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Brain Dev. 1989;11(5):332-7.

Clinical and electroencephalographical follow-up study of early myoclonic encephalopathy.

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  • 1Division of Pediatric Neurology, Osaka Medical Center, Japan.


We report a clinico-electroencephalographical follow-up study on a male patient with early myoclonic encephalopathy. Frequent massive and fragmentary myoclonic seizures, and myoclonic-clonic seizures were the initial symptoms at the age of 3 days. EEG revealed a suppression-burst pattern at the onset in which burst phases often coincided with myoclonic seizures. Subsequently, non-epileptic erratic myoclonus, various partial seizures and flexor spasms were observed. The partial seizures ceased at around 4 months of age, while the non-epileptic myoclonus and flexor spasms have persisted beyond the age of 6 months. The EEG pattern evolved into atypical hypsarhythmia at two months of age. No specific biochemical or neuroradiological findings were disclosed. His neuropsychiatric development was arrested from the onset. These observations suggest that early myoclonic encephalopathy is an independent epileptic syndrome and that it might be different from early-infantile epileptic encephalopathy described by Ohtahara.

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