A Familial Parkinsonism Presenting with Dementia, Freezing Gait, Marked Wearing off Phenomenon, and Frontal Atrophy.

Accession number;00A0222255
Title;A Familial Parkinsonism Presenting with Dementia, Freezing Gait, Marked Wearing off Phenomenon, and Frontal Atrophy.
Author; YUASA TATSUHIKO (Kokuritsu Seishin Shinkei Senta Konodai Byoin) NISHIMIYA JIN (Kokuritsu Seishin Shinkei Senta Konodai Byoin) YOSHINO HIDE (Kokuritsu Seishin Shinkei Senta Konodai Byoin)
Journal Title;Japanese Journal of National Medical Services
Journal Code:F0707A
ISSN:0021-1699
VOL.54;NO.1;PAGE.21-26(2000)
Figure&Table&Reference;FIG.4, TBL.1, REF.16
Pub. Country;Japan
Language;Japanese
Abstract;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 heredofamilial cases of Parkinson's disease and/or parkinsonisms drew attention in conjunction with gene mutations, and the Parkinson-related proteins, .ALPHA.-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 frontotemporal 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. (author abst.)