J Neurol Sci. 1993 Sep.
Treatment of complex I deficiency with riboflavin.
Bernsen PL, Gabreels FJ, Ruitenbeek W, Hamburger HL.
Department of Child Neurology, St. Radboud University Hospital, Nijmegen, The Netherlands.

We have evaluated the effects of treatment with riboflavin in five patients with a mitochondrial myopathy, associated with a complex I (NADH dehydrogenase) deficiency. Two patients suffered from a clinically pure myopathy and the other patients presented with encephalomyopathic features. Treatment with riboflavin resulted in a clear clinical improvement in the two patients with the myopathic form of complex I deficiency. However, only one of the patients with the encephalomyopathic form improved during therapy. In three of the four patients in whom complex I activity in muscle tissue has been determined again during therapy, complex I activity appeared to be normalized. The clinical effects of treatment in this group of patients do not correlate well with normalization of complex I activity.

Categories: 1993, Mitochondria, Electron transport chain, Complex I, Myopathy, Mitochondrial myopathy, Encephalopathy, Riboflavin - B2