title: Decompressive hemicraniectomy in a space-occupying presentation of hemiconvulsion-hemiplegia-epilepsy syndrome.
abstract: A case of an acute life-threatening presentation of hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome requiring an urgent decompressive hemicraniectomy is described. A 9 month-old baby had a status epilepticus following a sustained fever, leading to a comatose state and a right pupillary dilatation associated with a left hemiplegia. The MRI showed a swelling right hemisphere with marked temporal herniation. The baby underwent a decompressive right hemicraniectomy with temporal cortical biopsies. The post-operative course was favourable. The histological findings were unspecific, showing a gliotic spongiosis with disseminated granular cells. The post-operative MRI depicted a right hemisphere atrophy. To our knowledge, a space-occupying presentation of HHE syndrome requiring surgical decompression has never been described before while only a few reports dealt with the neuropathological aspects of this syndrome.
authors: Department of Neurosurgery, National Institute of Neurology, Rue Jebbari 1007 La Rabta, Tunis, Tunisia.
authors: Moncef
authors: M
authors: Berhouma
authors: Ridha
authors: R
authors: Chekili
authors: Ines
authors: I
authors: Brini
authors: Nidhameddine
authors: N
authors: Kchir
authors: Hafedh
authors: H
authors: Jemel
authors: Souad
authors: S
authors: Bousnina
authors: Moncef
authors: M
authors: Khaldi
mesh_terms: D001284:Atrophy; D001706:Biopsy; D001929:Brain Edema / Q000473:pathology / Q000601:surgery*; D054022:Cerebrum / Q000473:pathology / Q000601:surgery*; D003399:Craniotomy / Q000379:methods*; D019299:Decompression, Surgical / Q000379:methods*; D004677:Encephalocele / Q000473:pathology / Q000601:surgery; D006429:Hemiplegia / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D009460:Neurologic Examination; D011183:Postoperative Complications / Q000175:diagnosis; D003294:Seizures, Febrile / Q000473:pathology / Q000601:surgery*; D013226:Status Epilepticus / Q000473:pathology / Q000601:surgery*; D013702:Temporal Lobe / Q000473:pathology