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Title: Hemiconvulsion-hemiplegia-epilepsy evolving to contralateral hemi-Lennox-Gastaut-like phenotype.
Austin Authors: Myers, Kenneth A;Scheffer, Ingrid E ;Archer, John S 
Affiliation: Epilepsy Research Centre, Department of Medicine, The University of Melbourne, Austin Health, Heidelberg, Victoria, Australia
The Florey Institute of Neuroscience and Mental Health, Heidelberg, Victoria, Australia
Department of Paediatrics, The University of Melbourne, Parkville, Victoria, Australia
Division of Child Neurology, Montreal Children's Hospital, McGill University Health Centre, Montreal, Quebec, Canada
Department of Neurology, Royal Children's Hospital, Parkville, Victoria, Australia
Department of Pediatrics, Faculty of Medicine, McGill University, Montreal, Quebec, Canada
Issue Date: 16-Feb-2018 2018-05
Publication information: Brain & development 2018; 40(5): 425-428
Abstract: Hemiconvulsion-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.
DOI: 10.1016/j.braindev.2018.01.005
ORCID: 0000-0001-7831-4593
PubMed URL: 29459061
Type: Journal Article
Subjects: Focal epilepsy
Hemiconvulsion-hemiplegia epilepsy
Lennox-Gastaut syndrome
Tonic seizures
Appears in Collections:Journal articles

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