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CASE REPORT
Year : 2020  |  Volume : 15  |  Issue : 3  |  Page : 274-278
 

Hemiconvulsion-hemiplegia-epilepsy syndrome: A case series


Department of Paediatrics, Shyam Shah Medical College (SSMC), Rewa, Madhya Pradesh, India

Date of Submission15-Feb-2019
Date of Decision18-Nov-2019
Date of Acceptance13-May-2020
Date of Web Publication06-Nov-2020

Correspondence Address:
Dr. Harshit Bhargava
Department of Paediatrics, Gandhi Memorial Hospital (GMH) and Shyam Shah Medical College (SSMC), Rewa, Madhya Pradesh.
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jpn.JPN_27_19

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   Abstract 

Hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome is an uncommon outcome of prolonged focal status epilepticus in childhood. We present four cases with age between 6 months and 3 years diagnosed with HHE syndrome. Two patients were lost to follow-up; other two cases had severe developmental delay and refractory epilepsy. An early diagnosis, a good seizure control, and a better understanding of the underlying mechanisms of HHE are needed to improve the outcome of this condition.


Keywords: Febrile seizure, hemiconvulsion, hemiplegia, status epilepticus


How to cite this article:
Bhargava H, Dwivedi D. Hemiconvulsion-hemiplegia-epilepsy syndrome: A case series. J Pediatr Neurosci 2020;15:274-8

How to cite this URL:
Bhargava H, Dwivedi D. Hemiconvulsion-hemiplegia-epilepsy syndrome: A case series. J Pediatr Neurosci [serial online] 2020 [cited 2020 Nov 24];15:274-8. Available from: https://www.pediatricneurosciences.com/text.asp?2020/15/3/274/300056





   Introduction Top


The hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome is a rare consequence of febrile seizures during childhood. It was first described by Gastaut et al.[1] It is characterized by the presence of prolonged unilateral clonic seizure occurring during febrile illness in a child under 4 years of age. Then a flaccid unilateral hemiplegia with variable duration occurs. After a free interval of variable duration, 85% of the patient developed focal onset seizures with or without impairment of consciousness with temporal location during the next 3 years following the first seizure.[2] Neuroradiological studies show the presence of cytotoxic edema in the affected cerebral hemisphere followed by cerebral atrophy of the same hemisphere without any vascular cause.[3]


   Case 1 Top


A 5-month-old girl former full-term infant delivered vaginally presented with a history of fever followed by seizure followed by unilateral weakness of the body. There was no prior history of seizure and her motor and language development was age appropriate. Her parents stated that she was in her usual state of health and was acting normally, then she developed fever. Her mother later found that she developed seizure after few hours of fever after which the patient came to Gandhi Memorial Hospital (GMH), Rewa with active seizure for which she received intravenous (I/V) midazolam, phenytoin, valproate, and leviteracetam. Following the seizure, the patient developed weakness of the left side of the body. The total duration of seizure approximately was 10–15h. On day 3 of hospital stay, the patient mental status improved but she was noted with the left-sided hemiparesis and no spontaneous speech, then over a period of 4 days patient started improving on antiepileptics. Magnetic resonance imaging (MRI) scans showed diffuse right hemicerebral diffusion restriction, whereas the left cerebral cortex was normal [Figure 1]. MR angiography was carried out, which was normal. As of the last follow-up at 9 months of age, the baby did not had seizures and she was on the maintenance dose of syrup leviteracetam.
Figure 1: MRI findings of Case 1 (diffuse right hemi-cerebral diffusion restriction). (A) ADC coefficient. (B) DWI MRI image showing diffuse diffusion restriction in right hemicortex

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   Case 2 Top


A 1-year-old boy former full-term infant delivered vaginally presented with a history of fever and vomiting followed by seizure and loss of consciousness. This was followed by the unilateral weakness of the left side of the body. There was no prior history of seizure and his motor and language development was age appropriate. His parents stated that he developed high-grade fever associated with vomiting, which were relieved on medication only to reappear. After few hours, the patient developed seizure for which he was admitted to hospital with an active seizure but was not relieved and was referred to GMH, Rewa where he received I/V midazolam, phenytoin, valproate, and leviteracetam. Following the seizure, the patient developed weakness on the left side of the body. The total duration of seizure approximately was 15–20 h. On day 5 of hospital stay patient mental status improved but had the left-sided hemiparesis, then over a period of 15 days the patient started improving on antiepileptics. MRI scans showed diffuse right hemicerebral diffusion restriction, whereas the left cerebral cortex was normal [Figure 2]. The patient was discharged on the maintenance dose of valproate (30 mg/kg/d) and clobazam on which his seizures were well controlled. But after 1 year he again presented at the hospital with complaints of fever, vomiting, seizures, and altered consciousness. He was given I/V valproate (50 mg/kg/d) with leviteracetam and clobazam was stopped following which he recovered in 2 days. He was given oral carbamazepine (10 mg/kg/d) with valproate but developed carbamazepine induced hyponatremia so was shifted on oral leviteracetam with valproate (50 mg/kg/d) after which seizures were well controlled and the patient was discharged. As of the last follow-up at 2 years of age, seizures were still persisting and the child was diagnosed as refractory epilepsy and was advised to have a ketogenic diet.
Figure 2: MRI findings of Case 2

