Late-onset cerebellar ataxia with hypogonadism and muscle coenzyme Q10 deficiency.

Gironi, M; Lamperti, C; Nemni, R; Moggio, M; Comi, G; Guerini, F R; Ferrante, P; Canal, N et al. · Neurology · 2004

case_series · Level IV

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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.

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