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Home , Athetosis, Hemiballismus, Movement disorder

Radiologic-Clinical Correlation

James M. Provenzale and Michael A. Schwarzschild

From the Departments of Radiology (J.M.P.), Duke University Medical Center, Durham, and f'leurology (M.A.S.), Massachusetts General Hospital, Boston

Clinical History derived from the Greek word meaning "to A 65-year-old recently retired surgeon in throw," because the typical involuntary good health developed disinhibited behavior movements of the affected limbs resemble over the course of a few months, followed by the motions of throwing ( 1) . Such move­ onset of unintentional, forceful flinging move­ ments usually involve one side of the body ments of his right arm and leg. Magnetic res­ (hemiballismus) but may involve one ex­ onance imaging demonstrated a 1-cm rim­ tremity (monoballism), both legs (parabal­ enhancing mass in the left subthalamic lism), or all the extremities (biballism) (2, 3). region, which was of high signal intensity on The motions are centered around the shoul­ T2-weighted images (Figs 1A-E). Positive der and hip joints and have a forceful, flinging serum human immunodeficiency virus anti­ quality. Usually either the arm or the leg is gen and antibody titers were found, with predominantly involved. Although at least mildly elevated toxo­ some volitional control over the affected plasma titers. Anti- treatment limbs is still maintained, the involuntary with sulfadiazine and pyrimethamine was be­ movements typically can be checked by the gun, with resolution of the hemiballistic patient for only a few moments ( 1). The movements within a few weeks and decrease movements are usually continuous but may in size of the lesion. A stereotactic biopsy was be intermittent ( 4). Thus, the disorder can be performed but was nondiagnostic. disabling because of the disruptive effects on daily activities. The force of the motions is Discussion such that injury to the affected limbs may result unless efforts are made to prevent Clinical Features forceful contact with surrounding objects ( eg, by padding of bed rails) (3). The motions are The term hemiballismus refers to a rare most notable during rest and are increased by characterized by involun­ stress (3). They usually can be suppressed to tary, large-amplitude movements of the some degree· by concentration or voluntary limbs of one side of the body. The term is motions of the limbs and are typically absent during sleep (2). Early reports of hemiballis­ Received July 2, 1993; accepted after revision April 15, 1994. mus stressed a poor outcome, with death Address reprint requests to Jam es M. Provenza le, MD, Box 3808, usually resulting from infection related to dis­ Department of Ra diology, Duke University Medical Center, Durham, ability or from the effects of overactivity ( eg, NC 2771 0. Index terms: ; Brain, abscess; Acquired im m unodefi· cardiac failure and exhaustion) (5 ). Before ciency syndrom e (AIDS); Radiologic-clinical correlations; Movement the development of neuropharmacologic

AJ NR 15:1377-1 382, Aug 1994 0195-6108/94/1507-1377 treatment of this disorder, persistent hemibal­ © American Society of lismus was occasionally treated with drastic

1377 1378 HEMIBALLISMUS AJNR: 15, August 1994

measures to prevent self-injury and avoid ex­ haustion. These measures included binding the extremity to the torso, inducing flaccid of the arm by brachial plexus stretch injury or alcohol injection, and even, rarely, amputating (2). Hemiballismus is distinguished from hemi­ , another, closely related, involuntary dyskinetic movement disorder, by the fact that hemichoreic movements are slower, more randomly distributed, and less violent, have smaller excursions, and primarily in­ volve distal musculature (2, 6, 7). Hemibal­ lismus and hemichorea frequently coexist, however, and each type of movement disor­ der may evolve into the other ( 4). The two A disorders are considered by some authors to be two points on a clinical spectrum, with hemiballismus being a severe form of hemi­ chorea (4, 8). Furthermore, experimental ev­ idence in primates suggests that in both dis­ orders there is a decrease in synaptic activity within a common pathway involving fibers from the to the (7). Both hemiballismus and hemi­ chorea also may be associated with other types of abnormal movements, such as my­ oclonus, bradykinesia, , or ( 4 ). Most patients with hemiballismus are af­ fected in middle age or later life (2, 4, 5, 8). The rate of onset is variable and depends to a large extent on the cause. Hemiballismus secondary to infarction typically has a sud­ den onset. In the less common cases of hemi­ ballismus from infectious, inflammatory, metabolic, or neoplastic causes, the clinical course is frequently slowly progressive during days, weeks, or months (4). Despite early reports indicating poor outcomes (5), pres­ ently the prognosis is usually good, with the majority of patients recovering spontane­ C =Caudate ously within 6 months or responding to neu­ T = ropharmacologic therapy (9). A minority of P = patients have prolonged courses, lasting STN = Subthalamic nucleus years (9, 1 0). 8 Fig 1. A and B, Noncontrast sagittal T1-weighted (500/ 11 / 2 [repetition time/ echo time/ excitations]) image per­ Etiology formed 1 em to the left of midline demonstrates the lesion There is a wide variety of causes of hemi­ (arrowheads) as a region of hypointense signal with a very thin rim of hyperintense signal. The spatial relationship of the ballismus. In most elderly patients, a vascular lesion to the thalamus is optimally demonstrated in this imag­ origin (ie, a discrete infarction or hemor­ ing plane. rhage) is found, often involving the contralat- AJNR: 15, August 1994 PROVENZALE 1379

