EARLY INFANTILE EPILEPTIC ENCEPHALOPATHY (EIEE) (OHTAHARAS SYNDROME) WITH POLYMICROGYRIA

A boy, born after normal pregnancy and delivery, began to have fits at 3 days. The seizures were composed of tonic or tonic-clonic convulsio ns at the upper extremities but myoclonus was absent These attacks wer e not easy to control. There was gross developmental delay. Laboratory investigations were almost normal except for cerebrospinal fluid : pl eocytosis and high protein content. EEG showed << suppression-burst >> and MRI revealed high signal intensity in the left temporo-occipital region on T2 weighted image. At three and a half months of age, EEG ch anged into hypsarrhythmia. The child died at 5 months of age. At post mortem neuropathological examination, the cortical ribbon in the bilat eral parieto-occipital regions appeared thick, as if there were pachyg yria. Microscopically polymicrogyria was noted in these areas as well as in the insular cortex. This lesion showed a symmetrical distributio n. The cyloarchitectonic features of the polymicrogyric cortex did not consist of 4 layers. The other structures of the central nervous syst em were almost devoid of lesion. The number of clinico-pathological re ports on Ohtahara's syndrome is very limited and the etiopathogenesis of polymicrogyria is discussed.