Persistent superior oblique paresis as a manifestation of familial periodic cerebellar ataxia.

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A brother and sister complained of persistent diplopia due to superior oblique palsies. The cause of their symptoms became apparent when they were diagnosed as having familial periodic cerebellar ataxia (FPCA), a rare autosomal dominant condition. Oral acetazolamide (250 mg twice daily) not only prevented all the periodic symptoms but also relieved their diplopia, which had been present between attacks.