of exudate from a skin lesion shows Gram-negative diplococci. A lumbar puncture is performed; the cerebral spinal fluid (CSF) reveals a leukocyte count of 5/μL consisting of primarily neutrophils. A Gram stain of CSF is negative for bacteria. What is the most likely diagnosis? Answer Choices 1 Rocky Mountain spotted fever 2 Neisseria gonorrhoeae bacteremia 3 Listeria monocytogenes meningitis 4 Meningococcemia 5 Viral meningitis ANS:4 Meningococcemia Meningococcemia may evolve rapidly, with death ensuing before the development of meningitis. The presence of Gram-negative diplococci and the clinical picture are consistent with a diagnosis of bacteremia caused by Neisseria meningitidis. While also a Gram-negative diplococcus, N. gonorrhoeae is an unlikely cause of this clinical presentation. Listeria monocytogenes, transmitted by contaminated food, is also a common cause of meningitis. This organism is not typically associated with a petechial rash; it appears as a Gram- positive coccobacillus in the CSF, where mononuclear cells predominate. Rocky-Mountain spotted fever is caused by the Gram-negative obligate intracellular pathogen Rickettsia rickettsii, which is not detectable by Gram stain. Infection with R. rickettsii results in a macropapular rash. Viral meningitis is usually accompanied by monocytes in the CSF, although neutrophils may appear early in the disease. A 45-year-old woman presents with a 1-month history of double vision, tripping, urinary incontinence, and restless legs; the symptoms are accompagnied by weakness, numbness, and tingling sensations. She denies any headaches, difficulty with speech, or memory impairment. Her physical examination does not demonstrate impairments in mental status, speech, or memory. Her muscular strength is 2+ bilaterally in the upper and lower extremities, although it is diminished from a previous exam 1 year ago. Deep tendon reflexes are also diminished compared to 1 year ago. What is the most likely diagnosis? Answer Choices 1 Multiple sclerosis 2 Vitamin E deficiency 3 Vitamin B12 deficiency 4 Friedreich's ataxia 5 Charcot-Marie-Tooth Disease ANS:1 Multiple sclerosis Multiple sclerosis is correct because this patient demonstrates most of the distinguishing characteristics; spasticity (what she describes as restless legs), diplopia, unsteadiness, numbness, tingling, and urinary urgency/hesitancy (sphincter abnormalities). Vitamin E deficiency is incorrect because its main features are ataxia, slurred speech, sensory loss, and absent deep tendon reflexes. This patient doesn't report or exhibit speech impairment. Vitamin B12 deficiency is incorrect because its main features are polyneuropathy, mental status changes, and optic neuropathy. These patients do not exhibit or report mental status issues. Friederich's ataxia is incorrect because its main features are clumsy hands, ataxia, pes cavus, and sensory disturbances. Myasthenia Gravis can be considered since the patient appears to a have a motor weakness that began in bulbar musculature then spread distally (distended). This pattern of weakness can be seen in myasthenia gravis, but it is also seen in a relatively common variant of Guillain-Barre. However, in myasthenia gravis, the weakness is more proximal than distal, which is the opposite of the more distal severity expected in neuropathies like Guillain-Barre. Myasthenic weakness tends to improve after rest and worsen with exercise, a pattern not seen in this patient. Again, sensory findings would not be expected in myasthenia; also, they would not be expected in a patient of this age. A 55-year-old man with a history of heavy alcohol use presents for treatment of an infected gash in his hand. He is not currently intoxicated. His clothes fit loosely, and he mentions that he thinks he has lost weight since becoming homeless earlier in the year. In preparation for giving him antibiotics, he receives an intravenous infusion of 5% dextrose solution. 2 hours later, he is confused and ataxic, with bilateral 6th nerve palsies (intranuclear ophthalmoplegia) and nystagmus. What is the patient most likely exhibiting? Answer Choices 1 Alcoholic ketoacidosis 2 Wernicke encephalopathy 3 Friedreich ataxia 4 Sepsis 5 Malnutrition ANS:2 Wernicke encepahlopathy Ablation or destruction of the limbic areas highlights the role of limbic areas in the processing and storage of memory. Because of the protection afforded by the redundancy of having 2 hemispheres with overlapping functions, tumors, injuries or infarctions affecting limbic areas are often silent unless they cause pressure effects or electrical excitation. Toxic or metabolic damage, which typically affects symmetrical areas, can have a dramatic impact by contrast. The most common, clinically significant example is that of thiamine deficiency, which occurs in malnutrition, especially among alcoholics. Acute thiamine deficiency produces Wernicke encephalopathy. The cardinal features of this condition are delirium with ataxia, oculomotor dysfunction (especially 6th nerve palsy- intranuclear ophthalmoplegia), and sometimes anisocoria. Because thiamine is a cofactor for the cerebral metabolism of glucose, administering glucose to someone on the verge of thiamine deficiency can precipitate acute encephalopathy. Wernicke encephalopathy is prevented or rapidly reversed by administration of thiamine. Chronic thiamine deficiency causes alcohol-induced persisting amnestic disorder (Korsakoff's syndrome). The main sites of damage are paraventricular, symmetrical lesions in the mammillary bodies, the thalamus, and the hypothalamus. Lesions may also occur in the midbrain, pons, medulla, fornix, and cerebellum. Cerebellar and ocular signs may not be present. Diagnosis depends on a careful mental status examination. Patients with Korsakoff's syndrome have relatively preserved long-term memory, are socially appropriate, use language normally, and can do simple calculations. Their profound recent memory loss seems so disproportionate to the rest of their mental status examination that observers may misdiagnosis malingering, i.e., willfully faking abnormality for personal gain. Patients with Korsakoff's syndrome will occasionally try to conceal their deficits by confabulation, making up or agreeing to untrue scenarios given by the examiner. This is not the same thing as lying, but it is often mistaken for it. The mild macrocytosis is a common incidental finding in chronic alcoholics, but it does not account for the observed behavioral changes. A 34-year-old woman, an immigrant from South America, presents with an acute onset of chest palpitations and shortness of breath. The patient also gives a 1-week history of fever, fatigue, and weakness. Echocardiography reveals that her heart is dramatically increased in size. Electrocardiogram (EKG) shows low voltage QRS with atrioventricular (AV) conduction abnormality. It is determined that she has Chagas' disease. Question What is a complication that occurs during the acute phase of this patient's condition? Answer Choices 1 Megaesophagus 2 Thromboembolism 3 Gastric dilatation 4 Meningoencephalitis 5 Megaureter ANS:4 meningoencephalitis Chagas' disease is also referred to as American trypanosomiasis. Chagas' disease is seen in the Western Hemisphere, primarily in South America, Central America, and Mexico. It is caused by the protozoa Trypanosoma cruzi. It can be acute or chronic. The manifestations in the acute phase include prolonged fever, tachycardia, fatigue, anemia, weakness, mild hepatosplenomegaly, and lymphadenopathy. Meningoencephalitis is the complication occurring during the acute phase. The acute stage resolves spontaneously by 4-8 weeks in 90% of the individuals who are infected. The persistence leads to development of intermittent or chronic phase. Chronic infection can lead to cardiomyopathy. The cardiac changes seen with chronic Chagas' disease include an enlarged heart and EKG findings of right bundle branch block and premature ventricular contractions. Chronic infection can also lead to megaesophagus, megacolon, and thromboembolism. Gastric dilatation and megaureter may occur, but they are rare. A 15-month-old toddler presents with sudden onset of generalized tonic and clonic convulsions for the last 30 minutes. There is no history of trauma, fever, vomiting, or irritability prior to the onset of convulsions. This is the first episode of seizure, and there is no history of convulsions in the family. Birth history, neonatal period, and developmental milestones are normal. Question After initial stabilization by securing the airway, oxygenation, and controlling the acute convulsion, what is the investigation of choice for the above child? Answer Choices 1 Cerebrospinal fluid examination 2 CT scan of the head 3 Electroencephalogram 4 Cranial ultrasonography 5 MRI scan of the head ANS:2 CT scan of head CT scan of the head is the best diagnostic imaging study in a previously asymptomatic child who presents with status epilepticus, particularly if there is suspicion of intracranial hemorrhage, a space-occupying lesion, or midline shift. It is a non-invasive and rapid procedure and is widely available. It has a short imaging time and requires less sedation in young children. Though CT imaging is largely replaced by MRI, it still remains the most sensitive modality to detect intracranial hemorrhage and calcifications during the first 24 hours. Other indications are head injury, hypoxic ischemic encephalopathy, inflammatory disorders like neurocysticercosis, CNS tuberculosis, basal exudates, pyogenic abscess, and malformations like hydrocephalus and porencephaly. Myelination, posterior fossa, and brain stem structures are not well visualized by CT scan. As the child presented with his 1st episode of convulsion with status epilepticus without any prior symptoms, a CT scan of the head would be the first choice of investigation. Cranial ultrasonography (USG) is mainly used in the diagnosis and follow-up of newborns with intraventricular hemorrhage and for assessment of ventricular size. It can be performed through the open anterior fontanel, and it can also be performed at the bedside of the patient. It can visualize the ventricles, periventricular tissue, and a part of cerebral cortex. As the above child is 15 months old and the anterior fontanel is either closed or is very small at this age, it cannot be performed in this patient. Electroencephalogram (EEG) is commonly used to confirm a clinically doubtful convulsion, to characterize the type of convulsion, to locate an epileptic focus, or to distinguish a simple febrile convulsion from epilepsy. It can help to distinguish between a seizure and a non-seizure state, such as a fainting spell, hypoxic episode, or a breath-holding spell. It is especially helpful