title: Survival following decompressive hemicraniectomy for hemiconvulsion-hemiplegia-epilepsy syndrome: case report.
abstract: Hemiconvulsion-hemiplegia-epilepsy (HHE) is an uncommon epileptic syndrome that affects young children. Typical management includes early initiation of benzodiazepines to abate the initial seizure activity quickly. Patients in whom epilepsy develops require prolonged use of antiepileptic agents. Herniation due to diffuse cerebral edema from HHE is rare; however, decompressive craniectomy has been described as a lifesaving measure. The authors present the case of a patient in whom a decompressive craniectomy was performed. They advocate a proactive approach in the detection and management of cerebral edema in HHE causing intracranial hypertension. In HHE cases that exhibit radiographic evidence of malignant cerebral edema (although not previously described in this disease, but similar to the setting of stroke and trauma), the authors advocate early neurosurgical consultation and evaluation for insertion of an intracranial pressure monitor for those patients who do not have a reliable neurological examination (i.e., Glasgow Coma Scale score ≤ 8).
authors.affiliation: Division of Pediatric Neurosurgery, University of Florida Health;
authors.forename: Alexandra D
authors.initials: AD
authors.lastname: Beier
authors.affiliation: Division of Pediatric Neurosurgery, University of Florida Health;
authors.forename: Gemi E
authors.initials: GE
authors.lastname: Jannotta
authors.affiliation: Jacksonville Pathology Consultants, Chief of Pathology; and.
authors.forename: E Dayan
authors.initials: ED
authors.lastname: Sandler
authors.affiliation: Department of Neurology, Nemours Children's Specialty Care, Jacksonville, Florida.
authors.forename: Harry S
authors.initials: HS
authors.lastname: Abram
authors.affiliation: Department of Neurology, Nemours Children's Specialty Care, Jacksonville, Florida.
authors.forename: Raj D
authors.initials: RD
authors.lastname: Sheth
authors.affiliation: Division of Pediatric Neurosurgery, University of Florida Health;
authors.forename: Philipp R
authors.initials: PR
authors.lastname: Aldana
mesh_terms: D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology; D002675:Child, Preschool; D019299:Decompression, Surgical; D004827:Epilepsy / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology / Q000601:surgery*; D005260:Female; D006429:Hemiplegia / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology / Q000601:surgery*; D006801:Humans; D013577:Syndrome
keywords: EEG = electroencephalography; GCS = Glasgow Coma Scale; HHE; HHE = hemiconvulsion-hemiplegia-epilepsy; ICP = intracranial pressure; MRA = MR angiography; epilepsy; hemiconvulsion-hemiplegia-epilepsy syndrome; hemicraniectomy; technique