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Table of Contents    
Year : 2021  |  Volume : 69  |  Issue : 5  |  Page : 1449-1450

Favorable Response to Immunotherapy in a Child with Hemi-convulsion Hemiplegia Epilepsy Syndrome

1 Department of Pediatrics, Pt B D Sharma Post Graduate Institute of Medical Sciences, Rohtak, Haryana, India
2 Department of Neurology, Pt B D Sharma Post Graduate Institute of Medical Sciences, Rohtak, Haryana, India

Date of Submission09-Mar-2018
Date of Decision02-Dec-2018
Date of Acceptance15-Apr-2020
Date of Web Publication30-Oct-2021

Correspondence Address:
Jaya Shankar Kaushik
Department of Pediatrics, Pt B D Sharma Postgraduate Institute of Medical Sciences, Rohtak - 124 001, Haryana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.329569

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How to cite this article:
Garg D, Kavitha M, Kaushik JS, Bala K. Favorable Response to Immunotherapy in a Child with Hemi-convulsion Hemiplegia Epilepsy Syndrome. Neurol India 2021;69:1449-50

How to cite this URL:
Garg D, Kavitha M, Kaushik JS, Bala K. Favorable Response to Immunotherapy in a Child with Hemi-convulsion Hemiplegia Epilepsy Syndrome. Neurol India [serial online] 2021 [cited 2021 Dec 2];69:1449-50. Available from:


A 3-year typically developing girl presented with complaints of multiple (5–6 per day) episodes of right focal seizures in the form of brief clonic jerks. She was admitted nine months back with febrile focal status epilepticus requiring valproate, levetiracetam, midazolam infusion, and thiopentone infusion. She had developed paucity in speech output, and right sided hemiparesis at the time of discharge. Subsequently, after an interval of 2–3 months, she developed flurry of right-sided focal seizures. She was on a combination of five antiepileptic drugs at their ceiling doses which included phenytoin, oxcarbazepine, levetiracetam, valproate, and clobazam.

Magnetic resonance imaging (MRI) brain performed at the onset of illness revealed evidence of cerebral edema in the entire left cerebral hemisphere [Figure 1]a and [Figure 1]b with subsequent gliosis and encephalomalacia involving the entire left hemisphere [Figure 1]c. Electroencephalography (EEG) revealed left hemispheric slowing. Her cerebrospinal fluid examination for N-methyl-D-aspartate (NMDA) receptor antibody was negative. Serum voltage gated potassium channel-complex (VGKC) antibody was negative.
Figure 1: MRI brain T2-weighted images (a) showing gyral hyperintensity in the entire left hemisphere with diffusion restriction evident on DWI (b) with subsequent repeated MRI brain showing left hemispheric atrophy (c)

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The sequence of focal febrile status epilepticus followed by hemiparesis and subsequent ipsilateral focal epilepsy with contralateral hemispheric edema followed by gliosis favors a clinical diagnosis of hemiconvulsion hemiplegia epilepsy (HHE) syndrome. She was administered intravenous immunoglobulin (2 g/kg over 5 days) along with pulse intravenous methylprednisolone (30 mg/kg/day) for five days after ruling out latent tuberculosis. She was discharged on oral steroids for three weeks followed by tapering over next one week. There was marked a reduction in seizure frequency to 1–2 episodes per month for the first 3 months. Subsequently, she has achieved seizure freedom till 12 months of follow-up on levetiractem and oral clobazam.

