Periventricular heterotopia

Periventricular nodular heterotopia (PNH) is a malformation of cortical development due to failure of migration of neurons from the ventricular zone to the cortex during embryonic development, typically characterized by nodules of ectopic neurons lining the lateral ventricles, which can be identified on brain MRI. Clinical manifestations are heterogeneous and nonspecific, with the main features being focal seizures and variable degrees of cognitive impairment. Thanks to detailed clinical observations and higher-resolution imaging, it has become increasingly clear that PNH represents a clinically and genetically heterogeneous group of disorders.

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