in the diagnosis of absence attacks, herpes encephalitis, and myoclonic seizures. It is not the investigation of first choice in the above patient. Lumbar puncture (LP) and examination of cerebrospinal fluid (CSF) is usually indicated for the diagnosis of conditions like meningitis, encephalitis, subarachnoid hemorrhage, demyelinating illnesses, and slow virus infections of the CNS. As the above child was asymptomatic before the episode of convulsion, lumbar puncture is not the investigation of first choice in this child. MRI scan of the head can demonstrate myelination abnormalities, edema, and infarcts more clearly as compared to CT. MRI is the study of choice for evaluating the spine, spinal cord, and spinal canal in children. It is more sensitive than CT scan for most brain tumors involving the brainstem, posterior fossa, and spinal column. It is not the investigation of first choice for acute intracranial hemorrhage during the first 24 hours. Also, it is not widely available for use during emergency. A 40-year-old man presents with a 3-week history of a daily headache that awakens him from sleep. The headaches occur around the left eye. He notes that the left eye waters and his left nostril gets stuffy with each headache. What is the most likely diagnosis? Answer Choices 1 Sinusitis 2 Tension headache 3 Cluster headache 4 Intracranial mass tendon reflex is 2+ on the left and 2+ on the right. His grip strength on the right is diminished as compared to the left. The remainder of the physical exam is normal. Question Based on the above presentation, what is the cervical nerve root most likely affected? Answer Choices 1 C4 2 C5 3 C6 4 C7 5 C8 ANS:5 C8 The most likely nerve affected in this case is the C8 nerve root. Impingement of C8 may cause numbness and tingling primarily on the medial surface of the arm and into the lateral hand into the fourth and fifth digits. Impingement may also cause dysfunction of the hand, as it innervates the small hand muscles. Impingement of the C4 nerve root may cause neck and upper shoulder numbness and pain. Impingement of C5 nerve root may cause deltoid and shoulder numbness and pain, and biceps tendon reflex may be diminished. Impingement of the C6 nerve root can cause numbness and tingling down the arm into the thumb, with weakness in the bicep muscle and diminished brachioradialis tendon reflex in the affected extremity. Impingement of C7 also causes numbness and pain down the affected arm but into the middle finger, and the triceps reflex may be diminished on exam. A 32-year-old woman starts to notice that she is having difficulty brushing her hair. She also notices that her eyes are extremely tired after reading the evening newspaper. One night, her husband comments that she has droopy eyelids. She is relieved the next morning when her eyelids appear normal. Her condition deteriorates, and she has weakness and fatigability to the point that she finally sees her family doctor. On history, she denies pain. Her physical exam demonstrates weakness in her limbs. Her deep tendon reflexes are within normal limits. Her doctor wants to run a diagnostic test by injecting a drug. What is the most likely diagnosis? Answer Choices 1 Myasthenia gravis 2 Duchenne's dystrophy 3 Polymyositis 4 Familial periodic paralysis 5 Botulism ANS:1 myasthenia gravis This patient's symptoms indicate myasthenia gravis. Ocular muscle weakness and ptosis are common. Muscular weakness and fatigue are consistent with this condition. Myasthenia gravis occurs due to the presence of antibodies to the acetylcholine receptor. A diagnostic aid is the Tensilon test. The Tensilon test entails the administration of a rapid acting anticholinesterase, edrophonium. By administering an anticholinesterase, any acetylcholine released will be present longer and will have a longer period of time to act at the neuromuscular junction. Duchenne's dystrophy would present at a very young age. It is X-linked. Polymyositis can present with weakness. However, the weakness is proximal; it is usually of the hips and thigh, but sometimes it is of the shoulder girdles. Polymyositis would spare the ocular muscles. Familial periodic paralysis can present with weakness. However, the weakness would be in the extremities and the trunk. It would also spare the ocular muscles. Botulism can present with weakness. In addition, the ocular muscles can be involved. However, botulism would present with a progressive weakness, rather than an intermittent weakness. There would also be a loss of deep tendon reflexes. Furthermore, there is nothing in her history to suggest ingestion of botulism toxin. A 32-year-old man presents with a 2-day history of high fever and progressive, severe, headaches. It is associated with blurred vision and increasing confusion. The patient is normally healthy, and he does not have a remarkable past medical history. He is married. He does not drink alcohol, smoke, or use illicit drugs. He has not had any blood transfusions, and he takes no medications. On physical examination, he appears ill and disorientated to time, place, and person. His pulse is 110/minute, temperature 39 degrees Celsius (102 degrees Fahrenheit), respirations 22/minute and blood pressure is 115/70 mmHg. He is well-hydrated. He has no scleral icterus or oral candidal infection. Pupils are equal and reactive. There are no palpable adenopathy or rashes. His cranial nerve examination is intact. His fundi are normal. He can move all of his limbs. Deep tendon reflexes are normal; plantar reflexes are equivocal. His neck is supple. His lab work includes Hemoglobin = 13. 1 g/dL; hematocrit = 39%; white blood cell count (WBC) = 18x109/L with 70% polymorphonuclear neutrophils and 30% lymphocytes; platelets = 200x109/L; erythrocyte sedimentation rate = 90 mm/hour; sodium = 138 mEq/L; potassium = 4 mEq/L; chloride = 102 mEq/L; blood urea nitrogen (BUN) = 29 mg/dL; creatinine = 1mg/dL; glucose = 101mg/dL; calcium = 9. 2 mg/dL; total bilirubin = 1mg/dL; alanine transaminase (ALT) = 26 units/L; aspartate transaminase (AST) = 22 units/L; alkaline phosphatase = 112 units/L. Results of drug screening are negative and his urinalysis is normal. The lumbar puncture results are as follows: Cerebrospinal pressure Increased Protein level 180 mg/dL Glucose 92 mg/dL White blood cell count 116 per microliter (all lymphocytes) Red blood cell count 80 per microliter Gram-staining results White blood cells with no organisms Oligoclonal bands Absent IgG index Normal Question An MRI scan of the brain demonstrates low-density lesions in the brain; they represent areas of gross demyelination. What is the most likely diagnosis? Answer Choices 1 Acute multiple sclerosis 2 Central nervous system lymphoma 3 Herpes simplex virus (HSV) encephalitis 4 Multifocal leukoencephalopathy 5 Viral meningitis ANS:3 HSV encephalitis The patient has a fever, severe headache, altered mental status and has abnormal levels of lymphocytes in the CSF. These findings, along with the results of the MRI scan, suggest herpes simplex virus, encephalitis. HSV encephalitis occurs in 10% of all cases of encephalitis in the United States. There is a high mortality rate and morbidity rate. The latter is normal in the form of neurological damage. Herpes simplex type 1 is the most common cause and type 2 causes neonatal encephalitis (acquired by mother). The illness typically occurs over several days. The CSF findings in this patient typify the condition. Specifically, the CSF pressure is increased with pleocytosis. The red blood cells reflect the hemorrhagic nature of the CNS lesions. The protein level is increased; the glucose level may be normal or decreased. Tuberculosis or fungal meningitis is associated with decreased glucose levels, which can create confusion. Multiple sclerosis (MS) is a chronic CNS disease affecting young adults. There is the presence of demyelination and T-cell predominant perivascular inflammation in the brain white matter. Some axons may be spared from these pathological processes. Early symptoms may include numbness and/or paresthesia, mono- or paraparesis, double vision, optic neuritis, ataxia, and bladder control problems. Subsequent symptoms include upper motor neuron signs. Vertigo, incoordination and other cerebellar problems, depression, emotional lability, abnormalities in gait, dysarthria, fatigue and pain are also commonly seen. The presence of oligoclonal bands and/or elevated IgG index constitutes the basis for diagnosis. The most typical presentation of a primary CNS lymphoma is in an immunocompetent patient with progressive symptoms indicative of a mass lesion. Seizures may occur. Patients with AIDS are more likely to present with an encephalopathy than other patients with a primary CNS lymphoma. A careful sexual and drug abuse history is necessary. Isolated, ocular, or meningeal tumor may occur in the absence of any focal abnormalities on MRI. Multifocal leukoencephalopathy is a lethal secondary viral infection; it primarily occurs in AIDS patients with advanced immunodeficiency. The JC virus is a ubiquitous virus, able to enter the brain and lytically infect oligodendrocytes (the cells making myelin in the brain). This causes demyelination of the brain, which results in a wide variety of focal neurologic symptoms, including weakness, loss of sensation, visual loss, and changes in balance and coordination. Because it is usually relentlessly progressive, severe neurologic disability develops over a period Vascular dementia usually has a more sudden onset, and there is evidence of cerebrovascular disease with focal neurological signs and symptoms and usually a history of a previous stroke. Pseudo dementia is the term used to describe false dementia or major depression in the elderly; it usually consists of depressed mood, crying episodes, hopelessness, helplessness, and fatigue. Patients may also present with insomnia or hypersomnia. A 12-month-old girl presents with her parents after a 3-day history of intermittent episodes of strange behavior. A neurologist is consulted because the parents are concerned she is having some form of seizure activity. Her health history includes 2 episodes of otitis media, but she is otherwise healthy. Her initial vital signs and physical exam by the emergency room staff are all normal. Question What description of the infant's strange behavior would lead the neurologist to suspect a diagnosis of simple partial seizures? Answer Choices 1 Facial and right arm twitching lasting about a minute, then loss of consciousness 2 Blank stare and impaired awareness for 10-20 seconds 3 Eye deviation with facial twitching that lasts 1-2 minutes 4 Loss of consciousness, followed by stiffening of the body and extremities 5 Very brief periods of whole body going limp, followed by a brief loss of consciousness ANS:3 Correct Answer: Eye deviation with facial twitching that lasts 1-2 minutes Eye deviation with facial twitching that lasts 1-2 minutes' is the correct answer. Simple partial seizures can vary depending on what portion of the cortex has been effected. Patients may display isolated tonic or clonic activity or may have transient altered sensory perception. Clonic movements are jerks that may involve a specific body part or may spread to adjacent body parts, known as "Jacksonian march." Tonic activity is stiffening or sustained posturing, such as eye deviation in this case. Sensory seizures can involve illusions or hallucinations, but this would be difficult to diagnose in a young child. One important hallmark of a simple partial seizure is that the patient does NOT lose consciousness. 