HHE syndrome is characterized by prolonged unilateral convulsion followed by transient or permanent hemiparesis and epilepsy.[1] Epilepsy in HHE is often drug resistant requiring multiple antiepileptic drugs and surgical intervention [Table 1]. Pathophysiology of HHES is poorly understood. Plausible explanations for refractory seizures in HHES include prolonged status induced hippocampal injury, role of HHV-6 virus, and underlying genetic predisposition (CACNA1A).[2] An underlying immune basis is emerging in an increasing number of epileptic and encephalopathic syndromes.[3] Acute inflammation with cytokine cascade activation has been proposed in the acute phase of idiopathic HHES.[4] Inflammatory, degenerative changes have been shown histopathologically among children with HHES. In addition, therapy with adrenocorticotropic hormone (ACTH) in a child with idiopathic HHE had shown a favorable response in control of delayed epileptic seizures.[5],[6],[7],[8],[9],[10],[11]
Table 1: Treatment outcome among children with hemiconvulsion hemiplegia epilepsy syndrome (HHES)

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Immunomodulatory properties of steroids and intravenous immunoglobulin could probably decrease neuroinflammation that perpetuates the following onset of epilepsy in HHE as in our case. The present case is probably the first to describe short term favorable outcome following combined immunotherapy in a child with idiopathic HHE. This brings out a potential option of immunotherapy in children with refractory seizures in HHE syndrome. However, this anecdotal experience needs to be further explored in longitudinal studies.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Bajaj S, Tullu MS, Agrawal M. Hemiconvulsion-hemiplegia syndrome. Neurol India 2016;64:1094.  Back to cited text no. 1
[PUBMED]  [Full text]  
Auvin S, Bellavoine V, Merdariu D, Delanoë C, Elmaleh-Bergés M, Gressens P, et al. Hemiconvulsion–hemiplegia–epilepsy syndrome: Current understandings. Eur J Paediatr Neurol 2012;16:413-21.  Back to cited text no. 2
Pradhan S, Das A, Mani VE. Immunotherapy in autoimmune encephalitis-A need for “presumptive” diagnosis and treatment. Neurol India 2018;66:1584.  Back to cited text no. 3
Nabbout R. Autoimmune and inflammatory epilepsies: Immunity and inflammation in epilepsies. Epilepsia 2012;53:58-62.  Back to cited text no. 4
Shimakawa S, Nomura S, Ogino M, Fukui M, Kashiwagi M, Tanabe T, et al. ACTH therapy on intractable epilepsy in Hemiconvulsion–Hemiplegia–Epilepsy syndrome. Brain Dev 2015;37:733-7.  Back to cited text no. 5
Barcia G, Desguerre I, Carmona O, Barnerias C, Chemaly N, Gitiaux C, et al. Hemiconvulsion-hemiplegia syndrome revisited: Longitudinal MRI findings in 10 children. Dev Med Child Neurol 2013;55:1150-8.  Back to cited text no. 6
Van Toorn R, Janse van Rensburg P, Solomons R, Ndondo AP, Schoeman JF. Hemiconvulsion-hemiplegia-epilepsy syndrome in South African children: Insights from a retrospective case series. Eur J Paediatr Neurol 2012;16:142-8.  Back to cited text no. 7
Kim DW, Kim K-K, Chu K, Chung C-K, Lee SK. Surgical treatment of delayed epilepsy in hemiconvulsion-hemiplegia-epilepsy syndrome. Neurology 2008;70:2116-22.  Back to cited text no. 8
Serino D, Camassei FD, Delalande O, Marras CE, Specchio N, Vigevano F, et al. Hemiconvulsion-Hemiplegia-Epilepsy syndrome associated with inflammatory-degenerative hystopathological findings in child with congenital adrenal hyperplasia. Eur J Paediatr Neurol 2014;18:416-9.  Back to cited text no. 9
Berhouma M, Chekili R, Brini I, Kchir N, Jemel H, Bousnina S, et al. Decompressive hemicraniectomy in a space-occupying presentation of hemiconvulsion-hemiplegia-epilepsy syndrome. Clin Neurol Neurosurg 2007;109:914-7.  Back to cited text no. 10
Albakaye M, Belaïdi H, Lahjouji F, Errguig L, Kuate C, Maiga Y, et al. Clinical aspects, neuroimaging, and electroencephalography of 35 cases of hemiconvulsion-hemiplegia syndrome. Epilepsy Behav 2018;80:184-90.  Back to cited text no. 11


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