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CC BY-NC-ND 4.0 · International Journal of Epilepsy 2023; 09(01/02): 019-023
DOI: 10.1055/s-0044-1787988
Case Report

Idiopathic Hemiconvulsion Hemiplegia Epilepsy in an Eight-Year-Old Boy! Is It the Time to Revisit the HHE Definition?

Mahesh Kamate 
1   Department of Pediatric Neurology, KAHER's J N Medical College, Belgaum, Karnataka, India
,
Virupaxi Hattiholi
2   Department of Radiology, KAHER's J N Medical College, Belgaum, Karnataka, India
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Abstract

Idiopathic hemiconvulsion hemiplegia epilepsy (IHHE) syndrome is characterized by the combination of unilateral convulsive status epilepticus, mainly clonic, followed by transient or permanent ipsilateral hemiplegia. It occurs in infants during the course of a nonspecific febrile illness, mainly in the first 2 years of life and in any case before the age of 4 years. Most studies have mentioned that disease occurs in children younger than 4 years. The exact cause of unilateral involvement is not clear. Occurrence of this condition beyond 4 years of age has rarely been described. We report here a previously healthy 8-year-old boy who presented with right hemiclonic seizures for almost 2 weeks occurring along with nonspecific viral illness. He had residual right hemiparesis and his magnetic resonance imaging (MRI) of the brain showed left hemispheric cortical edema with diffusion restriction. The workup for neuroinfections, mitochondriopathy, and MR angiography was negative. The child improved with injection methyl prednisolone pulse therapy along with multiple intravenous and oral antiseizure medications. This case report suggests that IHHE can also occur beyond 4 years of age, and there is a need to revise the diagnostic criteria of IHHE.

Keywords

hemi-convulsion hemiplegia epilepsy - focal status epilepticus - hemiplegia

Author Contributions

M.K. diagnosed the patient and was involved in the management of patients. He will act as the guarantor of the study. V.H. read the MRI images. The final manuscript was approved by all the authors.




Publication History

Article published online:
18 June 2024

© 2024. Indian Epilepsy Society. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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