'Facial and right arm twitching lasting about a minute then loss of consciousness' is not the correct answer. While a patient having a simple partial seizure might experience facial and right arm twitching for that period of time, they are not going to have loss of consciousness. The type of seizure involved in this answer choice best describes a complex partial seizure. 'Blank stare and impaired awareness for 10-20 seconds' is not the correct answer. This description is best used to describe a patient who is having a nonconvulsive generalized seizure, otherwise known as an absence seizure. Patients having an absence seizure will rarely have any movement during the seizure. At most, they may have some minor motor activity, such as blinking. In an older child or adult, these episodes will leave the patient feeling as though they have short periods of time that they do not remember. 'Loss of consciousness followed by stiffening of the body and extremities' is not the correct answer. The fact that this description includes loss of consciousness is what excludes it from being a description of a patient having a simple partial seizure. This scenario better describes a person who is having a generalized tonic-clonic seizure. 'Very brief periods of whole body going limp followed by brief loss of consciousness' is not the correct answer. The fact that this description includes loss of consciousness is what excludes it from being a description of a patient having a simple partial seizure. This scenario better describes a person who is having an atonic seizure, which is characterized as an abrupt, brief loss of body tone, possibly followed by brief loss of consciousness. A 24-year-old man presents with head trauma received in a motor vehicle accident (MVA) 30 minutes ago. Paramedics relate loss of consciousness for 1 minute, with a complete recovery. At the initial evaluation, the physical exam reveals a patient with a Glasgow Coma Scale (GCS) of 12, right hemiparesis, and a left fixed dilated pupil. What is the most probable finding in the brain CT scan? Answer Choices 1 A left epidural hematoma 2 A right epidural hematoma 3 A left subdural hematoma 4 A right subdural hematoma 5 A subarachnoid hemorrhage ANS:2 left epidural hematoma The patient presents with a history of head trauma, a brief initial period of unconsciousness, a lucid interval lasting minutes, subsequent deterioration in neurological status revealed in a Glasgow coma scale of 12, right hemiparesis, and left fixed dilated pupil. This is a classical presentation of left epidural hematoma, which is seen as a biconvex hyper-density (whiteness) on a CT scan. A right epidural hematoma would produce a left hemiparesis and a right fixed dilated pupil. A 40-year-old man presents to the emergency department with severe pain in his left eye, decreased vision, nausea, and abdominal pain. On examination, the patient's left pupil is moderately dilated and nonreactive. The cornea is "steamy" in appearance and generally the eye is red. What do you suspect is the cause? Answer Choices 1 Conjunctivitis 2 Acute uveitis 3 Acute angle-closure glaucoma 4 Corneal ulcer 5 Corneal infection ANS:3 acute angle closure glaucoma This patient has acute angle-closure glaucoma. The typical characteristics of this condition are all exhibited by this patient (i.e., steamy cornea, severe pain, blurred vision, dilated, and nonreactive pupil). A physical exam finding in a patient with acute uveitis would be a small pupil. In addition to these symptoms, patients may also present with abdominal pain. A corneal infection and corneal ulcer would cause circumcorneal injection and watery or purulent discharge. A 48-year-old HIV positive man starts to develop headaches. At first, he attributes the headaches to stress. However, they persist and become worse over the next few weeks. He develops nausea and vomiting, and he thinks he has a fever. He starts to become confused, so he seeks medical attention. On physical examination, his temperature is 100°F. He has signs of meningeal irritation. A lumbar puncture is performed, and there is an elevation of his CSF pressure. Cerebrospinal fluid is sent to the lab. The CSF is centrifuged. A drop of India ink is placed on the slide along with a drop of the spun CSF. The India ink test is positive. Question What diagnostic sign may be found in this patient? Answer Choices 1 Kernig's sign 2 Babinski's sign 3 Kussmaul's sign 4 Quincke's sign 5 Chvostek's sign ANS:1 kerning sign Kernig's sign is a sign of meningeal irritation. The patient lies on his back, and his leg is raised and knee bent at a 90-degree angle. If there is pain or resistance to further extension when the examiner straightens the knee, Kernig's sign is said to be positive. This patient has signs and symptoms of cryptococcal meningitis. Cryptococcus neoformans is a yeast with a capsule made of polysaccharides. India ink exam is positive for Cryptococcus neoformans. When visualized on high dry magnification, encapsulated organisms (Cryptococcus neoformans) have capsules that look like halos because they exclude the India ink. Cryptococcus neoformans can cause meningitis, and there is an increased risk of Cryptococcus neoformansmeningitis in immunosuppressed individuals. Babinski's sign is an abnormal response to stimulation on the sole of the foot that results in dorsiflexion of the big toe and fanning of the other toes. Babinski's sign is seen with pyramidal disease. Kussmaul's sign is an increase in venous pressure during inspiration; it can be seen with cardiac tamponade. Quincke's sign is also called Quincke's pulse. This sign is seen in the nails. It consists of whitening and reddening of the nail bed coinciding with each heartbeat. Quincke's sign can be seen with aortic insufficiency. Chvostek's sign can be seen with tetany. When tapping on the facial nerve produces contraction on that side of the face, it is called Chvostek's sign. A 25-year-old woman is seeking medical attention because she is concerned about a genetic disease that is running in her family. Both her father and her paternal grandfather are affected. Her 50-year-old father has suffered for several years with coordination difficulties, chorea, decline in cognitive ability, and aggressive outbursts. His symptoms are getting progressively worse. This patient has a 50% chance of developing what disorder? Answer Choices 1 Creutzfeldt-Jakob disease 2 Early-onset familial Alzheimer disease 3 Hallervorden-Spatz disease 4 Huntington's disease 5 Lesch-Nyhan disease ANS:4 Huntington Disease Findings such as hydrophobia, pharyngeal spasm, and hyperactivity would be seen in a patient with rabies-induced encephalitis. A patient who has encephalitis from the varicella-zoster virus will more commonly have the dermatomal pattern of grouped vesicles that is typically seen in a zoster infection. A 66-year-old woman is brought to the emergency room by her daughter; the patient is becoming incoherent and confused. She was in good health until early that morning when she reported having a headache. She is not a known hypertensive or diabetic, and she is not on any medications. On examination, she is well-nourished, has a fever, and is disorientated to time, space, and person. Brudzinski's sign is positive. What would you expect to find on examination of her cerebrospinal fluid (CSF)? Answer Choices 1 CSF pressure 250 mm H2O, cells 2000/uL with predominant neutrophils 2 CSF pressure 150 mm H2O, cells 500/uL with predominant lymphocytes 3 CSF pressure 150 mm H2O, cells 250/uL with predominant lymphocytes 4 CSF pressure 150 mm H2O, cells 150/uL with predominant lymphocytes 5 CSF pressure 250 mm H2O, predominantly red blood cells ANS:1 CSF pressure 250 mm H2O, cells 2000/uL with predominant neutrophils The correct response is CSF pressure 250 mm H2O, cells 200/µL with predominant neutrophils. The clinical picture is suggestive of acute bacterial meningitis; this is an inflammatory disease involving the arachnoid layer of the meninges and the cerebrospinal fluid (CSF). It is a medical emergency and antibiotic treatment should be started immediately. Patients with acute bacterial meningitis usually have an increased CSF pressure, cells 500 - 5000/uL, and neutrophils are the predominant cells. The CSF glucose is decreased and the protein level is usually over 100 mg/dL. A Gram stain should be done on the CSF smear. Serologic latex agglutination tests for meningococcus, H. influenzae type B, and pneumococcal antigens in the CSF can provide a diagnosis rapidly. The CSF should be cultured. Other relevant cultures (e.g., blood, nasopharyngeal and respiratory secretions, urine, and skin lesions) should also be done. A lumbar puncture should be done to obtain CSF samples after a CT scan has excluded a mass lesion. Normal values of CSF parameters are as follows: A. CSF pressure 100 - 200 mm H2O B. Cells 0 - 3/uL C. Predominant cell type - lymphocyte D. Glucose 50 - 100 mg/dL E. Protein 20 - 45 mg/dL The following are the common bacterial causes of acute meningitis in the various age groups: Escherichia coli and Streptococci agalactiae are found in neonates and infants. Haemophilus influenzae type B, Streptococcus pneumoniae, and Meisseria meningitidis are found in early childhood. Neisseria meningitidis is found in adolescents and young adults. Meningococci exist in the nasopharynx of about 5% of the population and can spread by respiratory droplets through kissing, sneezing, or coughing. They tend to cause epidemics among closed populations (e.g., military barracks and boarding schools). Streptococcus pneumoniae is the most common cause of acute meningitis in people over 25 years of age. Especially at risk are alcoholics and persons with chronic otitis, sinusitis, pneumococcal pneumonia, sickle cell disease, and asplenia. Staphylococcus aureus is a common cause of acute meningitis in immunocompromised patients after penetrating head wounds or neurosurgical