Late-onset cerebellar ataxia with hypogonadism and muscle coenzyme Q10 deficiency
- 9 March 2004
- journal article
- case report
- Published by Wolters Kluwer Health in Neurology
- Vol. 62 (5) , 818-820
- https://doi.org/10.1212/01.wnl.0000113719.67643.b7
Abstract
Two brothers had late-onset progressive ataxia, cerebellar atrophy, and hypergonadotropic hypogonadism associated with coenzyme Q10 (CoQ10) deficiency in skeletal muscle. Both patients improved on high-dose CoQ10 supplementation, stressing the importance of CoQ10 deficiency in the differential diagnosis of cerebellar ataxia, even when onset is late.Keywords
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