T

IC

= Interpeduncular cistern = Subthalamic nucleus =Thalamus = Putamen c D SN = Substantia Nigra

Fig 1. C and 0 , Contrast-enhanced coronal T1-weighted image (500/11 /2) performed through the subthalamic region and the cerebral peduncles. A ring-enhancing lesion (arrowheads) is present within the left subthalamic region. The mass is located caudal to the left thalamus and medial to the left internal capsule.

era! subthalamic nucleus (2, 5, 8). In most medial and cephalad margin of the peduncu­ middle-aged patients, a nonvascular cause is lar portion of the internal capsule (Fig 1D) usually found ( 4). Reported causes other (18). The internal capsule thus is interposed than infarction include primary or secondary between the subthalamic nucleus and the (4, 11 ), arteriovenous malforma­ globus pallidus, with which it has important tions (12), (13), tubercu­ neural connections. The caudal portion of the lous ( 14), (4), sys­ subthalamic nucleus overlies the rostral por­ temic erythematosus (4), nonketotic tion of the substantia nigra. The vascular sup­ ( 15), and for ply of the subthalamic nucleus is derived (16). Rarely, hemiballismus is from branches of the anterior choroidal, pos­ induced by drugs, including oral contracep­ terior cerebral, and posterior communicating tives and phenytoin, but usually only in pa­ arteries (2), and an infarction causing hemi­ tients with preexisting basal ganglia abnor­ ballismus therefore can result from occlusion malities (2). Hemiballismus in the patient of branches of any of these arteries ( 19). presented here resulted from a toxoplasmosis The neural pathways connecting the sub­ abscess, an uncommon cause (4, 17). thalamic nucleus to its projection areas are complex. Large afferent and efferent path­ ways connect the subthalamic nucleus and the medial and lateral segments of the globus and Pathophysiology pallidus. These pathways, thought important Hemiballism typically results from a lesion in the development of hemiballismus, extend in the contralateral subthalamic nucleus, its both around and through the peduncular por­ efferent or afferent pathways, or their projec­ tion of the internal capsule (through the sub­ tion areas (3, 8). The subthalamic nucleus is thalamic fasciculus) (3). The subthalamic a lens-shaped structure located along the nucleus also has important efferent connec- 1380 HEMIBALLISMU S AJNR: 15, August 1994

tions to the substantia nigra, pars reticulata, and afferent connections from the motor and premotor cortex (3). The importance of the subthalamic nucleus in the development of hemiballismus was ini ­ tially established by ablation of the nucleus in primates (20). It should be noted that a lesion in the subthalamic nucleus does not always result in hemiballismus, for example, if the pyramidal tract and red nucleus are also de­ stroyed (3) . Thereafter, a number of pharma­ cologic means were developed to produce functional decrease in the activity of the sub­ thalamic nucleus without destroying nearby fiber tracts (21). Injection of a y-aminobutyric acid antagonist into, or very close to, the subthalamic nucleus is one means of induc­ ing hemiballismus that has the same quality as that produced by a subthalamic nucleus lesion, but which differs in latency of onset and duration (21 ). The extent and severity of E upper limb or lower limb involvement varies between the injection site chosen, suggesting that the subthalamic nucleus may have a so­ matotopic representation ( 21). Lesions affecting the afferent or efferent pathways of the subthalamic nucleus ( eg, the subthalamic fasciculus) (20) or the subtha­ lamic nucleus projection areas also can pro­ duce hemiballismus (2, 8). The neostriatum (ie, putamen and ) is the most common site involved other than the subthalamic nucleus ( 10, 22). Hemiballis­ mus also may result from lesions in the thal­ amus or, in rare instances, at sites distant from the basal ganglia with which the subtha­ lamic nucleus has major connections ( eg, the precentral or postcentral gyrus) ( 3). There is

