Un caso del cuerpo calloso y mano ajena, Monografías de Neurociencia. Universidad Autónoma Metropolitana
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Un caso del cuerpo calloso y mano ajena, Monografías de Neurociencia. Universidad Autónoma Metropolitana

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Aticulo cientifico, el sindrome de la mano extraña y una lesión en el cuerpo cayoso
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This article was downloaded by: [Universidad Autonoma Metropolitana] On: 31 May 2015, At: 23:03 Publisher: Routledge Informa Ltd Registered in England and Wales Registered Number: 1072954 Registered office: Mortimer House, 37-41 Mortimer Street, London W1T 3JH, UK

Neurocase: The Neural Basis of Cognition Publication details, including instructions for authors and subscription information: http://www.tandfonline.com/loi/nncs20

A case of the corpus callosum and alien hand syndrome from a discrete paracallosal lesion Raymond Faber a b c , Alvi Azad c d & Richard Reinsvold d a Laurel Ridge Treatment Center , San Antonio, TX, USA b South Texas Veterans Health Care System , San Antonio, TX, USA c University of Texas Health Science Center at San Antonio , TX, USA d Wilford Hall Medical Center, Lackland Air Force Base , San Antonio, TX, USA Published online: 11 Jan 2010.

To cite this article: Raymond Faber , Alvi Azad & Richard Reinsvold (2010) A case of the corpus callosum and alien hand syndrome from a discrete paracallosal lesion, Neurocase: The Neural Basis of Cognition, 16:4, 281-285, DOI: 10.1080/13554790903456217

To link to this article: http://dx.doi.org/10.1080/13554790903456217

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NEUROCASE 2010, 16 (4), 281–285

© 2010 Psychology Press, an imprint of the Taylor & Francis Group, an Informa business

http://www.psypress.com/neurocase DOI: 10.1080/13554790903456217

NNCSA case of the corpus callosum and alien hand syndrome from a discrete paracallosal lesion

Corpus Callosum and Alien Hand Syndrome Raymond Faber,1,2,3 Alvi Azad,3,4 and Richard Reinsvold4

1Laurel Ridge Treatment Center, San Antonio, TX, USA 2South Texas Veterans Health Care System, San Antonio, TX, USA 3University of Texas Health Science Center at San Antonio, TX, USA 4Wilford Hall Medical Center, Lackland Air Force Base, San Antonio, TX, USA

Here we present a patient with an isolated paracallosal brain lesion who exhibited behavioral changes associated with the corpus callosum syndrome (CCS) including features of the alien hand syndrome (AHS). The CCS is also known as the split-brain syndrome, the syndrome of hemisphere disconnection, the syndrome of brain bisection and the syndrome of the cerebral commissures. Because most reported cases of CCS were caused by tumors which extended beyond the corpus callosum (CC) and did not always induce a complete disconnection, there was much controversy about the role of the CC and the existence of a specific CCS. Aside from surgically based cases, the full complement of the CCS is infrequently clinically encountered. The patient described has a classic CCS from natural causes. This case report is unique in exhibiting a complete CCS with AHS secondary to an ischemic event affecting the left pericallosal region. To our knowledge this is the first case report of such a combination.

Keywords: Split-brain syndrome; Syndrome of hemisphere disconnection; Paracallosal lesion; Apraxia; Agraphia.

INTRODUCTION

We describe a patient with an isolated paracallosal brain lesion who exhibited behavioral changes associated with the corpus callosum syndrome (CCS) including features of the alien hand syndrome (AHS). The CCS is also known as the split-brain syndrome, the syndrome of hemi- sphere disconnection, the syndrome of brain bisection and the syndrome of the cerebral commissures (Bogen, 1979). Liepmann (1900) and Maas (Van Vleuten, 1907) are credited with the first case report of CCS with a callosal lesion causing left hand apraxia and agraphia. Because most reported cases of CCS were caused by tumors which extended beyond the corpus callosum (CC) and did not always induce a com- plete disconnection, there was much controversy

about the role of the CC and the existence of a specific CCS. Even patients who had surgical sectioning of the callosum were said to not neces- sarily display the CCS (Akelaitis, 1944). Fuller descriptions of the CCS were published in the 1940s following intentional surgical severing of the CC performed for uncontrolled epilepsy (Van Wagenen & Herren, 1940). The contemporary understanding of the role of the CC and the components of the CCS derive from the work of Roger Sperry who was awarded the Nobel Prize for this work (Sperry, 1961). Aside from surgi- cally based cases, the full complement of the CCS is infrequently clinically encountered. We describe a patient who had a classic CCS from natural causes. His symptoms included left hand apraxia, left hand tactile agnosia, left hand agraphia, impaired cross-replication of hand

Address correspondence to Raymond Faber, MD, 7400 Merton Minter Blvd. 116-A, San Antonio, TX, 78229, USA. (E-mail: [email protected]).

