EEG FINDINGS IN ACETAZOLAMIDE-RESPONSIVE HEREDITARY PAROXYSMAL ATAXIA

EEG studies were performed in six family members affected by acetazola mide-responsive paroxysmal ataxia. Intermittent rhythmic delta activit y was found at rest in five of them; low amplitude spikes were associa ted with delta waves in two cases, resulting in irregular spike and wa ve patterns. Slowing of background activity was present in three patie nts. EEG abnormalities were activated by hyperventilation and modified neither by intermittent photic stimulation, nor by acetazolamide ther apy. Our results suggest that EEG may be helpful to recognize this rar e, but well defined, treatable disorder.