Rare cause of dementia in childhood

Abstract

AbstractBackgroundDementia is rare in paediatric age group. While inborn errors of metabolism account for majority of the causes, structural abnormalities are also responsible for cognitive decline in few patients. We present such a rare case here.MethodAn eight year old female presented to Neurology department with complaints of decline in memory, poor judgement and social disinhibition since two months with three episodes of focal with impaired awareness seizures. On examination, she had MMSE score of 8/30 and no motor weakness or abnormal reflexes. She was evaluated for her symptoms.ResultMRI showed elongated curvilinear altered signal intensity structure consistent with fat at inter‐hemispheric fissure, pericallosal in location, adjacent to body, isthmus and splenium of corpus callosum indicating pericallosal lipoma (curvilinear type).ConclusionPericallosal lipoma is a rare congenital malformation of brain that can be associated with dysgenesis or agenesis of the corpus callosum. It is usually asymptomatic, but can manifest with seizures, dementia, paralysis or headaches. CT and MR imaging play an important role in making this diagnosis.