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Journal of the Neurological Sciences 341 (2014) 153–157
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A case of Parry–Romberg syndrome and alien hand
Gurjit Chokar a, Alfonso Cerase b, Andrew Gough c, Sibte Hasan d, David Scullion e, Hany El-Sayeh f, Rosaria Buccoliero a,⁎ a Department of Neurology, Harrogate District Foundation Trust, Harrogate, UK b Unit NINT Neuroimaging and Neurointervention, Department of Neurological and Sensorineural Sciences, Azienda Ospedaliera Universitaria Senese, “Santa Maria alle Scotte” General Hospital, Viale Mario Bracci, 16, 53100 Siena, Italy c Department of Rheumatology, Harrogate District Foundation Trust, Harrogate, UK d Department of Neurophysiology, York Teaching Hospital, York, UK e Department of Radiology, Harrogate District Foundation Trust, UK f Department of Psychiatry, Harrogate District Foundation Trust, UK
⁎ Corresponding author at: Harrogate District Hosp Tel.: +44 1423 553038/4482; fax: +44 1423553091.
E-mail address: [email protected] (R. Buc
http://dx.doi.org/10.1016/j.jns.2014.04.004 0022-510X/© 2014 Elsevier B.V. All rights reserved.
a b s t r a c t
Article history: Received 5 June 2013 Received in revised form 11 February 2014 Accepted 6 April 2014 Available online 13 April 2014
Keywords: Parry–Romberg syndrome MRI scan Immunosuppression Vasculitis Status epilepticus Alien limb
Parry–Romberg syndrome (PRS) is a rare condition characterised by progressive hemi-facial atrophy. Here we present a PRS case with alien-hand syndrome, which has not previously been described in adult onset disease. On the basis of the presumed auto-immune pathology of PRS we justify the treatment strategy we successfully used in this patient. A review of the literature was extensively done for understanding the history of alien hand sign over the years.
© 2014 Elsevier B.V. All rights reserved.
Parry–Romberg syndrome (PRS; also known as Romberg's disease or progressive hemi-facial atrophy) is a rare disease, causing progressive wasting of the skin, subcutaneous fat, muscle and occasionally bones of the face. It most commonly involves dermatomes of one or more branches of the fifth cranial nerve . Occasionally it can extend to involve the ipsilateral or contralateral limbs [2,3]. The clinical course typically begins in early childhood, with females beingmore commonly affected than males . It can progress for 2–20 years .
Many associations have been documented in the literature. Head- aches, facial pain and seizures (particularly partial seizures) are relative- ly common [5,6]. A survey of 205 patients in 2003 reported the presence of these symptoms in 52%, 46%, and 11% (4%) respectively . Eye and visual problems were reported by 46% of the patients and 17% had his- tory of uveitis. Other specific ophthalmic complications reported from the literature include enopthalmos with various eyelid modifications (ptosis, retraction, atrophy) . Amblyopia and diplopia may be due to ocular nerve involvement, muscle paresis or restrictive oedema [9,
ital, Harrogate HG2 7SX, UK.
10]. Visual acuity can also be affected by retinal vasculitis , optic atrophy  and uveitis .
Arteriovenous malformations and intracranial aneurysms have also been reported [14,15], however these findings occur less commonly in the literature.
Only one case of alien hand syndrome with PRS has been described and this was a paediatric patient .
The exact pathophysiology of the disease is unknown though many support the theory that it has an autoimmune aetiology , as there are features that overlap with scleroderma and en coup de sabre syndromes . Hence treatment strategies involving steroids and immunosuppressants have been used [6,19,20] in PRS with some success.
Here we describe the case of a 36 year old patient with PRS from childhood, who presented with new-onset headache, seizures and unilateral alien hand syndrome.We discuss the neuroradiology, investi- gations and treatment.
2. Case report
A 36-year-old Caucasian left handed lady with previously diag- nosed PRS, and a history of bulimia, depression and alcohol abuse (50 units/week), was admitted (day 1) after an episode of loss of con- sciousness. The day after admission she suffered status epilepticus
154 G. Chokar et al. / Journal of the Neurological Sciences 341 (2014) 153–157
whichwas treatedwith Lorazepam and Phenytoin and required intuba- tion. Her prior medication was Fluoxetine and Disulfiram only. Her rel- atives commented that a few days before the event she complained of intermittent right temporal headachewith vomiting, episodes of confu- sion, visual hallucinations consisting of seeing heads or bodies of people disproportionately large, and auditory hallucinations where she could hear music for hours not heard by other members of the family.