SN Substantia Nigra evidence that hemiballismus caused by le­ RN Red nucleus sions at sites other than the subthalamic nu­ M Mammillary bodies cleus may be more persistent than those 3 3rd Ventricle solely involving the subthalamic nucleus STN Subthalamic nucleus ( 10) . The mechanism by which hemiballismus is F produced is not completely understood. There is increasing evidence that the subtha­ Fig 1. E and F, Axial T2-weighted image (2500/ 80/l) per­ lamic nucleus has a tonic inhibitory influence formed through the subthalamic region just at the level of the on the thalamus by means of excitatory pro­ mamillary bodies (arrowheads ) and the most cephalad extent jections onto inhibitory in the medial of the mesencephalon. The lesion involves the subthalam ic nucleus ( curved arrow) but also extends to involve the left globus pallidus (23). These inhibitory fibers, substantia nigra (open arrow), which is displaced ventra lly in turn, project to the ventrolateral nucleus of by the mass. The red nucleus (arrow) is not involved by the the thalamus, from which excitatory thalamo­ mass. cortical pathways project. Therefore, a de- AJNR: 15, August 1994 PROVENZALE 1381

structive lesion in the subthalamic nucleus Treatment could result in disinhibition of these excita­ Many patients with hemiballismus second­ tory pathways, resulting in production of ary to cerebrovascular accidents have spon­ hemiballistic movements. Although the taneous, gradual improvement during the mechanisms by which lesions in the caudate course of weeks or months, possibly second­ and putamen produce hemiballismus are ary to resolution of edema surrounding the poorly understood, they are also presumed to infarct and reperfusion of affected tissue ( 4). be caused by interruption of topographic Before the development of present neuro­ connections from the caudate and putamen pharmacologic therapy, stereotactic ventro­ to the globus pallidus. lateral thalamotomy was the procedure of choice (29) and is still used in selected cases. However, persistent hemiballismus is often treated by neuropharmacologic means, Radiology which are usually effective in reduction of the Neuroradiologic examinations of patients movements (4, 8). This therapy is based on with hemiballismus or hemichorea must fo ­ the knowledge that dopaminergic activity re­ cus on the contralateral subthalamic nucleus sults in inhibition of subthalamic nucleus neu­ and its major pathways and projection sites. ronal firing , with the result that a decrease in Infarctions causing hemiballismus are usually activity should result in decrease seen as small low-attenuation regions on of the dyskinetic movements (2, 8). Dopa­ computed tomography or a focus of hyperin­ mine antagonists, especially and tense signal on T2-weighted images, within phenothiazines (8), are most commonly the subthalamic nucleus (Fig lE) (24). Hem­ used, with dopamine-depleting agents (eg, reserpine) less commonly used. The majority orrhage within the subthalamic nucleus is of­ of patients either improve spontaneously or ten on the basis of hypertension or rupture of respond to these medications (4). In the pa­ a small vascular malformation. A ring­ tient presented here, the hemiballistic move­ enhancing mass lesion in this region could be ments resolved after treatment of the specific caused by either a (most com­ cause of the hemiballismus (ie, antitoxoplas­ monly a metastasis rather than a primary mosis therapy). neoplasm) or an abscess. However, careful evaluation of the basal ganglia, thalamus, Acknowledgment and selected other regions of the brain is also indicated, because lesions causing hemibal­ We are grateful to Ernest Picard, MD, for clinical information regarding the patient presented in this arti­ lismus or hemichorea have been demon­ cle and for a helpful review of the m anuscript. strated by computed tomography or mag­ netic resonance in the caudate nucleus (25), thalamus (4), corpus (26), putamen References ( 15), lenticular nucleus (27), and corona ra­ 1. Meyers R. Ballismus. In : Vinken PJ , Bruyn GW, eds. Handbook diata (28). Computed tomographic identifi­ of Clinica l : Diseases of the Basal Ganglia. A m ster­ cation of lesions producing hemiballismus dam: North Holland Pu blishing, 1968:476-490 2. Shannon KM. Hemiba llismus. Clin Neuropharmacol1990;13: can prove difficult, because of their small size 4 13- 425 (24). The multiplanar capability of magnetic 3. Buruma OJS, Lakke JP. Ballism. In : Vinken PJ , Bruyn GW, resonance offers a number of distinct advan­ Klawans HL, ed . Handbook of Clinical Neurology: Extrapyra­ tages in demonstration of lesions involving midal Disorders. Amsterdam: Elsevier Science Publ ishers, 1986:369-380 the subthalamic nucleus or any of its projec­ 4. Dewey RB, Jankovic J. Hemiballism-hemichorea. Clinical and tions. Nonetheless, computed tomography pharmacologic findings in 21 patients. Arch Neural 1989;46: and magnetic resonance findings in a recent 862-867 series of patients with hemiballismus demon­ 5. Whittier JR. Ballism and the subthalamic nucleus (nucleus hypothalamicus; corpus Luysii). Arch Neural Psy chiatry 194 7; strated lesions in the subthalamic nuclei, 58:672-692 basal ganglia, or thalami in only slightly more 6. Lownie SP, Gilbert JJ. Hemichorea and hemiballismus: recent than 60% of cases ( 4). concepts. C/in Neuropalhol 1990;9:46-50 1382 HEMIBALLISMUS AJNR: 15, August 1994

