by Kozlowski et a12 and of our work is that heterotopic neurons appear resistant to the degenerative process that affects motor neurons in ALS, while other spinocerebellar neurons a r e frequently involved in the disease.E-10 Averback and Crockera found t h a t more t h a n one-third of neurons in Clarke’s nucleus were lost in patients dying with ALS, a finding confirmed by Williams et allo in a study using a monoclonal marker f o r neurons together with another monoclonal antibody that reacted more specifically for a surface marker on spinocerebellar neurons in Clarke’s dorsal nucleus and in the neurons of the cervical spinocerebellar and ventral spinocerebellar tracts. The lack of involvement of neurons located in the white matter of the spinal cord in ALS, SMA, and poliomyelitis suggests that these neurons are not part of the motor or spinocerebellar systems and that they may be sensory neurons, despite their frequent location in the ventral part of the white matter of the cord. Acknowledgments We t h a n k E n i d Henessey of t h e D e p a r t m e n t of Medical Statistics, The Royal London Hospital, for her help, and the patients and staff of St. Christophers Hospice, Sydenham, for their interest in our work. From the Department of Neuropathology, Institute of Pathology, The Royal London Hospital, Whitechapel, London, UK. Supported by the Motor Neuron Disease Association of Great Britain. J.E.M. is a Wellcome Trust Fellow and holds the Gillson Scholarship in Pathology of the Worshipful Society of Apothecaries. Hemiballismusand secondary mania following a right thalamic infarction Received August 3, 1992. Accepted for publication in final form November 13, 1992. Address correspondence a n d r e p r i n t r e q u e s t s t o Dr. J . E . M a r t i n , Department of Neuropathology, Institute of Pathology, The Royal London Hospital, Whitechapel, London E l lBB, UK. References 1. Mitsumoto H, Hanson MR, Chad DA. Amyotrophic lateral sclerosis. Arch Neurol 1988;45:189-202. 2. Kozlowski M A , Williams C, H i n t o n DR, Miller CA. Heterotopic neurons in spinal cord of patients with ALS. Neurology 1989;39:644-648. 3. Leigh PN, Anderton BH, Dodson A, GalIo J-M, Swash M, Power DM. Ubiquitin deposits i n anterior horn cells in motor neuron disease. Neurosci Lett 1988;93:197-203. 4. Lowe J, Lennox G , Jefferson D, e t al. A filamentous inclusion body within anterior horn neurons in motor neuron disease defined by immunocytochemical localisation of ubiquitin. Neurosci Lett 1988;93:203-210. 5 . Irving D, Rebeiz JJ, Tomlinson BE. The numbers of limb motor neurones in the individual segments of the human lumbosacral spinal cord. J Neurol Sci 1974;21:203-212. 6. Swash M, Leader M, Brown A, Swettenham KW. Focal loss of anterior horn cells in the cervical cord in motor neuron disease. Brain 1986;109:939-952. 7. Cooper S, Sherrington CS. Gower’s tract and spinal border cells. Brain 1940;63:123-134. 8. Averback P, Crocker P. Regular involvement of Clarke’s nucleus i n sporadic amyotrophic lateral sclerosis. Arch Neurol 1982;39:155-156. 9. Swash M, Scholtz CL, Vowles G, Ingram DA. Selective and asymmetric vulnerability of corticospinal and spinocerebellar tracts i n motor neuron disease. J Neurol Neurosurg Psychiatry 1988;51:785-789. 10. Williams C, Kozlowski MA, H i n t o n DR, Miller CA. Degeneration of spinocerebellar neurons in amyotrophic lateral sclerosis. Ann Neurol 1990;27:215-225. A r t i c l e abstract-An 81-year-old woman had the sudden onset of leftsided ballismus and a n accompanying behavioral change characterized by elation, distractibility, inflated self-esteem, and insomnia, suggesting secondary mania. An MRI revealed a small ischemic infarction of the right thalamus. NEUROLOGY 1993;43:1422-1424 Jaime Kulisevsky, MD; Marcel0 L. Berthier, MD; and Jesus Pujol, MD There are only occasional reports of mental changes associated with hemiballismus. We describe a patient who presented with acute hemiballismus and a disinhibition syndrome mimicking mania after a small right thalamic infarction. Case report. An 81-year-old, right-handed