A CASE OF EARLY INFANTILE EPILEPTIC ENCEPHALOPATHY (EIEE) WITH ANATOMICAL CEREBRAL ASYMMETRY AND MYOCLONUS

The authors report a case of early encephalopathy with myoclonus, toni c spasms and a suppression-burst pattern on electroencephalography (EE G) associated with unilateral cerebral hypertrophy following hemiatrop hy. This patient showed frequent myoclonus in relation to a suppressio n-burst pattern resembling that in early myoclonic encephalopathy (EME ). Moreover, the case also showed tonic spasms, from the age of 13 day s, in series, as seen in Ohtahara syndrome. On the other hand, there w as a previously undescribed peculiar CT scan finding, which showed hyp ertrophy of the right cerebral hemisphere at birth, following hemiatro phy. Neuropathological examination revealed cerebral atrophy associate d with heterotopia and an ependymal hyperplasia in the right hemispher e, suggesting hemimegalencephaly. This case should be classified as Oh tahara syndrome accompanied by myoclonus, because of the spasms in ser ies interrupting the suppression-burst pattern, and the etiological fa ctor of brain malformation. The nosological aspects of this epileptic encephalopathy are discussed.