In this report we present a family with late onset of parkinsonism, characterized by clinical features of freezing gait, wearing-off phenomenon, dementia, and frontal lobe atrophy. The father was said to have the same disease as his sons who showed Parkinson's symptoms starting at late 60's. Anti-Parkinson's drugs showed an improvement in their Parkinsonian symptoms, but only to a slight degree. A marked wearing-off phenomenon followed after the combination therapy of several anti-Parkinsonian drugs.
In recent years heredof amilial cases of Parkinson's disease and/or parkinsonisms drew attention in conjunction with gene mutations, and the Parkinson-related proteins, a-synuclein and the Parkin, were found to be related to chromosome 4q in one of the autosomal dominant types of parkinsonism, and to chromosome 6q 25-27 in autosomal recessive juvenile parkinsonism, respectively. Although most of the cases of familial parkinsonism including familial cases of diffuse Lewy body disease are unknown etiology yet, a peculiar cases with balloned neurons were reported by Mizutani with 17-chromosome linked parkinsonism and f rontotemporal dementia.
The cases presented here are such one of familial cases as yet to be determined the etiology, but from a clinical point of view we suspected this family to have parkinsonism of the type described by Mizutani.
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