Hemiconvulsion-hemiplegia-epilepsy evolving to contralateral hemi-Lennox-Gastaut-like phenotype
Kenneth A Myers, Ingrid E Scheffer, John S Archer
BRAIN & DEVELOPMENT | ELSEVIER | Published : 2018
BACKGROUND: 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. PATIENT: 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 af..View full abstract
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Awarded by Australian National Health and Medical Research Council (NHMRC)
This work was supported by the Australian National Health and Medical Research Council (NHMRC) (grant numbers 1091593, 1104831, 1108881).