Please use this identifier to cite or link to this item: https://ahro.austin.org.au/austinjspui/handle/1/17157
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dc.contributor.authorMyers, Kenneth A-
dc.contributor.authorScheffer, Ingrid E-
dc.contributor.authorArcher, John S-
dc.date2018-05-
dc.date.accessioned2018-02-22T01:09:09Z-
dc.date.available2018-02-22T01:09:09Z-
dc.date.issued2018-02-16-
dc.identifier.citationBrain & development 2018; 40(5): 425-428-
dc.identifier.urihttps://ahro.austin.org.au/austinjspui/handle/1/17157-
dc.description.abstractHemiconvulsion-hemiplegia-epilepsy (HHE) involves infantile-onset acute hemiconvulsive febrile status epilepticus with subsequent unilateral cerebral atrophy and hemiparesis. Chronic epilepsy later develops, typically involving refractory focal seizures; however, the underlying pathophysiology of this epilepsy is not well understood. We present a boy who had a typical acute presentation of HHE at 23 months, but an unusual evolution to chronic epilepsy in which the initially unaffected hemisphere was significantly abnormal. His initial acute presentation was right-sided hemiconvulsive febrile status epilepticus, with subsequent left cerebral hemiatrophy and hemiparesis affecting the right face, arm and leg. Focal seizures began at 5 years and were refractory to medical treatment. At 9 years, video EEG monitoring showed a striking pattern of interictal slow spike-wave and paroxysmal fast activity, maximal over the right, initially unaffected, hemisphere. He had primarily focal tonic seizures involving left-sided stiffening, also appearing to originate from the right hemisphere. Following left functional hemispherotomy he became seizure-free and parents reported improved cognitive function, attention and quality of life. This boy had classic features of Lennox-Gastaut syndrome, but expressed almost exclusively over the right hemisphere, which was initially unaffected in his acute presentation of HHE. His evolution to "hemi-Lennox-Gastaut-like phenotype" illustrates the importance of monitoring chronic epilepsy in patients with HHE; early surgical intervention might prevent pathologic recruitment of bilateral secondary networks leading to the refractory seizures and cognitive impairment associated with Lennox-Gastaut syndrome.-
dc.language.isoeng-
dc.subjectFocal epilepsy-
dc.subjectHemiconvulsion-hemiplegia epilepsy-
dc.subjectLennox-Gastaut syndrome-
dc.subjectTonic seizures-
dc.titleHemiconvulsion-hemiplegia-epilepsy evolving to contralateral hemi-Lennox-Gastaut-like phenotype.-
dc.typeJournal Article-
dc.identifier.journaltitleBrain & development-
dc.identifier.affiliationEpilepsy Research Centre, Department of Medicine, The University of Melbourne, Austin Health, Heidelberg, Victoria, Australia-
dc.identifier.affiliationThe Florey Institute of Neuroscience and Mental Health, Heidelberg, Victoria, Australia-
dc.identifier.affiliationDepartment of Paediatrics, The University of Melbourne, Parkville, Victoria, Australia-
dc.identifier.affiliationDivision of Child Neurology, Montreal Children's Hospital, McGill University Health Centre, Montreal, Quebec, Canada-
dc.identifier.affiliationDepartment of Neurology, Royal Children's Hospital, Parkville, Victoria, Australia-
dc.identifier.affiliationDepartment of Pediatrics, Faculty of Medicine, McGill University, Montreal, Quebec, Canada-
dc.identifier.doi10.1016/j.braindev.2018.01.005-
dc.identifier.orcid0000-0001-7831-4593-
dc.identifier.orcid0000-0002-2311-2174-
dc.identifier.pubmedid29459061-
dc.type.austinJournal Article-
local.name.researcherArcher, John S
item.openairecristypehttp://purl.org/coar/resource_type/c_18cf-
item.cerifentitytypePublications-
item.fulltextNo Fulltext-
item.grantfulltextnone-
item.languageiso639-1en-
item.openairetypeJournal Article-
crisitem.author.deptEpilepsy Research Centre-
crisitem.author.deptEpilepsy Research Centre-
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