Abstract
A 17-year-old boy with type I glycogen storage disease (GSD-I) and a stroke associated with moyamoya disease is described. This is the first report of this association, and only the second reported case of cerebrovascular disease in patients with GSD-I. The relationship of the primary metabolic error and the vascular injury is explored. This represents yet another disorder associated with moyamoya disease, which is considered to be a secondary phenomenon of vascular occlusion that occurs under a specific set of circumstances in a susceptible age group.

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