Hemiconvulsion hemiplegia syndrome in a young boy with developmental delay

Hemiconvulsion hemiplegia epilepsy (HHE) syndrome is a rare complication of prolonged focal seizures in children upto 4 years of age. It is usually idiopathic and seen in the setting of febrile seizures in otherwise normal children but less commonly is also associated with structural, infective, traumatic and degenerative diseases that predispose to seizures. It has 3 stages, the first of prolonged focal seizures, then the development of hemiplegia and then followed by final stage of development of epilepsy after a variable latent period. Early recognition and seizure control is important to prevent the development of hemiplegia and intractable epilepsy. We report a child with developmental delay and epilepsy who developed HH syndrome after prolonged unrecognized and difficult to control partial status epilepticus.