procedures. Gram-negative bacteria, such as Escherichia coli, Klebsiella, and Enterobacter, are common causes of nosocomial meningitis and meningitis after neurosurgical procedures, trauma, and genitourinary manipulation in the elderly. Listeria monocytogenes meningitis is common in patients with immunosuppression, carcinomas, and those receiving corticosteroids or cytotoxics. Patients usually present with headache, fever, chills, photophobia, vomiting, seizures, confusion, drowsiness, stupor, and coma (especially in the elderly). There may be a history of a prodromal upper respiratory tract infection, especially in patients with pneumococcal meningitis. Dehydration and vascular collapse due to adrenal insufficiency may lead to shock in Waterhouse- Friderichsen syndrome in meningococcal septicemia. Complications include cranial nerve palsies, cerebral infarction, convulsions, and mental retardation. On examination, patients may be febrile and have a stiff neck. A petechial or purpuric rash suggests meningococcal meningitis. Patients with increased intracranial pressure may have papilledema. The following signs may be positive: Brudzinski's sign ( flexion of the neck in a supine patient results in involuntary flexion of the hips and knees), and Kernig's sign (attempts to extend the knee from the flexed thigh position are met with strong passive resistance). There also may be evidence of extracranial infections (e.g., sinusitis, otitis, and mastoiditis). The differential diagnoses include viral meningitis, Lyme disease, tuberculous meningitis, and cerebral hemorrhages; all have different CSF findings. In tuberculous meningitis, the presentation is subacute or chronic and the CSF protein and pressure are increased. Lymphocytes are the predominant cell types with cell counts of 100 - 700/uL. In Lyme disease, the CSF protein is normal or increased; lymphocytes are the predominant cell types with cell counts of 0 - 500/uL. In viral meningitis, CSF protein and pressure may be normal or increased; lymphocytes are the predominant cell types with cell counts of 100 - 200/uL. In cerebral hemorrhages, the CSF is bloody, the pressure is increased, and red blood cells are the predominant cell types. The CSF glucose is within the normal range. Penicillin G is used for Streptococcus pneumoniae, and Neisseria meningitidis is sensitive to the drug. Ceftriaxone and chloramphenicol are used for Haemophilus influenzae and Gram-negative bacilli. Oxacillin is used for Staphylococcus aureus. Ampicillin is used for Listeria monocytogenes. Early use of corticosteroids, such as dexamethasone, may avert the development of major neurologic sequelae; they do so by inhibiting the release of pro-inflammatory cytokines triggered by bacterial fragments (which result from antibiotic activity). A 72-year-old man presents with the inability to comprehend spoken language. His speech is fluent and has a normal cadence and rhythm; however, when he talks, it is gibberish. He frequently substitutes one word for another. What is this phenomenon called? Answer Choices 1 Paraphasia 2 Echolalia 3 Alexia 4 Apraxia 5 Agraphia ANS:1 Paraphasia Paraphasia is a type of aphasia. The substitution of a similar sounding word for another word is called paraphasia. With paraphasia, the words can also be jumbled. If a patient is able to hear things and repeat them, it is called echolalia. With echolalia, the patient does not understand what he has heard. This is also referred to as echophrasia. Alexia is a type of aphasia. An aphasia in which there is a problem with reading is called alexia. Alexia is word blindness or text blindness. Alexia is also called optical aphasia or visual aphasia. Apraxia refers to the condition in which a patient has difficulty performing motor acts, despite having the muscular capacity and coordination to do so. A patient with apraxia cannot execute the intended movement. A writing disturbance is called agraphia. There are various forms of agraphia. With absolute agraphia, even simple letters cannot be written. This is also referred to as literal agraphia. Radiculopathy due to nerve root compression occurs most commonly at which nerve root within the brachial plexus? A C5 B C6 C C7 D C8 E T1 ANS:CC7 the C7 nerve root is affected the most often (approximately 45-60%). This radiculopathy can result from foraminal encroachment of the spinal nerve, cervical disk herniation, tumor, and multiple sclerosis. C7 radiculopathy can present with weakness in the triceps, which cause elbow extension, and finger flexion and extension. C6 is another common site of radiculopathy. C6 radiculopathy can present with weakness in the biceps, brachioradialis, and wrist extensor muscles. Cervical radiculopathy at the C5, C8, and T1 are less common, but still possible. C5 radiculopathy can present with deltoid and biceps muscle weakness. C8 radiculopathy can present with finger flexor weakness and T1 radiculopathy with finger abduction weakness. A 48-year-old woman presents with new-onset headache that she describes as nonspecific, worse on awakening, intermittent throughout the day but can worsen with bending over or coughing. Her husband reports that she has not been herself since the headaches started about 4 to 6 weeks ago. Which of the following tests would be best for determining the etiology of her presenting symptoms? A noncontrast head CT scan B lumbar puncture C contrast-enhanced brain MRI 2 Constipation 3 Thrombocytopenia 4 Leukopenia 5 Elevated liver enzymes ANS: 1 rash Explanation Skin rash is a common side effect of ampicillin. Rash occurs at a rate of approximately 3-7%. Constipation is not a side effect of ampicillin. Diarrhea is a side effect of ampicillin, rather than constipation. Thrombocytopenia, leukopenia, and elevated liver enzymes can be seen but less frequently than a rash. References: A 67-year-old man with an 80-pack/year smoking history and chronic bronchitis presents 30 minutes after suffering what was described as a "seizure" by his wife. According to his wife, this was his first seizure. During the seizure episode, the patient did not lose consciousness, but was observed to have developed an "involuntary jerking in his right arm and leg" that lasted about 5 minutes, with right arm weakness immediately following this involuntary activity. On physical exam, the patient appears drowsy, confused, and answers questions slowly. His right arm has objective weakness rated as 4/5; the remainder of the neurological exam is normal. Question Ico-delete Highlights What aspect of this patient's presentation most closely identifies this event as a simple focal seizure? Answer Choices 1 Involuntary extremity jerking 2 Right arm weakness is noted 3 There was no impairment of consciousness 4 There is postictal confusion 5 First seizure as an adult ANS: 3 there was no impairment of consciousness A 20-year-old college student describes his headaches as unilateral, a 5 out of 10 for pain, and with throbbing, associated with photophobia and often accompanied by nausea and vomiting. He states that the headaches occur two to three times a month and are associated with decreased sleep. He has taken OTC medication with relief and is trying to stay on a sleep schedule. He denies any other neurologic symptoms. Which of the following is the most appropriate next step for managing this patient? A Brain MRI B Hydrocodone-acetaminophen tablets C Physical therapy D Topiramate E Sumatriptan nasal spray ANS: Classic migraines often present with episodic, unilateral, and throbbing headache pain, associated with photophobia and phonophobia. Nausea and vomiting may also be involved. Management of migraines includes nonpharmacologic therapies, such as healthy eating, sleep pattern stabilization, caffeine avoidance, and stress relief. If nonpharmacologic therapies are not sufficient, or if symptoms impact activities of daily living, pharmacologic management is indicated. Medications are available for migraine sufferers and are typically catergorized as abortive or preventive. Abortive therapy for this patient may include nonsteroidal anti- inflammatory medications. Additionally, studies have shown that stimulation of the 5-HT receptors can successfully stop a migraine, and the 5-HT1 receptor agonists, with selective agents (the triptans) often used successfully for headache improvement. Nasal spray formulations are useful for nausea and vomiting. Topiramate, an anticonvulsant, has received FDA approval for migraine prevention therapy in patients with increasing migraine frequency or poor response to abortive therapies. Narcotics are typically avoided for migraine management, and MRI is not warranted without additional clinical signs and symptoms, and other differential diagnoses. Which of the following diagnostic studies is indicated for a patient with amaurosis fugax? A CT of the head B Intraocular pressure C Temporal artery biopsy D Carotid ultrasound E Ocular fluorescein angiogram ANS:D D Carotid Ultrasound Amaurosis fugax is a monocular vision loss that appears like a curtain passing over the eye, and comes from carotid artery disease. A CT of the head is indicated for lateralizing stroke symptoms. Intraocular pressure is taken for evaluation of chronic or acute glaucoma. A temporal artery biopsy is taken if giant cell arteritis is suspected. An ocular fluorescein angiogram is done to evaluate retinal disorders. Which of the following findings is consistent with a lower motor neuron deficit? A aphasia B dysdiadochokinesia C sensory loss D weakness E hyperreflexia ANS DD Weakness Weakness is one potential finding of a lower motor neuron process. Hyporeflexia is also common. Aphasia results from injury to the speech pathways within the brain. Sensory loss arises from many causes, but it is not a motor issue. Dysdiadochokinesia is consistent with cerebellar pathology. Hyperreflexia is typically a signal of upper motor neuron disease. Your patient is a 66-year-old female who has been dropping her coffee cup and concurrently slurring her speech. The episodes last for approximately 15 minutes. Her blood work, carotid dopplers, and MRI of the brain are normal and you suspect recurrent transient ischemic attacks (TIAs). Which of the following is NOT approved or recommended for the prevention of stroke in this patient? A aspirin B extended-release dipyridamole plus aspirin C clopidogrel D prasugrel E warfarin ANS:EE Warfarin Aspirin, aspirin plus extended-release dipyridamole, and clopidogrel are all antiplatelet agents and approved for use to reduce recurrent TIAs and ischemic cerebrovascular accidents (CVAs). Prasugrel is not FDA-approved for this indication. Warfarin is an anticoagulant and has no role in prevention of either recurrent TIA or ischemic CVA. A 68-year-old female presents