Brain MRI (Fig. 1A) was performed on day 2 and showed right cranio-facial atrophy which was obvious on clinical examination and had been documented previously. It also showed previously undocu- mented left cerebral hemisphere atrophy consistent with alcohol abuse, subtle thickening and signal alteration on T2-weighted images of the cortex of the right cerebral hemisphere and ipsilateral thalamus, consistent with changes related to the previous status epilepticus.
On day 3, once Glasgow Coma Scale returned to normal following extubation, neurological examination revealed ideo-motor apraxia only with the left hand. Indeed shewas unable, when asked, to perform with the left hand some activities as brushing teeth or the hair or show- ing how to put a picture on the wall. It was also noticed that the first three digits of the left hand were held at times in a flexed position with modest bradykinesia but no clear dystonia or dyskinesia was ob- served. The assessment of orientation in time and space, strength, tone, language (understanding and speech), cerebellar system, cranial nerves, was normal but at that time visual field evaluation was not done. Tendon reflexes were present in the upper and lower limbs with equivocal plantars and no clonus. She reported apallesthesia of the left big toe and reduced proprioception on the left side.
Her initial tests, taken in intensive care revealed only mild neutrophilia with a normal biochemical profile. Her ANA, nDNA, ANCA, ACA and lupus anticoagulant were negative. Wilson's disease was excluded with normal ceruloplasmin, serum and urinary copper levels.
Fig. 1.Neuroimaging of the brain and patient's left hand activity. Serial non-consecutive T2-wei 2 show the right cranio-facial brain atrophy (white arrows), and a subtle thickening and high-in well as high-intensity signal (black arrowhead) of the pulvinar of the ipsilateral thalamus. Note metallic foreign body. When status epilepticus had been treated, the patient's left hand does no tagons (short black arrow) below them (C). At brainMRI obtained at re-admission (D) on day 2 signal alteration. On day 57 (E), brain MRI shows clear-cut atrophy, and reduced signal altera images (F) show the absence of perfusion in the right temporo-parieto-occipital lobes that we
She had further visual hallucinations, noted on day 8, when she re- ported seeing people from her past. EEG performed on day 9 showed that the right hemisphere appeared epileptogenic without a clear focus.
Lumbar puncture was performed on day 13. Cerebrospinal fluid (CSF) appeared blood-stained with 7 lymphocytes/cm2 but no organ- isms seen or cultured. CSF protein was slightly raised at 0.43 g/L (NR 0.2 — 0.4 g/L). Serological tests for syphilis, Lyme disease (IgG and IgM) and HIV were negative. HSV, varicella zoster, enterovirus and parechovirus were not detected by PCR. On the basis of the episodes of confusion with hallucinations, seizures and 100% CSF lymphocytosis with a slightly raised protein, she was initially treated with acyclovir for possible viral encephalitis (this was stopped on day 20 when the PCR for HSV result came back negative).
On day 14, the patient stated that she wanted to discharge herself from hospital against medical advice. During examination of capacity to make this decision, through a thorough neurological examination a left homonymous hemianopia was incidentally observed during the as- sessment of the visual field test. The patient did not notice it and did not want to look into. She discharged herself but was given Carbamazepine to prevent further seizures and vitamin B strong given her alcohol histo- ry and bulimia.
She represented 3 days later, day 17 in relation to initial presenta- tion, with further headache and vomiting.
Minimental state examination score on day 20 was 27/30 making one mistake in orientation by giving the wrong month, unable to write a sentence and copying the design just with her left hand (Fig. 1B, C).
A head CT angiogram on day 21 to explore a possible subarachnoid haemorrhage was negative, but revealed a hypodense region in the right parieto-occipital hemisphere (not shown). The left homonymous hemianopia was confirmed by visual field testing on day 24 and was not attributable to disease of the optic nerve or retina. The neurological
ghted axial magnetic resonance (MR) images (A) obtained during status epilepticus on day tensity signal (long black arrows) of right temporal, occipital, and fronto-parietal cortex, as also some image distortion (white arrowhead) in the right frontal region, consistent with t provide understandable writing (B), and does not repeat the drawing of intersected pen- 4, the right cerebral hemisphere showsmarked cortical–subcortical swelling and thalamus tion. On day 83, axial, coronal and sagittal single photon emission computed tomography re found further involved at brain MRI on day 96 (G).
