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NEUROLOGY 2004;62:818-820
© 2004 American Academy of Neurology
Brief Communications

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

M. Gironi, MD, PhD, C. Lamperti, MD, R. Nemni, MD, M. Moggio, MD, G. Comi, MD, F. R. Guerini, PhD, P. Ferrante, MD, N. Canal, MD, A. Naini, PhD, N. Bresolin, MD and S. DiMauro, MD

From the Department of Neurology (Drs. Gironi, Nemni, and Canal) and Laboratory of Biology (Dr. Guerini), Don C. Gnocchi Foundation IRCCS, S. Maria Nascente, Department of Preclinical Sciences (Dr. Ferrante), University of Milan, and Unitá Operativa Neurologia (Drs. Lamperti, Moggio, Comi, and Bresolin), Ospedale Maggiore Milano, IRCCS, Universitá degli Studi di Milano, Centro Dino Ferrari, Italy; and Department of Neurology (Drs. Naini and DiMauro), Columbia University College of Physicians and Surgeons, New York, NY.

Address correspondence and reprint requests to Dr. S. DiMauro, 4–420 College of Physicians and Surgeons, 630 W. 168th St., New York, NY 10032; e-mail: sd12{at}columbia.edu

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.

Received July 30, 2003. Accepted in final form October 28, 2003.




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