Berhouma Moncef, Chekili Ridha, Brini Ines, Kchir Nidhameddine, Jemel Hafedh, Bousnina Souad, Khaldi Moncef
Department of Neurosurgery, National Institute of Neurology, Rue Jebbari 1007 La Rabta, Tunis, Tunisia.
Clin Neurol Neurosurg. 2007 Dec;109(10):914-7. doi: 10.1016/j.clineuro.2007.07.027. Epub 2007 Sep 17.
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.
本文描述了一例需要紧急进行减压性颅骨切除术的急性危及生命的偏瘫-偏瘫-癫痫（HHE）综合征病例。一名9个月大的婴儿在持续发热后出现癫痫持续状态，导致昏迷，并伴有右侧瞳孔散大及左侧偏瘫。磁共振成像（MRI）显示右侧半球肿胀，伴有明显的颞叶疝。该婴儿接受了减压性右侧颅骨切除术及颞叶皮质活检。术后过程顺利。组织学检查结果无特异性，显示为胶质海绵状变性伴散在颗粒细胞。术后MRI显示右侧半球萎缩。据我们所知，此前从未描述过需要手术减压的HHE综合征占位性表现，仅有少数报告涉及该综合征的神经病理学方面。
