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Journal of Pediatric Neurology 2014; 12(02): 109-113
DOI: 10.3233/JPN-140649
Case Report
Georg Thieme Verlag KG Stuttgart – New York

Clinicoradiological description of two children with hemiconvulsion-hemiplegia-epilepsy syndrome

Vykuntaraju K. Gowda
a   Department of Pediatric Neurology, Indira Gandhi Institute of Child Health, Bangalore, Karnataka, India
,
Bhaskar V. Madivala
b   Department of Neuroradiology, National Institute of Mental Health and Neurosciences, Bangalore, Karnataka, India
,
Sukanya Vignesh
c   Department of Pediatrics, Indira Gandhi Institute of Child Health, Bangalore, Karnataka, India
,
Praveen-Kumar Srikanteswara
d   Department of Neurology, Bangalore Medical College and research Institute, Bangalore, India
,
Sarala H. Vykuntaraju
e   Department of Anatomy, Kempegowda Institute of Medical Science, Bangalore, India
,
Premalatha Ramaswamy
c   Department of Pediatrics, Indira Gandhi Institute of Child Health, Bangalore, Karnataka, India
› Author Affiliations
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Publication History

24 November 2013

31 January 2014

Publication Date:
30 July 2015 (online)

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Abstract

Hemiconvulsion-hemiplegia-epilepsy syndrome is an uncommon consequence of prolonged focal febrile convulsive seizures in infancy and early childhood. We report two cases of hemiconvulsion-hemiplegia-epilepsy syndrome who presented acutely and had striking neuroimaging findings suggestive of diffuse cytotoxic edema confined to one hemisphere as documented by magnetic resonance imaging, including extensive diffusion weighted imaging abnormalities in both the cases.

Keywords

HHE syndrome - developmental delay - focal febrile status epilepticus