hemiplegia-hemiconvulsion-epilepsy syndrome

hemiplegia-hemiconvulsion-epilepsy syndrome

A condition characterised by the abrupt onset of unilateral clonic epileptic seizures (immediately followed by flaccid hemiplegia ipsilateral to the clonic seizures) of prolonged duration following febrile illness, which occur in children under age 4.

Imaging
Enlargement of cerebral hemisphere with sulcal effacement and diffuse white matter hyperintensities on T2 weighted images, and restricted diffusion suggestive of cytotoxic oedema. Follow-up imaging shows severe gliosis and unilateral cortical and the subcortical atrphy of cerebral hemisphere, with dilatation of the ipsilateral ventricular system.