to the emergency department with signs and symptoms of an acute ischemic stroke. The initial CT scan is normal. Her blood pressure is 164/105. What is the most appropriate treatment for the blood pressure of this patient? A Atenolol PO B Clonidine PO C Close monitoring D Labetolol IV E Nicardipine IV ANS:CC Close monitoring Aggressively lowering blood pressure may decrease blood flow to the ischemic tissue, thus decreasing the chances of recovery or increasing the risk of further infarction. In the setting of an acute ischemic stroke, blood pressure elevation should be monitored closely, with some elevation expected. This elevation is expected to decline without medication in the first few hours to days, but if elevation continues to a systolic blood pressure greater than 220mmHg, or mean arterial pressure greater than 120mmHg, medication is advised. Medications may include intravenous labetolol or nicardipine, with close monitoring of the patient. After the acute phase following a stroke, appropriate oral medications may be considered for outpatient hypertension management. Which of the following medication classes has been shown to improve the short-term rate of cognitive preservation in mild to moderate Alzheimer's dementia patients? A Acetylcholinesterase inhibitors B Decarboxylase inhibitors C Dopamine receptor agonists D Monoamine oxidase inhibitor E N-methyl-D-aspartate receptor antagonist ANS:A A Acetylcholinesterase inhibitors Alzheimer's dementia is a chronic, progressive, neurodegenerative disorder. Acetylcholinesterase inhibitors have been associated with a modest decrease of cognitive decline and increased functioning. These medications increase the levels of acetylcholine, a neurotransmitter, and increase cholinergic activity within the affected brain regions. They have been approved for use in mild to moderate Alzheimer's disease. Memantine, an N-methyl-D-aspartate receptor antagonist, is indicated for use in moderate to severe disease, has been associated with decreased destruction of cholinergic neurons, and may slow cognitive decline. It is often added to acetylcholinesterase inhibitor therapy for progressed disease, but may be first line in a patient unable to tolerate the acetylcholinesterase inhibitor medications. Decarboxylase inhibitor medications inhibit the metabolism of dopamine; one example, carbidopa, is used in conjunction with L-dopa in the treatment of Parkinson's disease. Dopamine receptor agonists activate dopamine receptors in the absence of dopamine, and are useful in conditions with low dopamine levels, such as Parkinson's, prolactinomas, and restless leg syndrome. progressed to weakness that now affects both lower and upper extremities. Within the past day, he has started to notice difficulty swallowing and double vision. He also feels it is difficult for him to take a big breath. His past medical history is noncontributory, and he takes no medications. Exam reveals bilateral absence of patellar and ulnar reflexes. Question What is the most likely prognosis for this patient? Answer Choices 1 Complete resolution of symptoms 2 Gradual resolution of paralysis and residual paresthesias 3 Gradual resolution of paresthesias and residual lower extremity weakness 4 Progressive paralysis and premature death most likely due to respiratory failure 5 Waxing and waning polyarticular weakness and paresthesias Complete Resolution The vast majority of patients (>80%) with Guillain-Barré syndrome (GBS) experience a full recovery with supportive care. In addition to supportive care, plasmapheresis and/or intravenous immune globulin are often used to reduce the duration and severity of symptoms once the diagnosis is recognized. Symmetrical ascending paralysis as described in this patient is indicative of Guillain-Barré syndrome (GBS). The cause of GBS is unknown, but it is generally thought to be an inflammatory autoimmune process. More than 50% of patients with GBS report an antecedent illness. The antibodies produced in response to antigens present in the infectious agent are thought to cross-react with components of human neurons, prompting an acute post-infectious demyelinating process. Lumbar puncture characteristically reveals elevated CSF protein content. Other results are normal, although the white blood cell count may be somewhat elevated. Decreased CSF glucose and increased polymorphonuclear cell counts are seen in acute bacterial meningitis. Decreased CSF glucose and elevated CSF lymphocyte counts are commonly seen with meningitis caused by fungi. Viral meningitis usually presents with elevated lymphocyte counts, normal CSF glucose, and normal or slightly elevated CSF protein levels. A 36-year-old woman presents with fatigue and diplopia; symptoms started in the morning and worsened during her time at work. On examination, the patient has ptosis bilaterally and decreased ocular muscle power. A CT scan of the chest shows a thymoma. What is the pathophysiology of this patient's neuromuscular disorder? Answer Choices 1 A genetic defect resulting in the production of atypical, or faulty, plasma cholinesterase 2 An autoimmune response that initially blocks, then destroys, nicotinic receptors at the neuromuscular junction 3 An increase in the ratio of muscle fibers to motor nerve fibers, resulting in production of a motor unit (MU) that provides less control over muscle actions 4 Production of IgA antibodies against acetylcholine receptors of the motor end plates of skeletal muscle 5 Genetic defect resulting in the deficiency of dystrophin An autoimmune response that initially blocks, then destroys, nicotinic receptors at the neuromuscular junction Myasthenia gravis is a neuromuscular disorder caused by immune mediated loss of acetylcholine receptors. It is associated with thymic hyperplasia in 65% of the cases and with thymoma in 15% of the cases. Myasthenia gravis (MG) is an autoimmune disorder affecting the neurochemical transmission of information between motor nerve fibers and skeletal muscle fibers. In this disorder, nerve impulses are transmitted in a normal fashion to the axon terminals, and skeletal muscle is capable of contracting normally. The dysfunction occurs at the interface between the 2 components of the neuromuscular junction (NMJ). In cases of MG, an autoimmune response causes antibodies of the IgG (not IgA) class to be produced against the nicotinic receptors of the NMJ. The antibodies fix onto the nicotinic receptors, block acetylcholine (ACh) from complexing with the receptors, and ultimately bring about destruction of the receptors. The patient may present with ophthalmic or non-ophthalmic manifestations. The ocular disturbances mainly include ptosis and diplopia; the non-ophthalmic symptoms include difficulty in talking, swallowing, or chewing and upper limb and trunk weakness. Anti-acetylcholine receptor antibodies assay is positive in 90% of the patients with generalized MG, whereas it is positive in 50-70% in ocular MG. Other tests include the Tensilon test, imaging studies such as CT or MRI scanning, repetitive nerve stimulation, and single fiber electromyography. Medical treatment includes neostigmine therapy, plasmapheresis, or intravenous immunoglobulin therapy. Thymectomy may be considered in cases of thymoma. The reduction in receptor population decreases neurotransmission, and the result is that muscle depolarization is not produced. The quantity and quality of cholinesterase enzymes is unaffected by the disorder, and so is any alteration in the composition of motor units at the neuromuscular junctions. A genetic disorder associated with deficiency of the structural protein dystrophin in muscles occurs in Duchenne muscular dystrophy, and it is not seen in myasthenia gravis. A 3-year-old boy presents following a car accident. He had not been buckled in, and he was thrown against the windshield. When the paramedics arrived at the scene, he was crying and clinging to his mother. Upon arrival at the emergency department, he is sobbing and does not react to her any more. He sustained lacerations of the face and both arms. His blood pressure is 150/84, pulse 62, and the pupils react sluggishly. He also has a temperature of 39.2° C. What do his symptoms indicate? Answer Choices 1 Viral infection 2 Reye's syndrome 3 Increased intracranial pressure 4 Anxiety attack 5 Guillain-Barré syndrome increased intracranial pressure This child most certainly sustained head injuries with increased intracranial pressure in the accident. It is very likely, since he was not buckled in, that his face and arms were cut when he was thrown against the windshield. His pupil reaction, disorientation, and not recognizing his mother all support that theory. Elevated temperature is common in patients with head injuries and is called 'central fever'. The only symptom that points to viral infection is the fever, but there would not be any changes in pupil reaction. Reye's syndrome is caused by aspirin medication with a viral infection being present. It affects the central nervous system, but it is not related to trauma. A child may cry and be very anxious in a hospital setting, but that would not cause temperature elevation and sluggish pupils. Guillain-Barré syndrome, or acute idiopathic polyneuritis, is a rapidly progressing ascending motor neuron paralysis of unknown etiology, which often follows viral infections. A 60-year-old woman presents for evaluation of loss of recent and remote memory and has psychomotor slowing with difficulty in complex tasks. On examination, the patient also shows some tremors in the hands and face. She also presents with a shuffling gait. Family history reveals 2 of her brothers had a movement disorder; they are now deceased. What would be your provisional diagnosis? Answer Choices 1 Delirium 2 Dementia of the Alzheimer's type 3 Vascular dementia 4 Parkinson's disease 5 Substance induced dementia Parkinson's Dementia due to medical conditions include loss of memory and other cognitive deficits due to diseases such as Huntington's disease, Parkinson's, Pick's disease, multiple sclerosis, HIV disease, vitamin B-12 deficiencies, and hypothyroidism. In addition, head traumas and tumors can cause dementia, and all physical causes have to be ruled out first. Vascular dementia presents with the same signs and symptoms as other dementias, but the patient usually has a strong history of cerebrovascular disease, multiple infarcts, or history of arteriosclerotic disease. Vascular dementia usually has a sudden acute onset after the infarcts. Vascular dementia may also include behavioral disturbances in the patient. In patients with a delirium, there are usually rapid fluctuations in cognitive functioning, brief durations of normalcy, and disturbances in the sleep-wake cycle. In