CLINICAL-FEATURES OF FAMILIAL DIFFUSE LEWY BODY DISEASE

We present 5 patients from two families with familial autosomal domina nt diffuse Lewy body disease. All 5 patients initially showed parkinso nian symptoms that responded well to levodopa therapy; however, dement ia followed and later progressed. In 3 patients, the disease was manif ested before age 40 years, exhibiting wearing-off phenomenon and chore ic limb dyskinesia. One patient was examined neuropathologically, and brain stem lesions compatible with Parkinson's disease were revealed, and Lewy bodies and senile plaques were found in the cerebral cortex. Familial cases of diffuse Lewy body disease may provide some explanati on of the pathogenesis of this disease.