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282 FABER, AZAD, REINSVOLD

postures, right hand constructional apraxia, inter- manual conflict, and anarchic (alien) hand syn- drome.

CASE REPORT

P.P. was a 56-year-old Caucasian man referred by the Occupational Therapy service of a Veterans Affairs medical center because of his lack of progress in improving his ability to perform bimanual activities in spite of good exercise capability, no focal gross motor or sensory defi- cits, and intact comprehension. His past medical history was significant for hypertension, atrial fibrillation, retinitis pigmentosa (legally blind in his right eye), Peyronie’s disease, Barrett’s esophagus, colonic polyps, peripheral vascular disease (status post graft placement), and cerebral vascular disease. His substance use history was significant for 60+ pack years of smoking, and occasional alcohol use. He had no history of psychiatric illness.

He was married twice, lived with his second wife, and had three children. He retired from the Air Force after a 20-year career and worked as a gunsmith and machinist until he retired at age 54. He enjoyed reading and watching sports on TV. When he was 46 years old he had a transient ischemic attack with a normal CT and minimal carotid disease on arteriogram; later in the same year he had an apparent stroke with right-sided paresis and only minimal effect on his speech. Post-stroke he became verbally abusive, developed marked difficulty with short-term memory, tended to perseverate, and he escalated his alcohol consumption. However, he was able to return to work. By age 50 he had several more TIA’s and developed severe peripheral claudica- tion. An arteriogram showed severe left carotid stenosis and prominent stenosis of the right renal, bilateral iliac and femoral arteries. Multiple peripheral bypass grafts were placed. At age 54 following a thrombectomy on a right axil- lofemoral graft, he developed a right hemiplegia and Broca’s aphasia. During this period, he experienced marked positive personality changes, becoming very affectionate and happy, while his short-term memory continued to deteriorate. Five days after this stroke he developed involuntary movements. He reported his ‘… right hand would slap and stick to his forehead until my left hand would peel it away’. He also reported developing

impaired bimanual coordination, for example tying his shoes, buttoning his shirt, eating and driving his car. His right hand would remove a utensil from his mouth, so he had to use his left hand to keep utensils in place. He reported after placing a car key in the ignition, his right hand would remove it, and his left hand would counter by replacing it. His wife said unwanted actions of right hand would be less prominent if she ‘made a joke of it around him’. He also had difficulties turning over in bed, coughing, and blowing his nose.

NEUROBEHAVIORAL ASSESSMENT

The patient was always quite cooperative. He never faltered in attempting to perform all tasks asked of him. He smiled broadly and made good eye contact. He was, in fact, quite engaging. He responded with pertinent answers and was apol- ogetic when he could not answer or perform correctly. He was never circumstantial or tan- gential in his speech. He maintained a consistent, stable euthymic mood without any trace of depression, anxiety, or dysphoria. He had excel- lent long-term memory in discussing his medical problems, family relationships, employment history, and recreational interests. He denied experiencing psychotic symptoms. He denied having obsessions or compulsions. He denied any thoughts of harming himself or others. He had fair but limited insight into his difficulties. In acknowledging and apologizing for social miscues, he displayed fair insight and good judgment.

He freely discussed instances of having inter- manual conflict. This included aforementioned difficulties with tying his shoes, using utensils, and keeping the car keys in the ignition. At times when asked to place a piece of paper in an enve- lope, he would correctly fold the paper and insert it but then almost immediately remove it. He admitted this removal was at odds with his conscious intentions. At a prior recent hospitali- zation, he was reprimanded on several occasions when his right hand reached out and grabbed the derriere of nearby female nurses. When confronted with this, he blushed and acknowl- edged doing this repeatedly but insisted he had absolutely no intention of acting in this manner. He expressed regret and empathy for the women he had offended.

