Document Detail

Hemiconvulsion-hemiplegia-epilepsy syndrome. A clinical, electroencephalographic and neuroradiological study.
MedLine Citation:
PMID:  9224914     Owner:  NLM     Status:  MEDLINE    
Six patients (4 boys and 2 girls) with hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome are described. They had prolonged seizures, lasting from 30 min to 12 h, at ages 1-4 years. These took the form of hemiconvulsion in three of the children and generalized tonic-clonic seizures in the others, being preceded by hemifacial twitching or head and eye deviation in two. They were followed by hemiplegia, which cleared with time in five patients, apart from subtle pyramidal tract signs. One child had spastic quadriparesis, choreiform movements, contracture deformities and severe mental retardation following repeated status epilepticus. Subsequent epilepsy developed in five patients and was satisfactorily controlled with carbamazepine and/or phenobarbitone. Cerebral hemiatrophy was documented in all patients by cranial computed tomography and/or magnetic resonance imaging. Single photon emission computed tomography (done in 4 patients) showed ipsilateral hypoperfusion (of the damaged hemisphere). Electroencephalography showed ipsilateral slowing and low voltage of background activity. Epileptiform discharges were found on the ipsilateral side in two cases and the contralateral side (the undamaged hemisphere) in one.
M A Salih; M Kabiraj; A S Al-Jarallah; M El Desouki; S Othman; V A Palkar
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery     Volume:  13     ISSN:  0256-7040     ISO Abbreviation:  Childs Nerv Syst     Publication Date:  1997 May 
Date Detail:
Created Date:  1997-09-22     Completed Date:  1997-09-22     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  8503227     Medline TA:  Childs Nerv Syst     Country:  GERMANY    
Other Details:
Languages:  eng     Pagination:  257-63     Citation Subset:  IM    
Department of Paediatrics, College of Medicine, King Khalid University Hospital, King Saud University, Riyadh, Saudi Arabia.
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MeSH Terms
Brain Mapping
Cerebral Cortex / blood supply,  pathology,  physiopathology
Child, Preschool
Dominance, Cerebral / physiology*
Epilepsy, Tonic-Clonic / diagnosis,  physiopathology*
Hemiplegia / diagnosis,  physiopathology*
Magnetic Resonance Imaging*
Neurologic Examination
Regional Blood Flow / physiology
Seizures / diagnosis,  physiopathology*
Tomography, Emission-Computed, Single-Photon*

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