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symptoms The diseases of which these symptoms are a part are considered elsewhere, mainly in Chap 39 Somewhat more ambiguous but in common clinical use is the term dyskinesia It encompasses all the active movement phenomena that are due to disease of the basal ganglia, usually implying an element of dystonia, but it has also been used to refer more speci cally to the undifferentiated excessive movements that are induced in Parkinson patients at the peak of L-dopa effect and to numerous dystonic and athetotic movements that may follow the use of neuroleptic drugs ( tardive dyskinesias, Chaps 6 and 43) Chorea Derived from the Greek word meaning dance, chorea refers to involuntary arrhythmic movements of a forcible, rapid, jerky type These movements may be simple or quite elaborate and of variable distribution Although the movements are purposeless, the patient may incorporate them into a deliberate act, as if to make them less noticeable When superimposed on voluntary actions, they may assume an exaggerated and bizarre character Grimacing and peculiar respiratory sounds may be other expressions of the movement disorder Usually the movements are discrete; but if they are very numerous, they become con uent and then resemble athetosis, as described below In moments when the involuntary movements are absent or brie y held in abeyance, volitional movements of normal strength are possible, since there is no paralysis; but these movements tend also to be excessively quick and poorly sustained The limbs are often slack or hypotonic and, because of this, the knee jerks tend to be pendular; with the patient sitting on the edge of the examining table and the foot free of the oor, the leg swings back and forth four or ve times in response to a tap on the patellar tendon, rather than once or twice, as it does normally A choreic movement may be superimposed on the re ex movement, checking it in ight, so to speak, and giving rise to the hung-up re ex The hypotonia in chorea as well as the pendular re exes may suggest a disturbance of cerebellar function Lacking, however, are intention tremor and true incoordination or ataxia In patients with hypernatremia and other metabolic disorders, it may be necessary to distinguish chorea from myoclonus Chorea differs from myoclonus mainly with respect to the speed of the movements; the myoclonic jerk is much faster and may involve single muscles or part of a muscle as well as groups of muscles Failure to appreciate this difference between chorea an myoclonus often results in an incorrect diagnosis Diseases characterized by chorea are listed in Table 4-4 Chorea is a major feature of Huntington disease (hereditary or chronic chorea), in which the movements tend more typically to be choreoathetotic Also, there is an inherited form of chorea of childhood onset without dementia that has been referred to as benign hereditary chorea The gene mutation is on chromosome 14, different from the expanded gene on chromosome 4 that characterizes Huntington disease There may be subtle additional ataxia of gait, as noted by Breedveld and colleagues Not infrequently, chorea has its onset in late life without the other identifying features of Huntington disease It is then referred to as senile chorea, a term that is hardly helpful in understanding the process Its relation to Huntington chorea is unsettled It may be a delayed form of the disease, and a few such patients we have seen have had atypical depressions or mild psychosis; but others remain for a decade with only chorea Genetic testing settles the issue A number of far less common degenerative conditions are associated with chorea, among them dentatorubropallidoluysian atrophy These are discussed in Chap 39.

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FIGURE 9-9

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Table 4-4 Diseases characterized by chorea Inherited disorders Huntington disease Benign hereditary chorea Neuroacanthocytosis Dentatorubropallidoluysian atrophy Wilson disease Rheumatic chorea Sydenham, chorea gravidarum Drug-induced chorea Neuroleptics (phenothiazines, haloperidol, and others) Oral contraceptives Phenytoin (occasionally other anticonvulsants) Excess dosages of L-dopa and dopamine agonist medications Cocaine Chorea symptomatic of systemic disease Lupus erythematosus with antiphospholipid antiboies Thyrotoxicosis Polycythemia vera Hyperosmolar, nonketotic hyperglycemia AIDS Paraneoplastic, often with other movements Hemichorea, rarely associated with Stroke Tumor Vascular malformation

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Typical choreic movements are the dominant feature of Sydenham chorea and of the variety of that disease associated with pregnancy (chorea gravidarum), disorders that are related in some way to streptococcal infection Striatal abnormalities have been demonstrated by magnetic resonance imaging (MRI) in some cases; they are usually transient but rarely persistent (Emery and Vieco) It is perhaps not surprising that antibodies directed against cells of the basal ganglia have been detected in both acute and late Syndeham chorea (Church et al) Following from the connection to streptococcal infection and the detection of these antibodies, in recent years it has been suggested that the spectrum of poststreptococcal disorders be extended to tic and obsessive-compulsive behavior in children In these cases the neurologic problems are said to arise suddenly, subside, and return with future streptococcal infections, as discussed in Chap 6 The administration of phenothiazine drugs or haloperidol (or an idiosyncratic reaction to these drugs) is a common cause of extrapyramidal movement disorders of all types, including chorea; these may become manifest during use of the drug or in a delayed tardive fashion, as already mentioned Excess dopamine administration in advanced Parkinson disease is perhaps the most common cause of a choreiform dyskinesia in practice, but the movements tend to be more complex and continuous than those seen in chorea The use of oral contraceptives sometimes elicits chorea in an otherwise healthy young woman, but the majority of such patients have underlying systemic lupus erythematosus and antiphospholipid antibodies Whether the chorea (usually unilateral) is the result of a small infarction (as suggested by the a mild hemiparesis on the affected side) or is an immunolgic condition is not settled The re-emergence of chorea in these circumstances as steroids are with-

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