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examination was unchanged in respect of the previous admission ex- cept for absence of bradykinesia with the left hand, previously noted.
Head MRI scan on day 24 confirmed the dramatic development of extensive right hemispheric cortical and subcortical swelling, with white matter oedema affecting temporal, parietal and occipital lobes with relative frontal sparing (Fig. 1D). Suspecting an underlying inflam- matory process, shewas commenced on oral Prednisolone 50mgonce a day with instructions to reduce the dose by 10mg per week. She asked to be discharged and she was followed up as outpatient.
Further headMRI scan (Fig. 1E), on day 57, revealed a significant re- duction in the extent of cerebral oedema. SPECT (Fig. 1F), performed on day 83, showed marked reduction of perfusion in the right cerebral hemisphere, consistent with the evolution of theMRI findings. Cerebral vasculitis was thought a possible explanation. A cerebral biopsy was proposed to confirm this but the patient declined. She also chose not to start any further steroid or immunosuppressive treatment at this stage.
At outpatient follow-up, approximately 6 months after initial pre- sentation, she complained of pain in her left upper limb, mainly her hand. She noted episodes of uncontrolled non-purposeful movements of this limb describing this as “doesn't go where you want it”, “gripping involuntary”, and “poking the eye of her mother while talking to her”. Her headaches also recurred and the left homonymous hemianopia persisted. The rest of the neurological examination was unchanged. Given the new features of suspected alien hand syndrome (never witnessed by professionals), she had a further head MRI scan on day 96which showed atrophy in the right temporal, and parietal and occip- ital lobes (Fig. 1G).
Following discussion at the Regional Neuroradiological Multidisci- plinary meeting it was concluded that cerebral vasculitis was the most likely cause of her clinical and radiological picture. On the basis of this she was treated with intravenous methylprednisolone 1 g daily for five days (commenced on 6 months and 4 days after initial presenta- tion) and then cyclophosphamide/methylprednisolone (10 mg/kg of each) pulses at 0, 2, 4, 7, 10 and 13 weeks. Following completion of this her follow-up head MRI scan (5 months since the previous one) was stable, her pain reduced and episodes of involuntary movement of the let upper limb became less frequent. Despite this the patient de- clined further immunosuppression to prevent relapse as she felt unwell post infusion.
Psychometric tests (conducted on several occasions between 7 and 9 months from initial presentation) showed severe depression and anx- iety, based on the Beck Depression Inventory—Second Edition (BDI-II) and the Beck Anxiety Inventory (BAI). Her pre-morbid intellectual abil- ity was assessed as average using the Wechsler Test of Adult Reading (WTAR) but the Wechsler Adult Intelligence Scale—Third Edition (WAIS-III) showed that she now had a full scale IQ of 79, which is in the borderline range as compared to age matched peers, revealing some decline in cognitive functioning. However she performed well in the subsets requiring verbal performance, showing that the decline was significantly in executive functioning, manipulation of visual infor- mation and those requiring spatial and perceptual ability (such as pic- ture arrangement or completion). She reported that she knew the answers to a number of questions but felt she was unable to articulate these. Interestingly, the behavioural assessment of dysexecutive syn- drome (BADS) test showed poor results in behavioural inhibition as well as planning. Problem solving however appeared to be preserved. She experienced significant difficulty on tests comprising the visuospa- tial/constructional and attentional functions of the Repeatable Battery for the Assessment of Neuropsychological Status (RBANS test), scoring in the 0.3rd percentile for her age group. These psychometric test results suggested lesions of the frontal and parietal regions. Further neuroim- aging still showed sparing of the frontal lobes but did show worsening atrophy of the same posterior lobe areas (images not shown).
Twelve months since initial presentation she reported worsening of the pain in the left upper limb, dropping things and more involuntary
movements with the left hand. She did then agree to start oral prednis- olone and azathioprine with improvement of her symptoms. She was able to draw and write with both hands.
Parry–Romberg syndrome has a well-established association with epilepsy, headaches and ophthalmological diseases. There is only one case report of an association with alien hand syndrome by Takenouchui and Solomon . They describe a 9 year old girl diagnosed with PRS at the age of 5, when she developed left side weakness and an abnormal gait. Four years later she presented with a few months history of brief paroxysmal semi-purposeful movements with the left hand. The head MRI scan showed a significant volume loss of the right thalamus. These findings were thought to be consistent with a “frontal alien hand syndrome”, despite no clear lesion on neuroimaging.
