Movement Disorders Vol. 2, NO. 1, pp. 6 1 ~ 6 6 1987 , Q 1987 Movement Disorder Society Case Report Generalized Chorea Due to Basal Ganglia Lacunar Infarcts' K. D. Sethi, F. T. Nichols, *F. Yaghmai Departments of Neurology and *Neuropathology, Medical College of Georgia, Augusta, Georgia, U.S.A. Summary: Although hemichorea is often ascribed to a vascular lesion, generalized chorea of adult onset is often thought to be due to degenerative diseases. We describe a 74-year-old woman with generalized chorea that was diagnosed at autopsy as due to multiple lacunar infarcts in the basal ganglia. Generalized chorea in adults may be caused by vascular disease of the basal ganglia. Key Words: Chorea-Lacunar infarcts-Basal ganglia. Generalized chorea may be seen in a number of diseases affecting the central nervous system (CNS). Although hemichorea or hemiballism is most frequently due to a vascular lesion in the contralateral subthalamic nucleus (l), the responsible lesion may lie outside the subthalamic nuclei (2-7). Generalized chorea in adults is often attributed to degenerative disease. Thus far, acute-onset generalized chorea has only rarely been recognized to be a result of cerebrovascular disease (8,9). We report a patient with generalized chorea in whom autopsy revealed multiple small lacunar infarctions in the basal ganglia. CASE REPORT A 74-year-old woman had an abrupt onset of involuntary jerky movements affecting all extremities and the face, accompanied by slurred speech. The involuntary movements were present constantly when awake, disappeared during sleep, and worsened with agitation or with movement. The movements were severe enough to interfere with walking and self-care. * A videotape segment accompanies this article. Address correspondence and reprint requests to Dr. K. D. Sethi at Department of Neurology, Medical College of Georgia, Augusta, GA 30912, U.S.A. 61 62 K . D . SETHI ET AL. Past Medical History The patient had a 4-year history of diabetes on 20 units of NPH insulin. She had hypertension for 3 years being treated with furosemide with good control. She had never been treated with neuroleptics, metoclopramide, or reserpine. There was no history of chorea or rheumatic fever. Family history was negative for movement disorders or mental illness. Examination revealed her to be alert and oriented. She had marked slumng of speech but was not dysphasic. There was continual head jerking and involuntary facial grimacing. She had quick, arrhythmic, flowing movements characteristic of chorea, involving the trunk and extremities (see accompanying videotape). These movements were accentuated by any motor activity. In addition she had intermittent flinging movements of the legs. There were no other focal motor or sensory deficits. The following investigations were normal: CBC, SMA-18 (including calcium and phosphate), serum copper and ceruloplasmin, antinuclear antibodies, chest X-ray film, thyroid function tests, and a peripheral blood smear. The antistreptolysin 0 titer was 50 and C-reactive protein was negative. Westergren sedimentation rate was 10 mmh. Computed tomographic (CT) scan of the head (Fig. 1) revealed bilateral caudate atrophy and a possible low density area in the head of the right caudate nucleus. Treatment with chlorpromazine 25 mg. t.i.d. resulted in some improvement (see videotape). Chlorpromazine was stopped after 2 weeks and subsequently the patient continued to improve without drugs. However, she was never free of these involuntary movements for the rest of her life. There was intermittent worsening of these movements without any definite precipitating factors. Six months after the onset of chorea she had the sudden onset of left facial palsy and a mild right hemiparesis that cleared within 2 weeks. She died of cardiac causes 1 year after the onset of chorea. FIG.1. CT scan of head showing bilateral caudate atrophy and a possible low density area (arrow) on the right side. Movement Disorders, Vol. 2. No. I , 1987 CHOREA AND BASAL GANGLIA U C U N A R INFARCTS 63 FIG. 2. Gross brain specimen showing discoloration of both putamen caudate and subthalamic nuclei and to a lesser extent the globus pallidii. Autopsy Findings General autopsy findings included dilatation of the right and left ventricle of the heart, a fresh thrombus in the right coronary artery, and marked nephrosclerosis. Neuropathologically there was evidence of marked atherosclerosis and ectasia of FIG. 3. Photomicrograph from the putamen showing severe vessel wall thickening and penvascular rarefaction ( x 95). Movement Disorders, Vol. 2, No.1 , 1987 64 K . D . SETHI ET AL. FIG. 4. Photomicrograph from the caudate showing an area of eosinophilic proteinaceous fluid and small vessel changes ( x 45). the major arteries at the base of the brain. There was brownish discoloration of putamen nuclei