addition, deliriums come on acutely and rapidly. It is somewhat common in older patients who have had heart surgery and in those who have experienced a change of environments, such as a hospital intensive unit. In addition, falls have been known to bring on a delirium. When an older adult has a fall, it is very important to perform a CT scan of the head to rule out concussions, head trauma, hematomas, and other injuries. Clients with substance-induced dementia have cognitive deficits related to a drug or alcohol use or abuse and/or toxicity of prescription drugs; the toxicity can be accidental or intentional. This appears to be more common in the elderly than once thought. Many elderly take prescription Answer Choices 1 Scissors gait 2 Steppage gait 3 Sensory ataxia 4 Cerebellar ataxia 5 Myopathic gait Cerebellar Ataxia: Cerebellar Ataxia= The gait characterized by cerebellar ataxia is associated with a disease of the cerebellum or its affiliated tracts. The gait is staggering, unsteady, and wide-based. The patient has difficulty with turns, and cannot stand steady when feet are together and eyes closed (Romberg's sign). Scissors gait is apparent when each leg is advanced slowly and the thighs tend to cross each other. The gait is stiff and the steps are short. This is associated with bilateral spastic paresis of the legs. Steppage gait is associated with a foot drop, often secondary to lower neuron disease. The patient drags his foot, or lifts the knee high, and slaps the foot against the ground. This gait may be unilateral or bilateral. Sensory ataxia gait is unsteady and wide-based. The feet are thrown forward, out, then down. There is a distinct 2-slap sound, secondary to the heel striking first then the forefoot. This is associated with polyneuropathy or posterior column damage. A myopathic gait is where there is weakness on one side. This displays a a drop in the pelvic area on the contralateral side of the pelvis while walking (Trendelenburg sign). When there is a bilateral weakness, it will display as a dropping of the pelvic girdle on both sides of the body while walking that results in a waddle. This is seen in patients whom are afflicted with myopathies, such as muscular dystrophy. A 72-year-old man presents with low-grade fever, nausea, confusion, and lethargy. His past medical history is significant for hypertension, hypercholesterolemia, and diabetes. He had sinusitis approximately 1 week prior to presentation; otherwise, he has been healthy. Laboratory workup shows a CSF with elevated opening pressure and blood glucose levels. They also show a low CSF glucose. A Gram stain on the CSF shows Gram-positive coccus. CBC shows an elevation of PMNs, but it is otherwise normal. What is the most appropriate treatment in this case? Answer Choices 1 Antibiotic therapy 2 Steroids 3 Supportive care 4 Transfusion 5 Antiviral treatment ABX therapy This patient is suffering from meningitis, which is an infection of the meninges that causes inflammation. It may be acute or chronic, and many different agents can cause it. Acute meningitis develops in a few hours to a few days, and chronic meningitis may develop weeks to months after exposure. Symptoms may be different in children or in older adults with underlying health conditions, such as diabetes or other immunocompromised states, IV drug users, those on a shunt for hydrocephalus, and extremes of age. Instead of presenting with headache and nuchal rigidity, they may present with lethargy and confusion. Both infectious and non-infectious agents can cause this disease. There are many infectious agents, including bacteria, viruses, fungi, and parasites. The Gram-positive cocci in the patient's CSF points to Streptococcus pneumoniae as the organism responsible. Bacterial meningitis is treated mainly with antibiotic therapy. The treatment usually consists of ampicillin plus ceftriaxone or cefotaxime plus vancomycin when meningitis is suspected and no other data is available. Vancomycin is added to cover penicillin-resistant S. pneumoniae, which may be present. If a Gram stain has been done and shows Gram-positive cocci, the treatment of choice is penicillin G or ceftriaxone. Meropenem is the drug of choice for cephalosporin- resistant isolates. When deciding on an antibiotic regimen, it is important to keep in mind the person's age and health status. Steroids are not considered first-line treatment. However, they can be used as an adjuvant therapy. They are thought to help lessen the severity of symptoms. Dexamethasone may be considered to reduce cerebral edema. Supportive care is important, especially if the patient's disease is advanced. However, antibiotic therapy should be initiated as soon as possible to avoid complications or death. On rare occasions, anticonvulsants may be required. Transfusion is not considered the treatment of choice for bacterial meningitis. If the patient has an underlying anemia or lacks certain clotting factors, transfusions would be supplied as part of his care. However, there is no indication of this in this case. Since he has a Gram stain showing Gram-positive cocci in his CSF, antiviral therapy is not indicated in this patient. Antibiotic therapy should be initiated immediately, and supportive care should be given as needed. A 37-year-old Hispanic man presents with a 4-month history of mild-to-moderate headaches; on average, he gets them 3 - 4 days per week. He has tried over-the-counter analgesics with minimal relief. He is seeking care now because he had been promoted to store manager several months prior to presentation, and he is worried that his headaches are affecting his concentration. His headaches are generalized in location, described as starting at the base of his head and extending all over, feeling "tight" in nature. He denies memory loss, photophobia, nausea/vomiting, rhinorrhea, lacrimation, and upper respiratory symptoms associated with the headaches. He also denies seizures, syncope, incoordination, vertigo, weakness, and paresthesias. The patient mentions his concerns for his work several times. Although he enjoys his work, he admits to having some anxiety about being able to handle his new duties. His family history is negative for headache. Physical exam is performed; vitals, neurological, cardiovascular, and HEENT findings are all normal. Question What is the most likely diagnosis? Answer Choices 1 Cluster headache 2 Headache secondary to brain tumor 3 Migraine headache 4 Subarachnoid hemorrhage 5 Tension headache Tension HA This patient presents with a most likely diagnosis of tension headache. Tension headaches, the most common type of headache, present as rather diffuse/generalized head pain, without the classic characteristics of the other types of headaches. A component of psychological stress is often present. Cluster headaches are a severe type of headache, most often occurring in middle-aged men, in which the severe headache is accompanied by several other symptoms, such as lacrimation, nasal rhinorrhea, and congestion. The pain is usually localized in the periorbital region. About 1/3 of patients with a brain tumor will present with a headache; however, tension-type headaches are far more common in the general population. Headaches secondary to brain tumor can vary in character and intensity. New onset headaches later in life, especially those without a coinciding significant psychosocial stressor, are of greater concern; they have a larger possibility of being caused by an intracranial mass. Nausea and vomiting or neurologic symptoms may accompany headaches caused by brain tumor. Migraine headaches are also quite common. They are classically described as throbbing in nature and are accompanied by nausea (and possibly vomiting), photophobia (sensitivity to light), and phonophobia (sensitivity to sounds)' visual changes and aura are also possible. Migraines are more common in women than men, but they can occur in men. They are usually a chronic condition, beginning in adolescence or the early adult years. Stress can be among many triggers for migraines. This patient's pain pattern and age of onset are inconsistent with migraine, and he lacks the associated symptoms. Subarachnoid hemorrhage (SAH) is classically described by patients as an acute headache that is the "worst headache of my life." There may be preceding trauma and associated nausea and vomiting; nuchal rigidity (due to meningeal irritation) and mental status changes are also seen. This patient's headache history is inconsistent with SAH. A 40-year-old woman presents with a 7-day history of pain in her right arm. The patient denies any trauma or injury to this extremity just prior to the pain starting, but she does admit to having a Colles' fracture in this arm about 2 months ago. She denies any injury to her back, neck, or other components of the musculoskeletal system prior to the event of pain. She describes the pain Her past medical history is unremarkable. She had 3 uncomplicated pregnancies and deliveries; she has no chronic medical conditions. Except for a mild tremor on the patient's right hand when held extended, the physical exam is unremarkable. Her neurological exam is otherwise normal. Question What is the most appropriate intervention for this patient's condition? Answer Choices 1 Advise the patient her condition is benign and no treatments are available 2 Initiate carbidopa/levodopa 3 Initiate methylphenidate 4 Initiate propranolol 5 Referral for physical therapy Initiate Propranolol Based on her history and exam, this patient has a benign essential (familial) tremor, for which it is appropriate to initiate propranolol. Propranolol is a non-selective beta-blocker. This patient has no contraindications to the therapy; she is troubled by the worsening tremor, which interferes with her daily activities. Essential tremor is indeed a benign condition. When the tremor is mild and does not cause embarrassment or interfere with the patient's activities, it would be reasonable to avoid medications, but it is inappropriate to tell the patient that no treatments exist. Especially in this case, the patient desires treatment. Carbidopa/levodopa would be an appropriate medication for an individual with tremor due to Parkinson's disease; however, this patient has no other neurological complaints and no history to suggest she currently has Parkinson's disease. Methylphenidate is a stimulant; it is typically used for the treatment of attention deficit hyperactivity disorder. Not only would it be ineffective in this patient's case, it would also likely make her tremor worse. Physical therapy has not been proven to have a role in treating essential tremor. It would not be helpful in this case. What is the most accurate statement concerning the treatment of migraine