7. Mitchell IJ, J ackson A, Sambrook MA, Crossman AR. Com­ 19. Fisher CM. La cunar and infarcts: a review. Neurology mon neural m echanism s in experimental chorea and hemibal­ 1982;32:871-876 lismus in the monkey: evidence from 2-deoxyglucose autora­ 20. Carpenter MB. Ballism associated with partial destruction of diography. Brain Res 1985;339:346-350 the subthalamic nucleus of Luys. Neurology 1955;5:4 79-489 8. Klawans HL, Moses H, Nausieda PA, et al. Trea tment and 21. Crossman AR, Sambrook MA, Jackson A. Experimental hemi­ prognosis of hemiballismus. N Eng/ J Med 1976;295:1348- chorea/hemiballismus in the monkey. Brain 1984; 1 07:579- 1350 596 9. Muenter MD. Hemiballismus. Neurology 1984;34 (suppl 1) 22. Schwarz GA, Barrows LJ. Hemiballism without involvement of (abstr): 129 Luys body. Arch Neuro/1960;2:420-434 10. Lang AE. Persistent hemiballismus with les ions outside the 23. Delong MR. Primate models of movement disorders of the subthalamic nucleus. Ca n J Neural Sci 1985; 12:125-128 basal ganglia. Trends Neurosci 1990;2:281-285 11. Glass PJ , Jankovic J , Borit A . Hemiballism and metastatic 24. Biller J, Graff-Radford NR, Soker WRK, Adams HP, Johnston brain tumor. Neurology 1984;34:204-207 P. MR imaging in "lacunar" hemiballismus. J Comput Assist 12 . Diam ond MS, Huang YP, Yahr MD. Sudden onset of involun­ Tomogr 1986;10:793-797 tary movement disorders with arteriovenous malformations of 25. Saris S. Chorea caused by caudate infarction. Arch Neural the basal ganglia. Mt Sinai J Med 1982;49:438-442 1983;40:590-591 13. Riley D, Lang AE. Hemiballism in multiple sclerosis. Move­ 26. Lodder J, Baard WC. Paraballism caused by bilateral hemor­ m ent Disorders 1988;3:88-94 rhagic infarction in basal ganglia. Neurology 1981 ;31 :484- 14. Babikian VL, Heydemann PT, Swisher CN. Extrapyramidal 486 movem ents in a patient with . Clin Pe­ 27. Mas JL, Launay M, Derouesne C. Hemiballism and CT-docu­ dialr 1985;24:113-115 mented lacunar infarct in the lenticular nucleus. J Neural Neu­ 15. A ltafull ah I, Pascuai-Leone A, Duvall K, A nderson DC, Taylor ros urg 1987;50:104-105 S. Putamina I hem orrhage accompanied by hemichorea-hemi­ 28. Barinagarrementeria F, Vega F, Del Brutto OH. Acute hemi­ ba llism (letter). 1990;21:1093-1094 chorea due to infarction in the corona radiata. J Neuro/1989; 16. Modesti LM, Van Buren JM. Hemiballismus complicating ste­ 236:37 1-372 reotacti c thalamotomy. Appl Neurophysio/1979;42:267-283 29. Martin JP, McCauiiR. Acute hemiballismus treated by ventro­ 17. Nath A, Jankovic J, Pettigrew LC. Movement disorders and lateral thalamolysis. Brain 1959;82: 104-108 AIDS. Neurology 1987;37:37-41 18. Carpenter MB. Core Text of Neuroanatomy. Baltimore: Williams and Wilkins, 1976:248