Epidermal nevus syndrome is a kind of neurocutaneous syndrome that is associated with epidermal nevus and a variety of congenital CNS disorders. Clinical presentations include seizures, paresis, mental retardation, and developmental delay. We report three cases with MR imaging and magnetoencephalography findings; one patient underwent ictal and interictal single photon emission CT. Both structu...

A 16-year-old woman with hypomelanosis of Ito (HI) was scheduled for surgical correction of a left wrist contracture and left cavus foot deformity under general anesthesia. She had left hemiplegia and right hemianopia, but otherwise normal cerebral function. At the age of 8 months she had undergone right functional hemispherectomy for medically intractable epilepsy caused by right hemimegalence...

There is little doubt that, in the adult, specific brain lesions cause specific language deficits. Yet, brain localizations of linguistic functions are made problematic by several reported cases of normal language in spite of major brain anomalies, mostly, but not exclusively, occurring early in life. The signal cases are hydrocephaly, spina bifida and hemispherectomy. These cases are discussed...

Patients with a porencephalic cyst frequently develop intractable temporal lobe epilepsy (TLE). We report a surgically-treated male patient with intractable mesial TLE (mTLE) secondary to a porencephalic cyst. Although magnetic resonance imaging showed no hippocampal abnormalities, long-term video-electrocorticography revealed seizure onset discharges in the hippocampus. Temporal lobectomy brou...

Hemispherectomy has been performed in the treatment of epilepsy in association with hemiplegia for over 50 years. However, the optimal timing of surgery with respect to age at presentation and the influence of underlying pathology on outcome is only slowly emerging. This study reports on the clinical course and outcomes of 33 children who underwent hemispherectomy at Great Ormond Street Hospita...


 Lennox-Gastaut syndrome (LGS) is a form of severe epileptic encephalopathy in children. LGS with encephalomalacia cysts rare We report six-year-old mentally retarded boy who was referred for an intractable seizure. Seizures were tonic, atonic, and dialeptic frequency. EEG showed generalized SSW discharges 1.5-2 Hz, polyspikes, burst suppression typical LGS. Head MRI cyst the right subcor...