Longitudinal MR findings in a patient with hemimegalencephaly associated with tuberous sclerosis.
A patient with an unusual association between tuberous sclerosis complex and hemimegalencephaly is reported. At 2 days of age, CT scan disclosed right hemimegalencephaly with periventricular faint calcification. At 1 month of age, magnetic resonance imaging showed right hemimegalencephaly with T1 and T2 shortening. The right frontal and parietal lobes were compatible with transmantle dysplasia. At 6 months of age, the right hemisphere had decreased in size. Atrophic changes continued to progress until 3 years of age. Periventricular calcification in the right hemisphere became marked with age. The patient developed frequent partial motor seizures from 6 weeks of age and infantile spasms from 5 months of age. Electroencephalogram revealed low amplitude of background activities in the right frontal area. The cause of this atrophic change in the hemimegalencephalic hemisphere, which could be a hamartomatous lesion, remains unknown.
Sakuma
Hiroshi
H
Department of Child Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders, NCNP, 4-1-1 Ogawahigashi-cho, Kodaira, Tokyo 187-8551, Japan.
Iwata
Ohsuke
O
Sasaki
Masayuki
M
D001284:Atrophy; D002540:Cerebral Cortex / Q000002:abnormalities*; D004569:Electroencephalography; D020938:Epilepsy, Partial, Motor / Q000209:etiology / Q000473:pathology; D006801:Humans; D007223:Infant; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging; D008297:Male; D013036:Spasms, Infantile / Q000209:etiology / Q000473:pathology; D014057:Tomography, X-Ray Computed; D014402:Tuberous Sclerosis / Q000150:complications / Q000473:pathology*