Epilepsy with Childhood-Onset Dentatorubral-Pallidoluysian Atrophy (DRPLA)

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  • 小児期発病の歯状核・赤核・淡蒼球・ルイ体萎縮症のてんかん
  • electrical statusを伴いミオクローヌスと脱力を主徴とする特異な病像を呈した症例
  • 3 Cases with a Peculiar Clinical Features Characterized by Myoclonus, Atonic Episodes and Electrical Status in Their EEGs

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Abstract

Clinicoelectrical features of epilepsy in 3 patients with childhood onset were invesitigated from a family with 7 patients of DRPLA, and the following results were obtained.<BR>(1) A peculiar clinical features with frequent myoclonus and atonic episodes resembling nonconvulsive status epilepticus (NCS), were observed from an early stage of illness.<BR>Within several years, generalized tonic or tonic clonic seizures, sometimes associated with convulsive status, developed and subsequently, mental regression became apparent.<BR>(2) Interestingly, their sequential EEGs revealed the electrical status epilepticus with almost continuous diffuse spike-and-wave discharges during the periods of NCS in all patients. With the progress of the disease, diffuse spike-and-wave discharges evolved into multifocal spikes, and progressive slowing of background activity became evident.<BR>In conclusion, intractable epilepsy was the early and major features in childhood onset DRPLA, and the DRPLA is one of the most important underlying pathology for PME syndrome, especially in Japan. NCS allied conditions should be paid attention as the initial symptom of DRPLA.

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