Bilateral temporal lobe epilepsy confirmed with intracranial EEG in chorea-acanthocytosis

Chorea-acanthocytosis (ChAc) is an uncommon basal ganglia disorder, in which the movement disorder element may be obscured by the predominance of seizures. We report a pertinent case of a patient who had undergone extensive evaluation for epilepsy, including intracranial EEG before finally the diagnosis of ChAc was made and confirmed by Western blot. We suggest that in patients with epilepsy, particularly of temporal lobe origin and with onset in the third decade with inconclusive findings on clinical examination and neuroimaging such as dyskinesias, dystonia and basal ganglia involvement, ChAc should be considered.