Article
Bilateral tonic-clonic seizure and focal cortical hyperexcitability in familial Creutzfeldt-Jakob disease with E200K mutation of the prion protein.
Epileptic disorders : international epilepsy journal with videotape - 1 Jun 2023
Kawaguchi Norihiko, Motoda Atsuko, Terada Tatsuhiro, Usui Naotaka, Terada Kiyohito, Matsubara Tomoyasu, Sato Katsuya, Kitamoto Tetsuyuki, Murayama Shigeo, Obi Tomokazu
Abstract excerpt
Convulsive epileptic seizures are rare in Creutzfeldt-Jakob disease (CJD), and their clinical and EEG features have not been reported in detail. We describe a case of familial CJD with an E200K mutation of the prion protein who presented with bilateral tonic-clonic seizures (BTCS) during long-term video-EEG monitoring. Semiologically, BTCS showed focal clinical signs such as head turning and eye deviation to the...
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