• Moncef Berhouma, Ridha Chekili, Ines Brini, Nidhameddine Kchir, Hafedh Jemel, Souad Bousnina, and Moncef Khaldi.
• Department of Neurosurgery, National Institute of Neurology, Rue Jebbari 1007 La Rabta, Tunis, Tunisia.
• Clin Neurol Neurosurg. 2007 Dec 1; 109 (10): 914-7.

AbstractA 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.

34 keywords...

hide…