woman suddenly developed involuntary movements of the left limbs. During the next 3 days, her relatives noted a marked c h a n g e i n h e r behavior. S h e became i n c r e a s i n g l y euphoric and talkative, and had grandiose delusions. She was admitted to hospital on the fourth day. She had diabetes and well-controlled hypertension but no familial or personal history of affective disorders. On hospital admission, she was globally oriented, but appeared distractible and had pressured speech and 1422 NEUROLOGY 43 July 1993 flight of ideas. She believed that her health was better than ever and joked inappropriately. She also reported a decreased need for sleep. Her mood changes, which were assessed with the Structured Clinical Interview from DSM-III-R,2 met criteria for an organic affective disorder, manic type. On a mania rating scale,3 her mania score was 15 points (definite mania). She scored 26 out of 30 on t h e Mini-Mental S t a t e E ~ a m i n a t i o nOn . ~ neurologic examination, she showed choreic and ballistic movements of the left limbs. There were almost-constant, smallamplitude, proximal and distal choreic movements, with superimposed brisk, large-amplitude proximal movements, primarily related to action. Sensation, strength, and tendon reflexes were normal. Plantar responses were flexor bilaterally. Ocular motility and visual fields were normal, and there was no evidence of visual neglect on a Figure. T,-weighted MRI (TR = 2,000; TE = 40) showing a hypointense thalamic lesion (arrows) at the level of the right ventral lateral and ventromedial nuclei in (panel a) the sagittal (A = anterior) and (panel b) the coronal planes (R = right). line crossing-out task. An MRI with 5-mm sections revealed a small area of hypointense TI signal in the right thalamus (figure).The lesion involved the ventral lateral and ventromedial nuclei but spared the cerebral peduncle and midbrain. The patient was started on haloperidol (15 mg/d), a n d the motor and behavioral disturbances improved by the third day. Affective disturbances subsided 1 month after the onset, and haloperidol was then gradually discontinued. At 3 months follow-up, the patient remained euthymic, but she still showed mild choreic movements of the left limbs. Discussion. Although most cases of hemiballismus result from small vascular lesions involving the contralateral subthalamic nucleus or its projection target areas, lesions of the contralateral thalamus may produce similar motor ~ y r n p t o m s There .~ are occasional reports of mental changes in patients with hemiballismus, particularly with lesions extending beyond the subthalamic nucleus.’I5 In these cases, hemiballismus and behavioral changes generally coexisted with autonomic and sensory symptoms and altered strength, muscle tone, stretch reflexes, or other clinical features suggesting large lesions. Our case is unique in that hemiballismus and secondary mania occurred without concomitant neurologic manifestations, and the responsible lesion was small and restricted to the right thalamus. Secondary mania after thalamic damage was reported occasionally in association with ischemic and hemorrhagic lesions, neoplasms, sarcoidosis, stereotactic thalamotomy, Kleine-Levin syndrome, o r a s a postoperative complication in coronary artery bypass surgery.6--8The secondary mania after a right thalamic lesion is in accord with previous reports implicating dysfunction of limbic and limbic-connected a r e a s ( i e , orbitofrontal o r basotemporal cortices, caudate nucleus, and thalamus) of the right hemisphere as a cause of organic mania.6.7The right thalamic infarction may have selectively disrupted the convergence of limbic and subthalamic pathways t o various thalamic nuclei as well as the thalamocortical connectivity. The coexistence of mania and movement disorders was reported in a number of basal ganglia diso r d e r s , specifically Huntington’s d i s e a s e , Sydenham’s chorea, postencephalitic parkinsonism, Wilson’s disease, and Tourette’s s y n d r ~ m e While .