bilaterally. The caudate nuclei showed mild atrophy and irregularity of the ventricular surface (Fig. 2). The atrophy correlated well with that seen on CT scan but no discrete lacune was seen macroscopically. Similar changes were noticed in the subthalamic nuclei. The globus pallidi were less involved. There was a small area of old cystic infarction in the left basis pontis and atrophy of the left medullary pyramid. Light microscopy revealed marked disseminated arteriolar changes in the form of hyaline atherosclerosis, onion skinning, and marked thickening of the wall of the penetrating vessels (Fig. 3). These changes were more severe in the basal ganglia than in the cerebral cortex. There was marked perivascular rarefaction of tissues in the involved areas of the basal ganglia. There were areas of eosinophilic proteinaceous fluid around and near the penetrating vessels (Fig. 4). In addition, areas of marked fibrillary astrocytosis were noticed in both caudate and putamen nuclei bilaterally. Subthalamic nuclei were less involved bilaterally. The globus pallidus was relatively spared from these changes. The substantia nigra was normal bilaterally. The pontine lesion was confirmed to be an old infarction with cystic change and infrtration of macrophages (Fig. 5). DISCUSSION Generalized chorea in adults may be caused by drugs (including antipsychotic drugs, phenytoin, metoclopramide), systemic lupus erythematosus, neuroacanth- Movement Disorders. Vol. 2, No. I , 1987 CHOREA AND BASAL GANGLIA LACUNAR INFARCTS 65 FIG. 5. Pontine lacunar infarct with infiltration of macrophages ( x 95). ocytosis, thyrotoxicosis, polycythemia Vera, chorea gravidarum, and Sydenham’s chorea. Other causes are Huntington’s disease and senile chorea. There has been only one reported case of .acute-onset generalized chorea with autopsy-proven vascular disease of the basal ganglia (8). The distinction between chorea and ballism, however, is somewhat arbitrary and some of these cases may have been reported as paraballism (bilateral ballism) (10,Il). The patient we describe developed generalized acute chorea. Autopsy revealed mutiple lacunar infarcts bilaterally in the caudates, putamina, and subthalamic nuclei with relative sparing of the globus pallidi. Hemiballism is thought to be due to a release of inhibitory input into the globus pallidus from other parts of the basal ganglia (12). This is supported by the observation of animal models of hemichorea, where destruction of globus pallidus results in disappearance of choreoid hyperkinesia (13). It is possible that a similar disinhibition of globus pallidi was operative bilaterally in our case. Generalized chorea due to lacunar strokes may be more common, especially in the elderly, than is currently believed. We believe that more sophisticated imaging techniques such as magnetic resonance imaging will help diagnose these cases during life (9). LEGENDS TO THE VIDEOTAPE SEGMENT 1. Patient exhibiting generalized chorea, facial grimacing and dysarthria. SEGMENT 2. Two weeks later. Note improvement in chorea. Movement Disorders, Vol. 2, No. 1. 1987 66 K . D . SETHI ET AL. REFERENCES 1 . Meyers R . Ballismus. In: Vinken PJ, Bruyn GW, eds. Handbook of clinical neurology, vol. 6 . Amsterdam: North Holland, 1968:76-90. 2. Martin JP. Hemichorea (hemiballism) without lesion in the corpus luysii. Brain 1957;8O:L-10. 3. Saris S . Chorea caused by caudate infarction. Arch Neurol 1983;40:590-1. 4. Kase CS, Maulsby GO, deJaun E, Mohr JP. Hemichorea-hemiballism and lacunar infarction in the basal ganglia. Neurology (NY) 1981;31:452-5. 5. Goldblatt D, Markesbery W, Reeves AG. Recurrent hemichorea following striatal lesion. Arch Neurol 1974;31:51-4. 6 . Davison C. Goodhart SP. Monochorea and somotovic localization. Arch Neurol Psychiatry 1940;43:792-803. 7. Schwarz GA, Barrow LJ. Hemiballism without involvement of Luy’s body. Arch Neurol 1960:2:420-34. 8. Folstein S, Abbott M,Moses R, Parlad I, Clark A, Folstein M. A phenocopy of Huntington’s disease: lacunar infarcts of the corpus striatum. Johns Hopkins Med J 1981;148:104-13. 9 . Tababton M, Mancardi G, Loeb C. Generalized chorea due to bilateral small deep cerebral infarcts. Neurology 1985;35:588-9. 10. Inbody S , Jankovic J. Hyperkinetic mutism: bilateral ballism and basal ganglia calcification. Neurology 1986;36:825-7. 11. Loddar J, Baard WC. Paraballism caused by bilateral hemorrhagic infarction in the basal ganglia. Neurology ( N Y) 198 1;3 1:484-5. 12. Johnson WF, Fahn S. Treatment of vascular hemiballism and hemichorea. Neurology (Minneap) 1977;27:634-6. 13. Carpenter MB, Whittier JR, Mettler FA. Analysis of choreoid hyperkinesia in the rhesus monkey: surgical and pharmacological analyses of hyperkinesia resulting from lesions in the subthalamic nucleus of Luys. J Comp Neurol1950;92:293-331. Movement Disorders, Vol. 2, No. 1 , 1987