Alien hand syndrome is a rare condition that occurs in patients with cerebral lesions following injury, tumours, stroke, and callosotomy and has been described in corticobasal degeneration , multiple sclerosis  and as a manifestation of seizure activity .
Alien hand syndromehas been classified using the anatomical site of the lesion. The frontal or anterior form is when complex uncontrolled purposeful exploratory reaching movements occur. In the callosal form, intermanual conflict occurs where the left and right sidesmay be- have antagonistically. The parietal/occipital (also called posterior) form is where the involuntary movements are not only less complex and ataxic in comparison to the anterior form but also nonpurposeful . A few case reports have suggested a posterior form where the hand or limb tends to levitate into the air, displaying ataxic and nonconflictual movements with relevant sensory impairment and evidence of visual and somatosensory dysfunction .
Much debate and confusion has occurred over the years regarding classification and criteria for diagnosis. There is currently no consensus on definition or classification, rather “alien hand” or “alien limb” serves as an umbrella term for a heterogenous set of symptoms  typically affecting the upper limbs, with a range of anatomical lesions identified.
For a diagnosis of “alien hand” or “alien limb” these abnormal behav- iours cannot be attributed to failure to understand, inattention, uncooperativeness, amnesia, delusions or dementia and identifiable neurological movement disorders need to be excluded . The history of the phenomenon may provide some context to the lack of a precise definition or classification and detail the anatomical correlates. We reviewed the literature and reflect on the observations of other authors who dealt with cases of alien hand.
In 1908, Goldstein described Spontanbewengungen (spontaneous movements) in a patient, combined with a feeling that they occurred as though someone else was moving the limb. This was attributed to a lesion in the contralateral cingulate gyrus. Despite reports of unwilled movements previously existing, this appears to be the first which de- scribes the control of an outside force [26,27].
In 1945, Akelaitis  described two patients in whom tasks were reversed by the opposite hand (i.e., The first patient described putting a stocking on with her right hand only to remove it afterward with her left hand. These “opposing” actions could also occur simultaneously i.e. if using her right hand to open a door the left hand would simulta- neously push it shut. The second patient would describe repetitive re- versals of actions where he would pick up a loaf of bread with his left hand but the right hand would put it back again repetitively over a pe- riod of 5 min). Both patients described periods of being unable to pro- ceed with tasks such as walking up the stairs. These actions could be overcome but would require greater concentration and effort. Akelaitis described this as “diagnostic apraxia” which occurred after partial and complete resections of the corpus callosum, as treatment of epilepsy.
In 1972, Brion and Jedynak  used the term lamain étrangère (the foreign hand) to describe four patients who could not recognise their left hand as their own when held by their right hand behind their
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back, i.e. out of sight. These patients did not complain of any involuntary movements. The causative lesions affected the posterior corpus callosum, with possible encroachment of the surrounding parietal cor- tex being a factor.
In 1979, Bogen  ascribed “intermanual conflict” to self-opposing actions in patients with surgical callosal lesions and coined the term “alien hand” to describe the “uncooperative” hand. However in 1981 Goldberg et al.  described two patients with infarction involving the left medial frontal cortex. They exhibited purposeful movements that ap- peared to be dissociated from conscious volition, described as the “alien hand sign”. One of these patients also exhibited forced grasping of the right hand which from this point also became a recognised symptom.
There has been support to delineate the symptom of feeling one's limb as foreign from the symptom of a limb which acts at opposites to the patient's will but that is perceived as part of their own body. In 1991Della Sala et al.  proposed the latter be termed “anarchic hand”.
In 1992 Feinberg et al.  proposed two types of alien hand syn- drome based on 21 cases. Firstly “frontal alien hand syndrome”, typified by reflexive grasping, groping, and compulsive manipulation of tools by the dominant hand caused by lesions of the supplementary motor area, anterior cingulate gyrus, medial prefrontal cortex of the dominant hemisphere and anterior corpus callosum. Secondly “callosal alien hand syndrome”, typified by intermanual conflict caused by anterior callosal lesions.
