{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,20]],"date-time":"2026-09-20T17:58:27Z","timestamp":1789927107882,"version":"4.0.1"},"reference-count":32,"publisher":"S. Karger AG","issue":"3","license":[{"start":{"date-parts":[[2009,1,1]],"date-time":"2009-01-01T00:00:00Z","timestamp":1230768000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"},{"start":{"date-parts":[[2009,1,1]],"date-time":"2009-01-01T00:00:00Z","timestamp":1230768000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neuroepidemiology"],"published-print":{"date-parts":[[2009]]},"abstract":"<jats:p>&lt;i&gt;Background:&lt;\/i&gt; The prevalence of disease subtypes of spinocerebellar degenerations (SCDs) varies between countries, and even between areas within a country. We report unprecedented epidemiologic data on SCDs in the Hokuriku district, which is located in the central, western part of Japan. &lt;i&gt;Methods:&lt;\/i&gt; Clinical and genetic data on SCD patients were obtained via questionnaires distributed to all the departments of neurology, psychiatry and internal medicine in the Hokuriku district (n = 418). &lt;i&gt;Results:&lt;\/i&gt; Among the SCD patients, autosomal dominant cerebellar ataxias (ADCAs) were noted in 40.4%, multiple system atrophy in 24.7%, cortical cerebellar atrophy in 13.3% and autosomal recessive cerebellar ataxia in 0.3%. Genetically confirmed ADCA patients included those with Machado-Joseph disease (MJD)\/spinocerebellar ataxia type 3 (SCA3; 63.3%), SCA6 (20.0%), ADCA linked to chromosome 16q22.1 (10.0%), dentatorubral pallidoluysian atrophy (4.4%), SCA1 (1.1%) and SCA2 (1.1%). MJD\/SCA3 was highly prevalent in the Toyama prefecture of the Hokuriku district, accounting for 90% of genetically confirmed ADCAs by birthplace; MJD\/SCA3 patients were concentrated in the Gosei area, the western part of the Toyama prefecture, giving an estimated prevalence of 19.1 per 100,000 inhabitants. &lt;i&gt;Conclusions:&lt;\/i&gt; The Hokuriku district, especially the Gosei area of Toyama, had a surprisingly high relative frequency and prevalence of MJD\/SCA3, which is comparable to that in the Azores, Portugal.<\/jats:p>","DOI":"10.1159\/000195686","type":"journal-article","created":{"date-parts":[[2009,1,26]],"date-time":"2009-01-26T15:51:04Z","timestamp":1232985064000},"page":"176-183","source":"Crossref","is-referenced-by-count":17,"title":["Prevalence of Spinocerebellar Degenerations in the Hokuriku District in 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