headache with sumatriptan? Answer Choices 1 Its effectiveness is related to its action as a serotonin antagonist 2 Its most common side effect is irritability 3 The recommended oral dose is 6 mg 4 It is indicated for both treatment and prophylaxis of migraine headache 5 If it is effective in the initial therapy, it is often effective in aborting a recurrence of symptoms If it is effective in the initial therapy, it is often effective in aborting a recurrence of symptoms f it is effective in the initial therapy, it is often effective in aborting a recurrence of symptoms. Sumatriptan is a serotonin agonist; it is believed that its effectiveness is related to the importance of serotonin in the regulation of cranial and intracranial vasoactivity. The most common side effect is nausea with or without vomiting; however, these are frequent symptoms of a migraine headache itself. It may also cause sedation and drowsiness, but irritability is not common. The recommended oral dose is 25 mg, and the recommended subcutaneous dose is 6 mg. Sumatriptan is indicated for the treatment of a migraine headache, but it is not recommended for prophylaxis. Clinical trials have shown that sumatriptan is effective in reducing the severity of a migraine headache in approximately 70 - 80% of patients, but recurrence of the headache is seen in as many as 35 - 45%; a 2nd dose of sumatriptan is effective in treating the recurrence in the majority of patients. A 61-year-old man with hypertension presents with sudden onset of right eye blindness while shaving this morning. He describes a "curtain falling over my right eye," which cleared spontaneously after 10 minutes. He had no other concurrent neurologic symptoms and no prior episodes of similar description. His general examination reveals a blood pressure of 140/90 mmHg, but it is otherwise unremarkable. Neurologic exam is normal. Question What is the most likely diagnosis? Answer Choices 1 Right internal carotid artery transient ischemic attack (TIA) 2 Basilar artery TIA 3 Left posterior cerebral TIA 4 Left internal carotid artery TIA Right ICA TIA The diagnosis in this patient is amaurosis fugax, or transient monocular blindness. This disorder is most often due to ipsilateral internal carotid artery (ICA) atherosclerosis, leading to a transient ischemic attack (TIA) of the retina. A TIA is defined as a focal neurologic deficit (e.g. amaurosis fugax, hemiplegia, aphasia) lasting less than 24 hours, which is referable to the distribution of the carotid or vertebral-basilar circulation. The vascular supply of the retina is through the ophthalmic artery, which is an intracranial branch of the ICA. Thus, this patient had a TIA of right ICA origin. Visual disturbances of the basilar or posterior cerebral circulation include diplopia, bilateral (binocular) blindness, and hemianopia. Atheroembolism from the ICA to the ipsilateral ophthalmic artery is the most likely mechanism of amaurosis fugax. Other causes of TIA include hemodynamic (low perfusion) impairment, arteritis, thrombocytosis, polycythemia vera, and cardiogenic embolus. Rarely, a cerebral mass lesion (neoplasm, abscess, vascular malformation) produces syndromes that masquerade as TIA's. Management of patients with carotid TIA is directed at identifying an accountable vascular (or cardiac) lesion, since the annual risk of ipsilateral stroke is approximately 15%. Appropriate workup for a patient with TIA includes: brain CT or MRI to visualize silent infarcts or mass lesions; carotid duplex, magnetic resonance or CT angiography, and conventional angiography to determine the presence of carotid stenosis; and cardiac evaluation (ECG, echocardiography, Holter monitor) to exclude a cardiogenic source of embolism. Medical therapy for stroke prevention includes antiplatelet agents (aspirin, ticlopidine) for patients with primary carotid disease with less than 70% stenosis; anticoagulation has been shown to be effective for cardiogenic stroke prevention (e.g. atrial fibrillation). Surgical treatment, i.e. carotid endarterectomy, is superior to medical therapy in patients with 70% or greater stenosis of the carotid artery. The latter was confirmed by the North American Symptomatic Carotid Endarterectomy Trial (NASCET) in 1991. The benefits of carotid endarterectomy decline in patients with significant comorbidity (i.e. cancer, coronary artery disease), and this procedure should be performed by experienced surgeons. The treatment of asymptomatic carotid stenosis remains controversial. What is the treatment of choice for primary generalized seizures? Answer Choices 1 Phenytoin 2 Phenobarbital 3 Valproic acid 4 Carbamazepine 5 Gabapentin Valporic Acid Epilepsy is defined as a syndrome characterized by recurrent, unprovoked seizures. A seizure is best defined as a discrete neurologic event caused by abnormal, excessive, spontaneous discharge of cerebral cortical neurons. The phenomenology of a single seizure is dependent upon the origin and subsequent spread of neuronal discharge. Epileptic seizures, as opposed to symptomatic seizures provoked by acute brain disturbances (e.g. hyponatremia, meningitis, cocaine use), are treated medically with anticonvulsants. Specific anti-epileptic drugs are efficacious for different types of seizure. Primary generalized epilepsy is commonly associated with developmental brain disorders of childhood (congenital and acquired). Types of primary generalized seizures include tonic-clonic, absence, tonic, clonic, myoclonic, and atonic. Anticonvulsants indicated for primary generalized seizures include valproic acid, clonazepam, and ethosuximide. Localization-related epilepsy results in partial (focal) seizures with or without secondary generalization. These are best treated with phenytoin, carbamazepine, phenobarbital, and valproic acid. The newer anticonvulsants, gabapentin, felbamate, and lamotrigine are also effective for partial seizures. A seizure is defined as a discrete neurologic disorder in which there is excessive and synchronous discharge of cortical neurons. Epilepsy is a syndrome characterized by recurrent, unprovoked seizures. A seizure may be classified as generalized or partial. In a generalized seizure, there is bilateral cerebral hemisphere disturbance; in partial seizures, the abnormal neuronal discharge is limited to a specific brain area (e.g., right anterior temporal lobe). Generalized seizures may be primary (bilateral brain involvement at seizure onset) or secondary generalized (seizure starts in a focal brain area, then spreads to encompasses both cerebral hemispheres). Valproic acid is usually the first-line medication for primary generalized seizures. Phenytoin, phenobarbital, carbamazepine, and gabapentin have efficacy in partial and secondary generalized seizures. Phenytoin and phenobarbital have a role in the treatment of primary generalized seizures, but valproic acid is superior. A 19-year-old man presents with a sudden onset of vomiting, headache, vision changes, numbness and tingling of extremities, difficulty speaking, difficulty writing, loss of coordination, and loss of balance. His past medical history is significant for sickle cell anemia. Physical exam findings are consistent with acute cerebrovascular insufficiency. Early detection and prevention of stroke in sickle cell anemia is now possible by using screening tests. What imaging modality is recommended as a screening test for cerebrovascular disease/stroke in sickle cell disease (SCD) patients? Answer Choices 1 Magnetic resonance imaging (MRI) 2 Computed tomography (CT) skull 3 X-ray skull 4 Neurocognitive testing 5 Transcranial Doppler ultrasonography (TCD) Correct Transcranial Doppler ultrasonography (TCD) Stroke or cerebrovascular insufficiency occurs in about 11% of patients under 20 years of age with sickle cell anemia. The major symptom includes sudden hemiparesis with or without aphasia. Most of these strokes occur in the distribution of the intracranial internal carotid artery or the middle cerebral artery. The Stroke Prevention Trial in Sickle Cell Anemia (STOP Trial) confirmed that TCD can identify children with SCD at high risk for first-time stroke. As the greatest risk of stroke occurs in early childhood, it is recommended that children aged 2 - 16 years receive TCD screening. Stroke in SCD is a medical emergency and requires mandatory exchange transfusion followed by maintenance hypertransfusion. Studies have shown that about 90% of strokes could be prevented in children with stenotic cranial-artery lesions as demonstrated on transcranial Doppler ultrasonography if they are maintained on a regular program of chronic transfusion. The chronic transfusion should be such that it suppresses erythropoiesis so that no more than 30% of the circulating red cells are their own; this is done in order to maintain the Hb S level below 30%. Computed tomography (CT) and magnetic resonance imaging (MRI) are used for diagnosing strokes, but they are not used as a screening modality. Patients with poor educational performance should undergo neurocognitive testing, as they may have early ischemia of the central nervous system. It is recommended that children with sickle cell disease be screened for cerebrovascular disease with TCD and not MRI, CT, X-ray skull, or neurocognitive testing. A 70-year-old woman presents with a 1-year history of short-term memory loss. She did not seek medical attention earlier because she believed the memory loss to be age-related. Her husband has also noticed that she is unable to perform her regular daily activities and is often misplacing things. Recently, she was unable to get back home after her evening walk, and neighbors brought her home. The husband also feels she has been acting strange lately, and she is suspicious of her own son. Her past medical history is significant for hypertension and she has been on medication for the past 3 years. On clinical examination, she appears conscious and alert. Her vitals are PR- 70/min, BP- 120/74 mmHg. She is afebrile. Question What is your provisional diagnosis? Answer Choices 1 Dementia of the Alzheimer's type 2 Dementia due to a medical condition 3 Vascular dementia 4 Major depression 5 Substance induced dementia Dementia of the Alzheimer's Dementia of the Alzheimer's type involves multiple cognitive deficits including 1 or more of the following: aphasia or language disturbance; apraxia, or the inability to carry out motor activities despite intact motor function; agnosia, or the inability to recognize familiar objects; and the disturbance of executive activities, such as planning, organizing, sequencing, or abstracting. There can be changes in personality and behavior, in addition to the inability to learn and recall new information. Long-term memory is affected. The