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drawn or birth control pills are introduced suggests a more complex process than simply a small deep infarction perhaps something akin to Sydenham chorea Also, only about one-third of cases involve a stroke, and some have demonstrated hypermetabolism of the basal ganglia, also as in Sydenham chorea A connection between hemichorea and the antiphospholipid syndrome alone, without lupus, is tenuous in our view The use of phenytoin or other anticonvulsant drugs causes chorea in sensitive individuals A transitory chorea may occur in the course of an acute metabolic derangement such as hyperosmolar hyperglycemia, hypoglycemia, or hyponatremia Rarely, chorea complicates hyperthyroidism, polycythemia vera, lupus erythematosus or other forms of cerebral arteritis AIDS has emerged as a cause of a number of subacutely progressive movement disorders, initially asymmetrical in our experience, and chorea has been one manifestation The usual associations have been with focal lesions in or near the basal ganglionic stuctures such as toxoplasmosis, progressive multifocal leukoencephalopathy, and lymphoma, but a number of instances are not explained by any of these An inherited chorea with acanthocytosis of red blood cells has been well described, but the nature of the lesions of the basal ganglia is unknown (Chap 39) A paraneoplastic variety may combine several aspects of chorea with athetosis and ballismus or dystonia; in ammatory lesions are found in the striatum (Chap 31) Chorea may be limited to one side of the body (hemichorea) When the involuntary movements involve proximal limb muscles and are of wide range and inging in nature, the condition is called hemiballismus (see further on) A cerebral infarction is the usual cause A number of rare paroxysmal kinesigenic disorders, discussed later in this chapter, may have a choreic component The review by Piccolo and colleagues puts the frequency of the various causes of chorea in perspective Of a large number of consecutive neurologic admissions to two general hospitals, they identi ed 23 cases of chorea, of which 5 were drug-induced, 5 were AIDS-related, and 6 were due to stroke Sydenham chorea and arteritis were each found in 1 case In 4 cases no cause could be determined, and 1 case proved to have Huntington disease The anatomic basis of chorea is uncertain or at least inconsistent In Huntington chorea, there are obvious lesions in the caudate nucleus and putamen Yet one often observes vascular lesions in these parts without chorea The precise localization of lesions in Sydenham chorea and other choreic diseases has not been determined beyond a generalized disturbance in the striatum, which is evident on some imaging studies It is of interest that in instances of chorea related to acute metabolic disturbances, there are sometimes small infarctions in the basal ganglia or metabolic changes in the lenticular nucleus, as shown by imaging studies One suspects from their close clinical similarity that chorea and hemiballismus relate to disorders of the same system of neurons; however, the subthalamic nucleus is affected only slightly in Huntington chorea Athetosis This term stems from a Greek word meaning un xed or changeable The condition is characterized by an inability to sustain the ngers and toes, tongue, or any other part of the body in one position The maintained posture is interrupted by relatively slow, sinuous, purposeless movements that have a tendency to ow into one another As a rule, the abnormal movements are most pronounced in the digits and hands, face, tongue, and throat, but no group of muscles is spared One can detect as the basic patterns of movement an alternation between extension-pronation and.

TABLE 1-5

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exion-supination of the arm and between exion and extension of the ngers, the exed and adducted thumb being trapped by the exed ngers as the hand closes Other characteristic movements are eversion-inversion of the foot, retraction and pursing of the lips, twisting of the neck and torso, and alternate wrinkling and relaxation of the forehead or forceful opening and closing of the eyelids The movements appear to be slower than those of chorea, but all gradations between the two are seen; in some cases, it is impossible to distinguish between them, hence the term choreoathetosis Discrete voluntary movements of the hand are executed more slowly than normal, and attempts to perform them may result in a cocontraction of antagonistic muscles and a spread (over ow) of contraction to muscles not normally required in the movement (intention spasm) The over ow appears related to a failure of the striatum to suppress the activity of unwanted muscle groups Like polymyoclonus (see Chap 6), some forms of athetosis occur only during the performance of projected movement (intention or action athetosis) In other forms, the spasms appear to occur spontaneously, ie, they are involuntary and, if persistent, give rise to more or less xed dystonic postures, as described by Yanagisawa and Goto Athetosis may affect all four limbs or may be unilateral, especially in children who have suffered a hemiplegia at some previous date (posthemiplegic athetosis) Many athetotic patients exhibit variable degrees of rigidity and motor de cit due to associated corticospinal tract disease; these may account for the slower quality of athetosis compared to chorea In other patients with generalized choreoathetosis, as pointed out above, the limbs may be intermittently hypotonic The combination of athetosis and chorea of all four limbs is a cardinal feature of Huntington disease and of a state known as double athetosis, which begins in childhood Athetosis appearing in the rst years of life is usually the result of a congenital or postnatal condition such as hypoxia or rarely kernicterus Postmortem examinations in some of the cases have disclosed a peculiar pathologic change of probable hypoxic etiology, a status marmoratus, in the striatum (Chap 38); in other cases, of probable kernicteric (hyperbilirubinemic) etiology, there has been a loss of nerve cells and myelinated bers a status dysmyelinatus in the same regions In adults, athetosis may occur as an episodic or persistent disorder in hepatic encephalopathy, as a manifestation of chronic intoxication with phenothiazines or haloperidol, and as a feature of certain degenerative diseases, most notably Huntington chorea but also Wilson disease, Hallervorden-Spatz disease, Leigh disease, and other mitochondrial disease variants; less frequently, athetosis may be seen with Niemann-Pick (type C) disease, Kufs disease, neuroacanthocytosis, and ataxia telangiectasia It may also occur as an effect of excessive L-dopa in the treatment of Parkinson disease, in which case it appears to be due to a decrease in the activity of the subthalamic nucleus and the medial segment of the globus pallidus (Mitchell et al) Athetosis, usually in combination with chorea, may occur rarely in patients with AIDS and in those taking anticonvulsants Localized forms of athetosis may occasionally follow vascular lesions of the lenticular nucleus or thalamus, as in the cases described by Dooling and Adams Ballismus This term designates an uncontrollable, poorly patterned inging movement of an entire limb As remarked earlier, it is closely related to chorea and athetosis, indicated by the frequent coexistence of these movement abnormalities and the tendency for ballismus to blend into a less obtrusive choreoathetosis.

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