SCA type 6 in 20.0%, ADCA linked to chromosome 16q22.1 in 10.0%, dentatorubral pallidoluysian atrophy in 4.4%, SCA type 1 in 1.1% and SCA type 2 in 1.1%. SCA type 3 was highly prevalent in the Toyama prefecture of the Hokuriku district, particularly in the Gosei area (the western part of Toyama prefecture), with a prevalence of 19.1 per 100,000 people [10] , which is very similar to the prevale...

Motor and sensory conduction studies have been performed in 10 patients from three families with uncomplicated familial spastic paraplegia whose ages ranged from 4 to 41 years. In all cases the values fell within the control range. The findings may be contrasted with those in Friedreich's ataxia and some other spinocerebellar degenerations in which peripheral nerve abnormalities are present.

It has been reported that patients with spinocerebellar degenerations (SCDs) have cognitive dysfunction as well as limb and truncal ataxia, dysarthria and dysphagia. We review cognitive dysfunction in common types of SCD, including spinocerebellar ataxia types 1, 2, 3, 6, and 17, dentatorubral-pallidoluysian atrophy, Friedreich's ataxia, and multiple system atrophy. There are few studies that a...

Leucocyte glutamate dehydrogenase (GDH) activity was measured in 26 normal control subjects, 20 patients with multiple system atrophy presenting features of either olivopontocerebellar atrophy or striatonigral degeneration and in a heterogenous group of 15 patients with spinocerebellar degenerations. A broad range of GDH activity was found in all three groups. Low activity failed to correlate w...