EEG studies were performed in six family members affected by acetazolamide-responsive paroxysmal ataxia. Intermittent rhythmic delta activity was found at rest in five of them; low amplitude spikes were associated with delta waves in two cases, resulting in irregular spike and wave patterns. Slowing of background activity was present in three patients. EEG abnormalities were activated by hyperventilation and modified neither by intermittent photic stimulation, nor by acetazolamide therapy. Our results suggest that EEG may be helpful to recognize this rare, but well defined, treatable disorder.