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CORPUS CALLOSUM AND ALIEN HAND SYNDROME 283

In systematic testing with his vision blocked, he could not name any object placed in his left hand. Though he could grasp, balance and manipulate what was placed in his left hand, he often said there was nothing in it when asked to name the object. Without being aware of the object he was unable to draw or write the name of the object. When the same object was placed in his right hand, he would immediately name it correctly. After having different objects placed into his right or his left hand, he consistently pointed to the correct object from an array of multiple choices when using the same hand in which the object had been placed, in spite of not being aware of the object when it was placed in his left hand. However, when he was asked to point to the object placed in his contralateral hand, right or left, he would say ‘I don’t know’ or none in the array had been in his hand. With his right hand, he was very adept in demonstrat- ing how to use or manipulate a wide array of objects or do things like salute, wave goodbye, hitchhike, throw a kiss, and make the sign of the cross. With his left hand, he could not perform any of these gestures. He would move his left hand, raise it, and at best make an approximate attempt such as rubbing his forehead when asked to touch his left ear. Similarly, with his vision blocked, he could not replicate with either hand the position of one hand with some fingers extended and some folded. His handwriting with his left hand was far worse than expected of a right-hander. Copies of drawings using his right hand were judged to be poor, though no worse than those done with his left hand.

IMAGING STUDIES

Other imaging studies included an echocardiogram at age 54 which showed a normal left ventricle and left atrium, and mild mitral and mild tricuspid insufficiency. He was treated with warfarin beginning at age 46 but it was discontinued when he was 55 because of ongoing fall risk. He had arterial imag- ing studies done the same year which showed severe superior mesenteric artery stenosis with occluded internal mammary artery, severe left renal and moderate right renal stenosis, right aor- tobifemoral graft occlusion and a patent femoral to femoral bypass graft. A brain MRI (Figures 1–6) was also performed when he was 55 and showed a slit-like left paracallosal lesion extending from the

genu towards the splenium of the CC. This was interpreted as an infarct affecting the left perical- losal artery or possibly a watershed infarct in the same region.

Figure 1. Sagital T1 image showing lucency superior to anterior portion to left edge of CC.

Figure 2. Sagital T1 image showing lucency slightly lateral to Figure 1 to the left of the central portion of the CC.

Figure 3. Coronal T1 showing lucency extending superior from enlarged left ventricle along side of the CC.

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284 FABER, AZAD, REINSVOLD

DISCUSSION

Complete callosal syndromes as seen in this case report are quite rare. Out of 282 consecutive ischemic stokes, eight cases with callosal lesions were identified. Five of these had incomplete

callosal syndromes with left ideomotor apraxia, constructional apraxia, and left-hand agraphia. Two patients had alien hand phenomena (Giroud & Dumas, 1995). A series of 100 consecutive anterior cerebral artery infarctions found no exam- ples of the callosal syndrome in spite of frequent involvement of the corpus callosum (Kang & Kim, 2008). Two cases of classic AHS with intermanual conflict but without other signs of the CCS were reported secondary to infarction of the pericallosal artery (Suwanwela & Leelacheavasit, 2002). A case of classic CCS but without AHS was reported secondary to a left periventricular lesion adjacent to the posterior half of the corpus callosum and partially destroying the splenium (Habib, Ceccaldi, & Poncet, 1990). Our patient may be the first reported with complete CCS and AHS from natural causes.

The term AHS has a complex history and has recently taken on a variety of definitions in the lit- erature. We define it as a phenomenon of purpose- ful but unintentional activity of an arm at odds with what the patient wishes the arm to do. The subject may disavow this activity, which sometimes acts at cross-purposes with the other arm. The term ‘anarchic’ hand syndrome better fits our patient as he did not deny ownership of his hand while experiencing his alien hand symptoms (Della Sala, Marchetti, & Spinnler, 1991). At times he also clearly displayed ‘intermanual conflict’.

P.P. displays the ‘classic’ AHS first described in 1897 by Kaiser (Schuster, 1923) as well as complete CCS. AHS has taken on evolving definitions. As corpus callosectomy became an increasingly used technique to control refractory seizures, reports of this self oppositional behavior became increasingly noted (Van Wagenen & Herren, 1940). Denny- Brown expanded upon the notion of corpus callosal involvement as well as other brain regions in experimental work with monkeys and correlative observations in humans (Denny-Brown, 1956, 1958). He found that frontal injury, particularly in the supplemental motor area (SMA), resulted in an involuntary contralateral grasping reflex to a stim- ulus. He also reported rare instances of parietal lobe injury causing patients to extend their fingers when approached with a stimulus. The term alien hand syndrome is a translation from the French ‘le signe de la main etrangere’ (Brion & Jedynak, 1972). Recent decades have seen an emergence of different varieties of AHS that can be correlated with different locations of cerebral lesions from a broad spectrum of etiologies to include stroke

Figure 4. Similar but more anterior to Figure 3.

