Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
3041963 | Clinical Neurology and Neurosurgery | 2007 | 4 Pages |
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.
Related Topics
Life Sciences
Neuroscience
Neurology
Authors
Moncef Berhouma, Ridha Chekili, Ines Brini, Nidhameddine Kchir, Hafedh Jemel, Souad Bousnina, Moncef Khaldi,