disease also has a gradual onset and continuing decline. Focal neurological signs and symptoms are usually absent. If the disease occurs before age 65, this is early-onset. If it occurs after age 65, the disease is late-onset. Vascular dementia has the same general symptoms and signs as Alzheimer's disease, except that the onset is usually more sudden and acute. In addition, it is picked up easily by clinical or laboratory evidence of a vascular cause, a history of cerebrovascular disease, or multiple infarctions. Dementia due to medical conditions may present with similar or the same symptoms as other dementias, but are due to other medical or neurological diseases such as head trauma, Parkinson's disease, Pick's disease, Huntington's disease, HIV, multiple sclerosis, vitamin deficiencies, and numerous other medical conditions. Many elderly suffer from major depression. Symptoms include short-term or recent memory loss, fatigue, insomnia or hypersomnia, low appetite, weight loss, overeating, and an inability of handling activities of daily living. In major depression, deficits in various cognitive domains may be present. Temporal lobe magnetic resonance imaging (MRI) is helpful in differentiating dementia of the Alzheimer's type from depression and other disorders that may cause diagnostic difficulties in clinical practice. Substance-induced dementia includes cognitive defects caused by inhalants, sedative drugs, hypnotic drugs, prescription side effects, overdoses of prescription drugs, or other substances. This is common in the elderly. A 10-year-old boy is referred to you for being fidgety at school, though he makes good grades. Prior history is unremarkable, and there has been no recent illness. The father had a similar history as a child. While speaking with the patient, you notice that he clears his throat several times per minute. Examination is otherwise normal, except for rapid, nonrhythmic jerking movements of the face, neck, and shoulders while at rest. Question What is true of this patient's case? Answer Choices 1 Does not have Tourette syndrome due to the absence of coprolalia 2 Has a factitious disorder 3 Has a condition that clusters in families 4 Must start a regimen of levodopa 5 Suffers from Sydenham chorea Has a condition that clusters in families The combination of vocal tics (here, throat clearing) and motor tics should suggest Gilles de la Tourette (Tourette) syndrome, which is a common tic disorder that clusters in families. Once thought to be a single gene, autosomal dominant condition, it appears that multiple genes and nongenetic factors may be at play. Coprolalia is but one of many vocal tics seen in Tourette syndrome (throat clearing, humming, whistling) and, although a notorious one, is not a necessary feature. The positive family history makes it unlikely that the condition described is factitious. Pimozide, SSRIs, and other agents have been used in the treatment of Tourette syndrome, but levodopa is not used. The movement disorder of Sydenham chorea, as seen after a streptococcal infection, is choreiform - that is, tends to be slower and rhythmic, as opposed to the rapid, nonrhythmic jerks seen in Tourette syndrome. Also, vocal tics are not typically seen with Sydenham chorea. A 28-year-old man presents following a road traffic accident 20 minutes prior. He states that it was just a "minor bump from behind," but feels he might have whiplash. His neck is stiff and sore, and he has developed numbness and tingling on the lateral surface of his right arm extending into his right fourth and fifth digits. On physical examination, his bicep strength is +5/5 on the left and +5/5 on the right. His biceps tendon reflex is 2+ on the left and 2+ on the mononuclear pleocytosis (100 white blood cells), elevated protein (100 mg/d), and a normal serum to glucose ratio; EEG shows focal abnormalities over temporal lobes, and her CT is normal. While waiting for the results of polymerase chain reaction, you start supportive care, anticonvulsants, diuretics, and acyclovir. Question What factor guides you to the choice of acyclovir? Answer Choices 1 EEG changes 2 CSF findings 3 Glucocorticoid therapy 4 Focal neurological deficit 5 Psychiatric symptoms EEG In an immunocompromised patient with this clinical picture, you should think about herpes encephalitis. Its presentation is often atypical: fever, headache, and alteration of consciousness, focal neurological deficits, seizures, and other signs of acute encephalitis. When you suspect encephalitis, the workup must be initiated rapidly, but the workup should not delay treatment. There are no pathognomonic clinical findings in herpes simplex encephalitis, but electroencephalography (EEG), with a specificity of 32% and sensitivity of 84%, should guide your decision about the therapy. Usual abnormal EEG patterns are focal abnormalities (periodic complexes and periodic lateralizing epileptiform discharges, spike and slow- or periodic sharp- wave patterns) over temporal lobes or diffuse slowing. The diagnosis will be confirmed only by polymerase chain reaction or brain biopsy, but those results will not be available immediately, and they should not delay therapy. In this case, the CT excluded a space-occupying lesion so that a lumbar puncture could be performed. CSF findings are not specific; they will only raise the suspicion of viral cause of the disease. Patients on glucocorticoid therapy and consequent immune suppression are prone to different infections, not only herpes simplex encephalitis. A focal neurological deficit is a non-specific finding. Focal neurologic deficits, cerebrospinal fluid pleocytosis, and abnormalities on computed tomography may initially be absent in herpes simplex encephalitis. Psychiatric symptoms are not specific for herpes simplex encephalitis; they are part of clinical picture of a variety of CNS disorders. A 45-year-old man goes to a party and enjoys several glasses of an alcoholic cocktail. His past medical history is significant for headaches. The drinks trigger a unilateral right-sided headache. The headache is behind his right eye and spreads to his forehead. He also notices that his right nostril has a watery discharge and his right eye is tearing. He describes the pain as if he were "being stabbed in my eye". Question What kind of headache does he have? Answer Choices 1 Tension headache 2 Hangover headache 3 Common migraine 4 Classic migraine 5 Cluster headache Cluster HA This patient has a cluster headache. Typically, cluster headaches occur in the spring and fall, as the case here. During a cluster period, the headaches can be triggered by alcohol. During an attack, there can be discharge from the nose and eye on the same side of the face that the headache is on. The pain of a cluster headache begins rapidly. The pain is described as stabbing or knifelike, as the case here. The headaches last up to 2 hours. Tension headaches are associated with stress. This kind of headache would not be associated with water discharge from the nose and eye. A hangover headache would not occur while the man is still drinking. This kind of headache would not be associated with water discharge from the nose and eye. Common migraine and classic migraine would not be associated with water discharge from the nose and eye. A patient presents with bilateral spastic paresis of his legs. His gait is noticeably stiff and his legs are advanced slowly. He uses short steps and crosses his legs in front of the other as he advances. What type of gait does he have? Answer Choices 1 Scissors gait 2 Steppage gait 3 Sensory ataxia gait 4 Cerebellar ataxia gait 5 Myopathic gait Scissors Gait Scissors gait is apparent when each leg is advanced slowly and the thighs tend to cross each other. The gait is stiff and the steps are short. This is associated with bilateral spastic paresis of the legs. Steppage gait is associated with a foot drop, often secondary to lower neuron disease. The patient drags his foot, or lifts the knee high and slaps the foot against the ground. This gait may be unilateral or bilateral. Sensory ataxia gait is unsteady and wide-based. The feet are thrown forward, out, and then down. There is a distinct 2-slap sound, secondary to the heel striking first, then the forefoot. This is associated with polyneuropathy or posterior column damage. The gait characterized by cerebellar ataxia is associated with a disease of the cerebellum or affiliated tracts. The gait is staggering, unsteady, and wide-based. The patient has difficulty with turns, and cannot stand steady when feet are together and eyes closed (Romberg's sign). A myopathic gait is where there is weakness on one side. This displays a a drop in the pelvic area on the contralateral side of the pelvis while walking (Trendelenburg sign). When there is a bilateral weakness, it will display as a dropping of the pelvic girdle on both sides of the body while walking that results in a waddle. This is seen in patients whom are afflicted with myopathies, such as muscular dystrophy. What condition is characterized by fine movements that are absent at rest, occur with activity, and worsen as the target is neared? Answer Choices 1 Postural tremor 2 Asterixis 3 Intention tremor 4 Chorea 5 Static tremorIntention Tremor Intention tremors are fine movements of the hands that are absent at rest, occur with activity, and worsen as the target is neared. They occur with multiple sclerosis and cerebellar disease. Choreiform movements are brief, rapid, jerky, unpredictable motions of the hands. They occur both at rest and during normal actions. They are associated with both Huntington's and Sydenham choreas. Asterixis is non-rhythmic flapping movements of the hands, especially if the wrists are dorsiflexed. It is associated with liver failure, renal failure, and pulmonary insufficiency. Static, or resting, tremors are coarse movements of the hands that are present at rest, disappear with movement, and may involve alternation of the fingers in a "pill-rolling" manner. They are associated with Parkinson's syndrome and extrapyramidal dysfunction. Postural tremors are fine movements of the hands that occur when the hands are held in an active position, usually against gravity. Examples of postural tremors are those resulting from anxiety, fatigue, and hyperthyroidism, as well as familial postural tremors. A 23-year-old man presents after being thrown from a motorcycle onto a cement median. He is awake and appears alert on exam. He believes that his helmet was knocked off at point of impact, but he cannot recall the specifics of his crash. A head CT scan is consistent with a cerebral contusion. What findings on a CT scan would you see with this diagnosis? Answer Choices 1 A peripheral ring of enhancement around areas of hematoma