[An autopsied case of manifesting chorea, serum antibody to brain proteins, neuronal degeneration in striatum and grumose degeneration in dentate nucleus].

A 49-year-old man complained of difficulty in writing due to choreic involuntary movement in the right hand, which spread gradually to his left hand, face, lower extremities and trunk during next nine years. He also showed difficulties in speech and swallowing. His family noticed his memory disturbance and change of his character. Ten years later, mental deterioration, dysarthria and dysphagia were manifested. He showed facial grimacing and dancing gait. Deep tendon reflexes were exaggerated with no pathological reflexes. Muscle tone was rigid at his ankles. He showed no ataxia nor sensory impairment. At about 61 years old, choreic movement was decreased and rigidity spread in all extremities with joints' constructure. During next one or two years, choreic movement diminished and was restricted to his face. The extremities became more rigid and voluntary movement was severely impaired. He became mutistic with severe mental deterioration. He died at the age of 65. None of his family suffered from the same disease. Routine blood examination was normal, and there were no acanthocytes. Brain CT showed marked dilatation of lateral and third ventricles, and mild cerebral cortical atrophy. The head of caudate nucleus was also atrophic. The total protein level in the CSF was elevated to 79-121 mg/dl, and IgG% was 13.7-26.6 for last six years. Anti-CNS antibodies in the serum reacted with brain proteins of 156 kDa and 82 kDa. Pathological examination showed severe atrophy of the caudate nucleus, putamen, globus pallidus, cerebral cortex and cerebral white matter.(ABSTRACT TRUNCATED AT 250 WORDS)