~,~ episodes of m a n i a a r e relatively f r e q u e n t i n patients with hyperkinetic movement disorders such a s e a r l y Huntington’s disease, t h e y a r e uncommon in patients with hypokinetic movement disorders s u c h a s P a r k i n s o n ’ s disease. I n Parkinson’s disease, manic states a n d ballistic movements similar t o those seen in our patient were most often reported in patients with levodopainduced “on-off” motor fluctuations. I n t h e s e patients, manic swings and dyskinetic movements mainly occurred during “on” periods a n d were attributed to striatal and limbic dopaminergic overactivity.I0 An analogous dopaminergic overactivity is postulated in Tourette’s syndrome, a condition in which a disinhibition syndrome is frequently seen.g Therefore, it is possible that a similar biochemical mechanism could be implicated in the pathogenesis of mania and hemiballismus in our patient. In support, haloperidol, a postsynaptic D,-receptor antagonist, resulted in suppression of mania and hyperkinetic movements. Our patient’s improvement with haloperidol is consistent with the literature7 in which reported cases with secondary mania had good response to treatment with the usual antimanic drugs-lithium or neuroleptics. Perhaps prompt treatment of hemiballismus patients with neuroleptic therapy masks accompanying behavioral changes, such as mania. July 1993 NEUROLOGY 43 1423 ~ From the Department of Neurology (Drs. Kulisevsky and Pujol), Sant Pau Hospital, Autonomous University of Barcelona, and Virgen de la Victoria University Hospital (Dr. Berthier), Malaga, Spain. Received August 17, 1992. Accepted for publication i n final form November 3, 1992. Address correspondence and reprint requests to Dr. Jaime Kulisevsky, Servicio de Neurologia, Hospital Sant Pau, Sant Antoni M. Claret, 167, 08025 Barcelona, Spain. References 1. Barraquer-Bordas L, Peres-Serra J . Sindrome subtalamico cerebelo-extrapiramidal oculosimpatico acompafiado de insomnio y de expansion del humor. Arch Neurobiol (Madr) 1965;28:409-413. 2. SDitzer RL. Williams JBW. Gibbon M. First MB. Structured clkical interview for DSM-111-R-patient edition (SCID-PI, version 1.0. Washington, DC: American Psychiatric Press, 1990. 3. Bech P, Kastrup M, Rafaelsen OJ. Mini-compendium of rating scales for states of anxiety, depression, mania, and ~ Familial band heterotopias simulating tuberous sclerosis schizophrenia, with corresponding DSM-I11 syndromes. Acta Psychiatr Scand 1986;73(suppl236):29-31. 4. Folstein MF, Folstein SE, McHugh PR. “Mini Mental State”: a practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res 1975;12:189-198. 5 . Buruma OJS, Lakke JPWF. Ballism. In: Vinken PJ, Bruyn GW, Klawans HL, eds. Handbook of clinical neurology, vol5. Amsterdam: Elsevier Science Publishers, 1986:369-380. 6. Starkstein SE, Mayberg HS, Berthier ML, e t al. Mania after brain injury: neuroradiological and metabolic findings. Ann Neurol 1990;27:652-659. 7. Starkstein SE, Boston JD, Robinson RG. Mechanisms of mania after brain injury: 12 cases and review of the literature. J Nerv Ment Dis 1988;176:87-100. 8. Cummings JL, Mendez MF. Secondary mania with focal cerebrovascular lesions. Am J Psychiatry 1984;141:10841087. 9. Comings DE. A controlled study of Tourette syndrome. VII. Summary: a common genetic disorder causing disinhibition of the limbic system. Am J Hum Genet 1987;41:839-866. 10. Menza MA, Sage J, Marshall E, Cody R, Duvoisin R. Mood changes and “on-off” phenomena in Parkinson’s disease. Mov Disord 1990;2:148-151. Article abstract-We report the clinical and neuroimaging findings of a mother and daughter with seizure disorders and band heterotopias seen on magnetic resonance imaging studies. These clinicoradiologic findings simulate those for a diagnosis of tuberous sclerosis complex. Clinicians should be aware of this migrational anomaly and its neuroimaging characteristics, as well as the potential for this specific migrational anomaly to be genetically transmitted. NEUROLOGY 1993;43:1424-1426 Francis J. DiMario, Jr., MD; Richard J. Cobb, MD; Gale R. Ramsby, MD; and Carol Leicher, MD MRI allows t h e