KUMJ | VOL. 15 | NO. 3 | ISSUE 59 | JULY-SEPT. 2017

Hemiconvulsion-Hemiplegia-Epilepsy Syndrome in a Girl Presented with Complex Partial Seizures
Joshi A, Shrestha PS, Dangol S, Shrestha NC, Poudyal P, Shrestha A

The mechanisms underlying the Hemiconvulsion-Hemiplegia-Epilepsy syndrome remains unclear. The current proposed pathogenic mechanism is a neuronal injury induced by venous thrombosis and/or hypoxia. Children develop hemispheric brain atrophy with contralateral hemiplegia, epilepsy, and a variable degree of cognitive deficit. We report a 33 months old female child a case of hemiconvulsionhemiplegia-epilepsy syndrome with right hemisphere unilateral brain edema and left sided hemiplegia and aphasia who presented with left upper extremities complex partial seizures with generalization to tonic clonic seizures and developed status epilepticus that posed diagnostic and therapeutic challenges. Progressive atrophy of the right cerebral hemisphere was noted after 3 months of follow up. Hemiconvulsion-Hemiplegia-Epilepsy syndrome should be suspected in a child with unilateral cerebral hemisphere brain edema and hemiplegia with cognitive deficit following status epilepticus to provide patients and families with an accurate prognosis regarding the subsequent development of epilepsy.
Keyword : Hemiconvulsion-Hemiplegia-Epilepsy syndrome, Hemispheric brain atrophy, Status epilepticus, Unilateral brain edema