Publication: Hemiconvulsion-Hemiplegia-Epilepsy Syndrome in a Girl Presented with Complex Partial Seizures
Date
2017
Journal Title
Journal ISSN
Volume Title
Publisher
Kathmandu University
Abstract
ABSTRACT
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 hemiconvulsion-
hemiplegia-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.
KEY WORDS
Hemiconvulsion-Hemiplegia-Epilepsy syndrome, Hemispheric brain atrophy, Status
epilepticus, Unilateral brain edema
Description
Joshi A, Shrestha PS, Dangol S, Shrestha NC, Poudyal P, Shrestha A
Department of Pediatrics
Dhulikhel Hospital, Kathmandu University Hospital,
Dhulikhel, Kavre, Nepal
Keywords
Hemiconvulsion-Hemiplegia-Epilepsy syndrome, Hemispheric brain atrophy, Status epilepticus, Unilateral brain edema