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CLINICAL EVALUATION AND MRI FINDINGS IN EARLY INFANTILE EPILEPTIC ENCEPHALOPATHY WITH SUPPRESSION-BURST

English (英語)

The Kobe journal of the medical sciences

34(1)

17-29

1988-02

The clinical courses and a follow-up study on the MRI findings in four cases with early infantile epileptic encephalopathy (EIEE) are reported. The patients consisted of one male and three females. The age at onset was before 15 days on life and the etiology was unknown in all cases. EEG improvement and a decrease in seizure frequency were seen after treatment with ACTH and anticonvulsants in three of the four patients, while no treatment was effective in the other patient who developed Lennox-Gastaut syndrome through West syndrome. Psychomotor development of all patients was severely retarded, and it was impossible for three cases to gain head control until 12 months old. MRI findings revealed dysmyelination of white matter in the cerebrum in three patients and asymmetrical myelination in the other patient. These results suggested that EIEE is based on brain immaturity combined with dysmyelination.

dysmyelination

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