The ketogenic diet is a medically supervised high-fat, low-carbohydrate diet that has been found useful in patients with refractory epilepsy. It has been shown to be effective in treating multiple seizure types and epilepsy syndromes. In this paper, we review the use of the ketogenic diet in epileptic encephalopathies such as Ohtahara syndrome, West syndrome, Dravet syndrome, epilepsy with myoc...

Mutations in the guanine nucleotide-binding protein (G protein), a activating activity polypeptide O (GNAO1) gene have recently been described in 6 patients with early infantile epileptic encephalopathies. In the present study, we report the phenotype and the clinical course of a 4-year-old female with an epileptic encephalopathy (Ohtahara syndrome) and profound intellectual disability due to a...

Mutations in the guanine nucleotide-binding protein (G protein), α activating activity polypeptide O (GNAO1) gene have recently been described in 6 patients with early infantile epileptic encephalopathies. In the present study, we report the phenotype and the clinical course of a 4-year-old female with an epileptic encephalopathy (Ohtahara syndrome) and profound intellectual disability due to a...

Early infantile epileptic encephalopathy or EIEE (Ohtahara syndrome OS) is a kind of intractable seizure that begins in neonatal age with sudden onset of tonic spasms in series or single suppression-burst S-B in EEG.I Imaging shows anatomic defects such as migration disorders and generalized atrophy" with essentially normal metabolic tests. The seizures often change to West's syndrome (WS) ...

how to cite this article: gunawan pi, lusiana l, saharso d. hemispherectomy procedure in proteus syndrome. iran j child neurol.summer 2016; 10(3):86-90   objective proteus syndrome is a rare overgrowth disorder including bone, soft tissue, and skin. central nervous system manifestations were reported in about 40% of the patients including hemimegalencephaly and the resultant hemicranial hyperpl...