CC BY-NC-ND 4.0 · International Journal of Epilepsy 2017; 04(01): 079-086
DOI: 10.1016/j.ijep.2016.12.004
Case report
Thieme Medical and Scientific Publishers Private Ltd.

Clinical, MRI and electrographic characteristics of three children with Hemiconvulsion-Hemiplegia/Hemiconvulsion-Hemiplegia-Epilepsy (HH/HHE) syndrome–A rare childhood epileptic encephalopathy

Yeeshu Singh Sudan
1  Division of Pediatric Neurology, Department of Neurology, Amrita Institute of Medical Sciences, Amrita University, Kochi, India
,
K. P. Vinayan
1  Division of Pediatric Neurology, Department of Neurology, Amrita Institute of Medical Sciences, Amrita University, Kochi, India
,
Arun Grace Roy
1  Division of Pediatric Neurology, Department of Neurology, Amrita Institute of Medical Sciences, Amrita University, Kochi, India
› Author Affiliations
Further Information

Publication History

Received: 01 August 2016

Accepted: 21 December 2016

Publication Date:
06 May 2018 (online)

  

Abstract

Hemi convulsion-Hemiplegia-Epilepsy (HH/HHE) syndrome is a very rare catastrophic epileptic syndrome in childhood which follows a prolonged focal motor status epilepticus in infancy and early childhood. Here we are describing the clinical, MRI and electrographic characteristics along with long term outcome of three children with HH/HHE syndrome. A review of the current literature on HH/HHE syndrome is attempted stressing on the diagnostic features and the neurobiological relationship between prolonged focal motor status epilepticus and subsequent development of HH/HHE syndrome. Early identification of this syndrome may help the treating physician in providing families with a relatively accurate prognosis regarding the functional outcome and subsequent development of epilepsy.