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 di...
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| Veröffentlicht in: | Clinical neurology and neurosurgery 2007-12, Vol.109 (10), p.914-917 |
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| container_title | Clinical neurology and neurosurgery |
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| creator | Berhouma, Moncef Chekili, Ridha Brini, Ines Kchir, Nidhameddine Jemel, Hafedh Bousnina, Souad Khaldi, Moncef |
| description | 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. |
| doi_str_mv | 10.1016/j.clineuro.2007.07.027 |
| format | Article |
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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.</description><identifier>ISSN: 0303-8467</identifier><identifier>EISSN: 1872-6968</identifier><identifier>DOI: 10.1016/j.clineuro.2007.07.027</identifier><identifier>PMID: 17875361</identifier><identifier>CODEN: CNNSBV</identifier><language>eng</language><publisher>Amsterdam: Elsevier B.V</publisher><subject>Atrophy ; Biological and medical sciences ; Biopsy ; Brain Edema - pathology ; Brain Edema - surgery ; Cerebrum - pathology ; Cerebrum - surgery ; Craniotomy - methods ; Cytotoxicity ; Decompression, Surgical - methods ; Decompressive hemicraniectomy ; Encephalocele - pathology ; Encephalocele - surgery ; Epilepsy ; Family medical history ; Hemiconvulsion–hemiplegia–epilepsy syndrome ; Hemiplegia - pathology ; Hemiplegia - surgery ; Humans ; Infant ; Male ; Medical imaging ; Medical sciences ; MRI ; Neurologic Examination ; Neurology ; Neuropathology ; Neurosurgery ; Pathogenesis ; Postoperative Complications - diagnosis ; Seizures, Febrile - pathology ; Seizures, Febrile - surgery ; Status epilepticus ; Status Epilepticus - pathology ; Status Epilepticus - surgery ; Studies ; Surgery (general aspects). Transplantations, organ and tissue grafts. 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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.</description><subject>Atrophy</subject><subject>Biological and medical sciences</subject><subject>Biopsy</subject><subject>Brain Edema - pathology</subject><subject>Brain Edema - surgery</subject><subject>Cerebrum - pathology</subject><subject>Cerebrum - surgery</subject><subject>Craniotomy - methods</subject><subject>Cytotoxicity</subject><subject>Decompression, Surgical - methods</subject><subject>Decompressive hemicraniectomy</subject><subject>Encephalocele - pathology</subject><subject>Encephalocele - surgery</subject><subject>Epilepsy</subject><subject>Family medical history</subject><subject>Hemiconvulsion–hemiplegia–epilepsy syndrome</subject><subject>Hemiplegia - pathology</subject><subject>Hemiplegia - surgery</subject><subject>Humans</subject><subject>Infant</subject><subject>Male</subject><subject>Medical imaging</subject><subject>Medical sciences</subject><subject>MRI</subject><subject>Neurologic Examination</subject><subject>Neurology</subject><subject>Neuropathology</subject><subject>Neurosurgery</subject><subject>Pathogenesis</subject><subject>Postoperative Complications - diagnosis</subject><subject>Seizures, Febrile - pathology</subject><subject>Seizures, Febrile - surgery</subject><subject>Status epilepticus</subject><subject>Status Epilepticus - pathology</subject><subject>Status Epilepticus - surgery</subject><subject>Studies</subject><subject>Surgery (general aspects). 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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.</abstract><cop>Amsterdam</cop><pub>Elsevier B.V</pub><pmid>17875361</pmid><doi>10.1016/j.clineuro.2007.07.027</doi><tpages>4</tpages></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0303-8467 |
| ispartof | Clinical neurology and neurosurgery, 2007-12, Vol.109 (10), p.914-917 |
| issn | 0303-8467 1872-6968 |
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| source | MEDLINE; Access via ScienceDirect (Elsevier); ProQuest Central UK/Ireland |
| subjects | Atrophy Biological and medical sciences Biopsy Brain Edema - pathology Brain Edema - surgery Cerebrum - pathology Cerebrum - surgery Craniotomy - methods Cytotoxicity Decompression, Surgical - methods Decompressive hemicraniectomy Encephalocele - pathology Encephalocele - surgery Epilepsy Family medical history Hemiconvulsion–hemiplegia–epilepsy syndrome Hemiplegia - pathology Hemiplegia - surgery Humans Infant Male Medical imaging Medical sciences MRI Neurologic Examination Neurology Neuropathology Neurosurgery Pathogenesis Postoperative Complications - diagnosis Seizures, Febrile - pathology Seizures, Febrile - surgery Status epilepticus Status Epilepticus - pathology Status Epilepticus - surgery Studies Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases Temporal Lobe - pathology |
| title | Decompressive hemicraniectomy in a space-occupying presentation of hemiconvulsion–hemiplegia–epilepsy syndrome |
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