BACKGROUND AND PURPOSE Hemimegalencephaly is a rare but well-known congenital malformation with ipsilateral enlargement of the hemicerebrum. However, very little is known about changes in structures outside the involved hemisphere in patients with this condition. We investigated morphologic abnormalities occurring outside the affected hemisphere by MR imaging in a large series of patients with ...

BACKGROUND AND PURPOSE In hemimegalencephaly, MR imaging often reveals midsagittal bandlike structures between the 2 lateral ventricles. To determine whether these structures are aberrant midsagittal fibers, we retrospectively reviewed them on conventional MR imaging and prospectively examined them by diffusion tensor MR and fiber tract (FT) reconstruction imaging. MATERIALS AND METHODS We re...

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 hyperplasia, convulsions and mental deficiency. We report a 1-month-old male baby referred to Pediatric Neurology Clinic Soetomo Hospital, Surabaya, Indonesia in...

Hemimegalencephaly on magnetic resonance imaging scan (MRI) consists of cortical gray matter almost uniformly abnormal, areas of increased thickness of the cortical gray matter (GM), abnormal gyral patterns, blurring of the grey-white matter transition, atrophy or hemispheric hypertrophy, demyelination, gliosis. We present a case of ten-year-old boy with a history of infantile spasms and develo...

A 15-month-old girl presented with developmental delay, intractable right focal motor seizures, hyperpigmented patch on the left side of the face, facial asymmetry, and right hemiparesis (figure 1). A diagnosis of linear nevus sebaceous syndrome (LNSS) was considered. Brain MRI revealed hemimegalencephaly (figure 2). EEG showed spike/wave discharges in left occipital leads. Ophthalmologic and s...

A girl with Klippel-Trenaunay-Weber syndrome with partial motor seizures is reported. She had hemimegalencephaly and band heterotopia on MRI of the brain.