Decompressive hemicraniectomy in a space-occupying presentation of hemiconvulsion-hemiplegia-epilepsy syndrome.
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.
Berhouma
Moncef
M
Department of Neurosurgery, National Institute of Neurology, Rue Jebbari 1007 La Rabta, Tunis, Tunisia.
Chekili
Ridha
R
Brini
Ines
I
Kchir
Nidhameddine
N
Jemel
Hafedh
H
Bousnina
Souad
S
Khaldi
Moncef
M
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