Striatonigral Degeneration and Sporadic Olivopontocerebeller Atrophy: A Consideration of the Clinical Entity of Multiple System Atrophy.

Accession number;99A0551104
Title;Striatonigral Degeneration and Sporadic Olivopontocerebeller Atrophy: A Consideration of the Clinical Entity of Multiple System Atrophy.
Author; MIWA HIDETO (Juntendo Univ., Sch. of Med.) KONDO TOMOYOSHI (Juntendo Univ., Sch. of Med.) MIZUNO YOSHIKUNI (Juntendo Univ., Sch. of Med.)
Journal Title;Brain Nerve
Journal Code:Z0685A
ISSN:0006-8969
VOL.51;NO.4;PAGE.305-312(1999)
Figure&Table&Reference;FIG.1, TBL.5, REF.33
Pub. Country;Japan
Language;Japanese
Abstract;Striatonigral degeneration(SND) and sporadic olivopontocerebellar atrophy(sOPCA) are categorized under multiple system atrophy(MSA), since these disorders have common clinical and pathological features. However, it is still uncertain whether these disorders are manifestation of the same disease. In this study, we performed both clinical and neuroradiological studies on patients with SND or sOPCA in whom clinical diagnosis was based on a criteria during eight years in our hospital. A total of forty patients had SND and thirty-one patients had sOPCA. The onset ages of patients with SND (60.7.+-.8.7, mean .+-.SD) were significantly higher than those with sOPCA (55.4.+-.7.9). In both SND and sOPCA patients, about 20% had clinical symptoms suggesting the involvement of multiple systems: pyramidal, cerebellar, extrapyramidal and autonomic symptoms. In 55% of the SND patients, cerebellar symptoms could be observed, and the same percentage of sOPCA patients had parkinsonism. Although, as defined, cerebellar symptoms were predominant in sOPCA patients and parkinsonism was predominant in SND patients, the SND patient group was particularly homogeneous with respect to clinical characteristics. The initial symptoms of SND were parkinsonian gait or tremors. Almost all patients exhibited asymmetrical appearance of the parkinsonian symptoms, such as rigidity, tremors, and bradykinesia. Tremors at rest were observed in two-thirds of the patients with SND during the course of their illness, but dementia was infrequently observed. There was no detectable limitation in horizontal eye movements in patients with SND. The progression of the disability of patients with SND was rapid; according to the clinical rating scale of parkinsonism, the average level of disability deteriorated to Hoehn-Yahr's stage III after three years from disease onset, and then deteriorated to stage IV after four years.... (author abst.)
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