This was utilised in 1999 by Chan and Liu's  finding from seven patients with anterior cerebral artery territory infarction and symptoms of “alien hand syndrome”. Four of the patients had isolated callosal midbody and isthmus lesions associated with intermanual conflict. Three patients with the medial frontal cortex and corpus callosum (genu andwhole body) extensively involved displayed amixed “frontal and callosal alien hand syndrome” showing impulsive reaching and grasping behaviours of the contralesional hand and intermanual con- flict, suggesting mixed types of “alien hand syndrome” occurring in the same patient.
In 2001 Carrilho et al.  reported that four patients, displaying involuntary, purposeless hand levitation secondary to contralateral parietal lobe involvement, should be regarded as another “alien hand syndrome”.
In 2003 Kato et al.  provided a review of a third type, namely “posterior” or “sensory alien hand syndrome”. Their patient complained of clumsiness of his left hand and later that it was controlled by some- one else. They hypothesised that the patient was unable to recognise the motion of his left hand due to the left homonymous hemianopia, left spatial neglect and left limb ataxia he suffered following acute cere- bral infarction at right posterior cerebral artery territory including right thalamus. Similar cases were caused by lesions in the basal ganglia, pons, thalamus, parietal lobe and occipital lobe [37,38].
Classically, “frontal alien hand syndrome” is reported only in domi- nant hand, and “posterior alien hand syndrome” only in non-dominant hands, whereas the “callosal” form is reported in both. However, “poste- rior alien hand syndrome” in dominant hands has beendescribed [38,39].
Aboitiz et al. , also in 2003, described a new form, “agonistic dyspraxia”, which does not fit the above classifications and so propose a five category system: (i) diagnostic dyspraxia and related syndromes, (ii) alien hand, (iii) way-ward hand and related syndromes, (iv) super- numerary hands and (v) agonistic dyspraxia (the “alien hand” executes the request when the hand that has been instructed to cannot, in a pa- tient with callosal haemorrhage).
In 2011, Yuan et al.  provided further evidence for sub-classes of “mixed alien hand syndrome” as their patient presents with intermanual conflict (“callosal type”) and left hemianaesthesia (“posterior type”) secondary to corpus callosum infarction. Further confusion arises from the presence of “mixed” signs in a patient with a parieto-occipital lesion of the dominant hemisphere but no callosal involvement .
There is no definitive explanation of why all the brain lesions previ- ously described lead to “alien hand phenomena” but one influential
theory is the dual premotor systems theory proposed by Goldberg et al. . They suggested that control of limbmovements was separat- ed into themedial premotor system for planned or “willed”movements of the contralateral limb, and the lateral premotor system for direct or “unwilled” reactions of the contralateral limb to external stimuli. It is postulated that normally these two areas coordinate movements by mutual inhibition, but a lesion of the medial premotor system would leave the contralateral limb more influenced by the lateral premotor system with predominance to “unwilled” movements and reacting to the environment rather than the “will” of the person.
McBride et al.  provide evidence for this theory by showing that involuntary grasping responses are made significantly more quickly by the alien hand and therewas no evidence of suppression of thesemove- ments in the alien limb when compared to the normal limb.
The original theory localised the medial premotor system to include the supplementary motor area and cingulated gyrus and the lateral system to include the arcuate premotor area. Also recent studies found activation of parietal areas during alien movements, such as the inferior frontal gyrus, related to inhibition control, and precuneus, relat- ed to agency, which may play a role [44,45].
Modern theories are based around an imbalance of inhibitory systems or a disconnection between areas of the brain associated with planning actions and those which perform more reflexive actions to the environment .
We believe that our patient exhibits features consistent with the “posterior alien hand syndrome”, reflected by the history and neurora- diological findings. That cerebral vasculitis was the underlying process is supported by the improvement with steroids and the significant lack of perfusion in the right temporo-parietal region showed with SPECT. However, as the patient declined a biopsy, we have been unable to prove this. On the basis of this, and the available literature about the presumed autoimmune pathogenesis of PRS [1,6,16,18,19] we used cy- clophosphamide as induction immunosuppressive therapy. As no guidelines exist, our experience in successfully treating this patient may prove useful to others. The premature cessation of therapy brought about a rapid deterioration in the clinical and MRI features, underlining the importance of maintenance immunosuppression in this situation.
In conclusionwe present the first adult formof alien hand syndrome in Parry–Romberg disease, which we believe is a form of autoimmune vasculitis. Our findings suggest that prompt and aggressive treatment with immunosuppressive agents is warranted in such cases in order to avoid permanent brain damage and long-term disability.
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