Epileptic Seizures in Dentatorubral-Pallidoluysian Atrophy

Four Cases with Marked Deterioration of Clinical Condition Corresponding to the Appearance of Epileptic Seizures

Abstract

We describe four patients with dentatorubral-pallidoluysian atrophy (DRPLA), who showed marked deterioration involving dementia, choreoathetosis and cerebellar ataxia, corresponding to the appearance of epileptic seizures. The patient in case 1 with the juvenile type, showed clustering of focal and generalized clonic seizures over a one-month period. During this episode, her clinical condition deteriorated, and slowing of background EEG activity and exaggeration of paroxysms was also observed. In the other three cases, who showed the adult type, the clinical conditions deteriorated soon after the clustering of clonic seizures or the appearance of tonicclonic convulsions, making the patients unable to walk, sit, eat or communicate. It is suggested that epileptic seizures may herald acute deterioration of the clinical symptoms and reflect the acute progress of DRPLA pathophysiology.