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

M Gironi, C Lamperti, R Nemni, M Moggio, G Comi… - Neurology, 2004 - AAN Enterprises
M Gironi, C Lamperti, R Nemni, M Moggio, G Comi, FR Guerini, P Ferrante, N Canal, A Naini…
Neurology, 2004AAN Enterprises
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.
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.
American Academy of Neurology