Focal paroxysmal kinesigenic choreoathetosis.
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A family is presented with paroxysmal dystonic choreoathetosis transmitted as a dominant trait over five generations. The family is unusual in the marked responsiveness of the episodes to short periods of sleep in several members, in the very variable age of onset, and in the association with prominent myokymia in some cases. These overlap features suggest a link between paroxysmal dystonic choreoathetosis and familial paroxysmal ataxia with myokymia.
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Hypoglycaemia due to an insulinoma can mimic acute disorders of cognition, consciousness, epilepsy, transient ischaemia, or psychosis, and chronic disorders of dementia and neuropathy. Misdiagnosis and delay in treatment are common and prolonged hypoglycaemia can lead to permanent neurological deficit or fatal coma. A 27 year old woman with hypoglycaemia induced by an insulinoma presented with features typical of paroxysmal non-kinesiogenic dystonic choreoathetosis. Striatal dysfunction as a consequence of hypoglycaemia has rarely been described. Insulinoma is a readily treatable condition that should be considered in the differential diagnosis of a paroxysmal movement disorder.
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Paroxysmal kinesigenic choreoathetosis is an unusual movement disorder often triggered by attempts to use the limbs, and has sometimes been associated with diffuse or focal brain injury. We report its occurrence in hyperthyroidism, with which choreoathetosis has rarely been described in the past without known cause. Choreoathetosis has also occurred with other metabolic and toxic disorders, and the mechanism is uncertain. The development of involuntary movements activated by limb motion during hyperthyroidism suggests an influence by thyroid hormone or metabolic state on motor neurone excitability or motor system organization.
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