Figure 5. Axial T1 showing left paracallosal lucency.

Figure 6. Axial T2 showing left paracallosal lesion.

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CORPUS CALLOSUM AND ALIEN HAND SYNDROME 285

limited to the middle and posterior portions of the body of the corpus callosum (Geschwind et al., 1995), partial status epilepticus (Feinberg, Roane, & Cohen, 1998), cortical-basal degeneration (Riley, 1990), and toxoplasmosis (Caixeta et al., 2007).

CONCLUSIONS

We believe the case reported is unique in exhibiting a complete CCS with AHS secondary to an ischemic event affecting the left pericallosal region. To our knowledge this is the first case report of such a combination.

Original manuscript received 4 August 2009 Revised manuscript accepted 1 October 2009

First published online 11 January 2010

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Akelaitis, A. J. (1945). Studies on the corpus callosum IV: Diagnostic dyspraxia in epileptics following partial and complete section of the corpus callosum. American Journal of Psychiatry,101, 594–599.

Bogen, J. E. (1979). The callosal syndrome. In K. M. Heilman & E. Valenstein. (Eds.), Clinical neuropsy- chology (1st ed., pp. 308–359). New York: Oxford University Press.

Brion, S., & Jedynak, C. P. (1972). Troubles du transfert interhemispherique (callosal disconnection). A propos de trios observations de tumeurs du corps calleux. Le signe de la main etrangere. Revue Neurologique (Paris),126, 257–266.

Caixeta, L., Maciel, P., Nunes, J., Nazareno, L., Araujo, L., & Borges, J. R. (2007). Alien hand syndrome in AIDS. Dementia & Neuropsychalgia,1(4), 418–421.

Della Sala, S., Marchetti, C., & Spinnler, H. (1991). Right-sided anarchic (alien) hand: A longitudinal study. Neuropsychologia, 29, 1113–1127.

Denny-Brown, D. (1956). Positive and negative aspects of cerebral cortical functions. North Carolina Medical Journal,17, 295–303.

Denny-Brown, D. (1958). The nature of apraxia. Journal of Nervous and Mental Disease,126, 9–32.

Feinberg, T. E., Roane, D. M., & Cohen, J. (1998). Par- tial status epilepticus associated with asomatagnosia and alien hand-like behaviors. Archives of Neurology, 55, 1574–1676.

Geschwind, D. H., Jacoboni, M., Mega, M. S., Zaidel, D. W., Cloughesy, T., & Zaidel, E. (1995). Alien hand syndrome: Interhemispheric motor disconnection due to a lesion in the midbody of the corpus callosum. Neurology,45, 802–808.

Giroud, M., & Dumas, R. (1995). Clinical and topo- graphical range of callosal infarction: A clinical and radiological correlation study. Journal of Neurology, Neurosurgery and Psychiatry,59(3), 238–242.

Habib, M., Ceccaldi, M., & Poncet, M. (1990). Callosal disconnection syndrome caused by left hemisphere infarction. Revue Neurologique (Paris),146(1), 19–24.

Kang, S. Y., & Jong. K. S. (2008). Anterior cerebral artery infarction: Stroke mechanism and clinical- imaging study in 100 patients. Neurology,10(70), 2386–2393.

Liepmann, H. (1900). Der krankheit der apraxie, motorischen asymbolie. Monatsschr Psychiatrie und Neurologie,11, 15–44,102–132, & 182–197.

Riley, D. E., Lang, A. E., Lewis, A., et al. (1990). Corti- cal-basal ganglionic degeneration. Neurology, 40, 1203–1212.

Schuster, P. (1923). Zwangsgreifen und Nachgreifen, zwei posthemiplegische Bewegungstorungen. Zeitschrift fur die gesamte. Neurologie und Psychiatrre,81, 586–609.

Suwanwela, N. C., & Leelacheavasit, N. (2002). Isolated corpus callosal infarction secondary to pericallosal artery disease presenting as alien hand syndrome. Journal of Neurology, Neurosurgery and Psychiatry, 72(4), 533–536.

Sperry, R.W. (1961). Cerebral organization and behavior. Science,133, 1749–1757.

Van Vleuten, C.F. (1907) Linksseitige motorische Apraxie. Ein beitrag zur physiologie des balkens. Allgemeine Zeitschrift fur Psychiatrie,64, 203–239.

Van Wagenen, W. P., & Herren, R. Y. (1940). Surgical divi- sion of commisural pathways in the corpus callosum. Archives of Neurology and Psychiatry,44, 740–59.D

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