appreciation of more detailed in vivo neuroanatomy. A number of neuronal migrational abnormalities have become more easily recognized as compared with conventional CT scanning.1-3A recently recognized migrational anomaly, band heterotopias, bears a resemblance to t h e s u b e p e n d y m a l n o d u l e s ( S E N ), a m i g r a t i o n a l anomaly seen in patients with tuberous sclerosis complex (TSC).4-6 We report a 16-year-old patient and her mother, both with band heterotopias, and describe how this syndrome is distinguishable from TSC and SEN. Case report. A 16-year-old woman of Native American and German extraction with a mild seizure disorder was referred to the neurogenetics clinic at the University of Connecticut for evaluation of possible tuberous sclerosis. Referral was prompted by the recognition of periventricular heterotopias on the head CT. She was born after a n uneventful pregnancy at full term, attaining appropriate developmental milestones. S h e h a d a first and second generalized tonic-clonic seizure associated with fever between ages 3 and 5 years. Neurologic and general examinations as well a s a n EEG a t that time were normal. A head CT reportedly showed enlarged ventricles. Intermittent phenobarbital was administered but not maintained. At age 10, she experienced her first nonfebrile generalized seizure. A repeat EEG demonstrated bicentral paroxysmal activity with 1424 NEUROLOGY 43 July 1993 right-sided predominance and generalized paroxysmal discharges during sleep. A head CT at this time demonstrated enlarged ventricles and periventricular irregulari t i e s subsequently defined a s heterotopias. Carbamazepine was prescribed after a second nonfebrile, complex partial seizure. The frequency of seizures was limited to isolated yearly complex partial events during the first 3 years of therapy and remitted over the past 2 years. She remains a n excellent student. Her past history is otherwise unremarkable. The parents denied consanguinity. The patient’s 35-year-old mother was diagnosed with complex partial seizures at age 21 after a n evaluation for recurrent episodes of right arm numbness, aphasia, and altered consciousness. Her head CT was normal, and she was eventually treated with carbamazepine and phenobarbital. Seizure frequency is about twice per year. The maternal grandmother, now deceased, also had epilepsy requiring therapy with phenytoin. Details of her clinical examination are lacking, but she did not have renal problems or obvious facial angiofibroma. The remainder of the family history was noncontributory. On examination under Wood‘s lamp, the patient had a difficult-to-recognize single ill-defined hypopigmented macule and a small caf6 a u lait spot on the right flank. There were no facial angiofibromas, poliosis, confetti lesions, gingival or ungual fibromas, connective tissue nevi, collagen plaques, or dental pits. Dilated funduscopy was normal. The remainder of her general and neurologic examinations were normal. Subsequent brain MRI Hemiballismus and secondary mania following a right thalamic infarction Jaime Kulisevsky, Marcelo L. Berthier and Jesús Pujol Neurology 1993;43;1422 DOI 10.1212/WNL.43.7.1422 This information is current as of July 1, 1993 Updated Information & Services including high resolution figures, can be found at: http://www.neurology.org/content/43/7/1422.full.html Citations This article has been cited by 2 HighWire-hosted articles: http://www.neurology.org/content/43/7/1422.full.html##otherarticles Permissions & Licensing Information about reproducing this article in parts (figures,tables) or in its entirety can be found online at: http://www.neurology.org/misc/about.xhtml#permissions Reprints Information about ordering reprints can be found online: http://www.neurology.org/misc/addir.xhtml#reprintsus Neurology ® is the official journal of the American Academy of Neurology. Published continuously since 1951, it is now a weekly with 48 issues per year. Copyright © 1993 by the American Academy of Neurology. All rights reserved. Print ISSN: 0028-3878. Online ISSN: 1526-632X.