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Neuro otological analysis in patients with spinocerebellar degeneration and shy drager syndrome with special reference to the problems of classification and autonomic disturbances



Neuro otological analysis in patients with spinocerebellar degeneration and shy drager syndrome with special reference to the problems of classification and autonomic disturbances



Practica Otologica Kyoto (Suppl. 36): 90-97



In 28 patients with spinocerebellar degeneration and 3 patients with Shy-Drager syndrome (SDS), who had visited the Toyama Medical and Pharmaceutical University Hospital during the period from 1983 to 1988, the neuro-otological and autonomic nervous system examinations were made in the Department of Neurotology. According to Hirayama's classification (1982), the patients were classified into three groups, i.e., 15 cases of OPCA and Menzel type (OPCA group), 7 of with LCCA and Holmes type (LCCA group), and 6 cases of miscellaneous type from the standpoint of neural functions. The results obtained were summarized as follows and were evaluated with special references to the comparison of the groups and SDS. The incidence of gaze nystagmus and rebound and rebound nystagmus was higher in the group of OPCA than LCCA. Both horizontal and vertical smooth pursuit eye movement and OKN were disturbed in those groups including SDS. FFS (failure of fixation suppression) was found more often in the LCCA group than OPCA type. LCCA might affect the Purkinje cell. In some cases the discrepancy between positive FFS and smooth pursuit deficit was recognized. There may not be a common pathway in these two mechanisms. Autonomic nerve system disturbances were observed in patients with not only SDS and OPCA but with LCCA. The test battery of image analysis and neuro-otological and autonomic nerve examinations are important to differentiate those disease entities.

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Accession: 007587142

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DOI: 10.5631/jibirinsuppl1986.1989.supplement36_90


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