Subcortical band heterotopia and pachygyria with cognitive deterioration in an elderly patient

Abstract

Subcortical band heterotopia and pachygyria are rare conditions characterized by ectopic neuronal migration, leading to the appearance of "continuous double cortex appearance" and loss of normal convolutions of the cortex, respectively on neuroimaging. They usually present in childhood with disorders of muscle tone, craniofacial dysmorphism, refractory seizures, intellectual disability and feeding disturbances. Patients with such presentations have lowered life expectancy, but many mild cases survive till adulthood. Here we report subcortical band heterotopia and pachygyria – an incidental finding – in the case of an elderly lady with intellectual disability and seizure disorder, who presented with recent onset of cognitive decline and behavioural symptoms.