Abstract

Multiple-system atrophy is a neurologic disorder characterized by orthostatic hypotension, Parkinsonian signs, and cerebellar signs. Mutations in COQ2, an enzyme involved in coenzyme Q10 (CoQ10) synthesis were recently associated with familial and sporadic cases of multiple-system atrophy. I hypothesized that people with symptoms suggesting multiple-system atrophy might benefit from CoQ10 administration. Five patients with symptomatic orthostatic hypotension were treated in an unrandomized manner with 240±40 mg CoQ10 daily for 8±3 months - two had Parkinsonian and one had cerebellar signs. Prior to starting CoQ10, systolic BP fell 30±5 mmHg upon standing from a sitting position. After treatment with CoQ10, systolic BP fell 8±5 mmHg upon standing from a sitting position (p=0.013 for change in systolic BP fall by paired t test). These data suggest that orthostatic hypotension could possibly improve with CoQ10 administration and that a randomized clinical trial to test this hypothesis should be begun.

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