Outcome of bilateral periventricular nodular heterotopia in monozygotic twins with megalencephaly.
Bilateral periventricular nodular heterotopia (BPNH) is a neuronal migration disorder, characterized by grey-matter cellular rests in the periventricular regions. Patients frequently present with seizures during childhood or later in life. Monozygotic twin sisters were found to have enlarged head circumferences (>95th centile) during a routine medical visit at 6 months of age. A brain CT scan revealed grey-matter cellular rests in the subependymal regions extending to the subcortical white matter. In some areas these cell aggregates had nodular margins, consistent with the diagnosis of BPNH. At 6 years of age they are cognitively and neurologically normal. They have not had any seizures and their heads continue to grow along the 95th centile.
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Department of Paediatrics, King Abdulaziz University Hospital, Kingdom of Saudi Arabia.
D001927:Brain Diseases / Q000000981:diagnostic imaging / Q000473:pathology*; D002465:Cell Movement; D002508:Cephalometry; D002552:Cerebral Ventricles / Q000002:abnormalities*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D012640:Seizures / Q000209:etiology; D014057:Tomography, X-Ray Computed; D014430:Twins, Monozygotic