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   Case 3 Top


Another 3-year-old boy former full-term infant delivered vaginally presented with a history of fever followed by seizure and loss of consciousness. As in other cases there was no prior history of seizure and his motor and language development was age appropriate. As per his parents, he developed high-grade fever which was followed by abnormal body movements involving the right side of the body and loss of consciousness. He was admitted to a local hospital with active seizure for 5 days but was not relieved and was referred to GMH, Rewa where he received I/V midazolam, phenytoin, valproate, and leviteracetam. The total duration of seizure approximately was 5 days. On day 8 of hospital stay, the patient became conscious but had the right-sided hemiparesis and dysarthria. MRI scans showed similar findings as in other cases [Figure 3] and [Figure 4]. The patient was clinically stable on maintenance doses of valproate (30 mg/kg/d) and leviteracetam.
Figure 3: MRI findings of Case 3

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Figure 4: Epileptiform discharges in left hemicortex in EEG of Case 3

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   Case 4 Top


The fourth case was a one and a half year-old girl, former preterm very low birth weight (1.2kg) infant delivered vaginally to an eclamptic mother, presented with a history of fever associated with seizure and loss of consciousness. Like other cases there was no prior history of seizure and development was age appropriate. As stated by her parents, she developed high-grade fever which was followed by abnormal body movements involving all four limbs and loss of consciousness. She was admitted to a local hospital with active seizure for 6 days where she was diagnosed with cerebral malaria but seizures were not controlled, and then she was referred to GMH, Rewa where she received I/V midazolam, phenytoin, and valproate. Following the seizure, the patient developed weakness on the left side of the body. The total duration of seizure approximately was 6 days. On day 9 of hospital stay, the patient became conscious but had the left-sided hemiparesis. MRI scans were similar to other cases. The patient was kept on a maintenance dose of valproate (30 mg/kg/d) on which seizures were well controlled.


   Discussion Top


Children with HHE are usually less than 4 years of age with a history of concurrent febrile illness. HHE syndrome is of two types: idiopathic and symptomatic. Idiopathic is associated with fever and extracranial infection, whereas the symptomatic one is associated with fever and predisposing factors such as head trauma or cerebrovascular disease. HHE has the following characteristics: (1) duration of hours and at times more than 24h, (2) variable location of the jerking, with a possibility of contralateral seizures if it is prolonged, (3) inconstancy of impairment of consciousness, (4) variable onset with possible head/eye deviation, unilateral jerking, or bilateral jerks evolving to unilateral jerks, and (5) the possibility of severe autonomic symptoms such as hypersalivation, respiratory disorders, and cyanosis.

HHE results in cerebral edema followed by cerebral atrophy and chronic epilepsy many times, spongiosis, and disruption of normal cellular architecture. The earliest changes seen are edema of the subcortical white matter in the affected hemisphere.[4] This is followed by the development of global cerebral hemiatrophy over weeks to months.[5] An important distinguishing feature from stroke is that these abnormalities are independent of any vascular territory. In young children, brain is immature which develops unilateral ictal discharges in the case of HHE; this ictal activity causes excessive neuronal activity via N-methyl-D-aspartate (NMDA) receptor, which causes increased calcium causing cytotoxic edema and then necrosis. Recent association with CACNA1A mutation 1q43q44 deletion syndrome, and possible cerebral vasospasm has been documented in HHE.[6],[7] We could not find a study related to any particular infection and development of HHE syndrome, but one of our cases had malaria as a prodromic infection [Table 1].
Table 1: Demographic and clinical data of the cases

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MRI and MR angiography

MRI shows cerebral edema of subcortical white matter, prominent diffusion restriction within the internal capsule, basal ganglia, and portions of thalamus. MR angiography used to be normal and so in these cases.

Treatment for HHE syndrome during the early acute phase of the illness is mainly supportive and in the short term most children do well once the initial status epilepticus is controlled. However, it has been proposed, based on radiologic and pathologic findings, that the use of anti-edema agents and NMDA-type glutamate receptor antagonists during the acute period could help to stop neuronal injury.[8] No guidelines exist as to whether children with HHE syndrome should be on chronic anticonvulsant medication to prevent the remote seizures, although three of our four cases required long-term preventive antiepileptic therapy [Table 1]. There is evidence that surgical treatment of delayed intractable epilepsy in HHE syndrome is beneficial.


   Conclusion Top


HHE syndrome is a rare entity; early diagnosis and intervention help in better outcome and rehabilitation of the child. HHE crosses three stages, first of prolonged focal seizures, then development of hemiplegia, and then development of epilepsy. Early recognition and seizure control are important to prevent development of hemiplegia and intractable epilepsy.[9] It is also evident that all cases in our study presented during the peak season of infectious diseases (especially viral infections and malaria). This suggests infectious etiology as a possible predisposing factor, which needs further evaluation in the future.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Gastaut H, Poirier F, Payan H, Salamon G, Toga M, Vigouroux M H.H.E. syndrome: hemiconvulsions, hemiplegia, epilepsy. Epilepsia 1960;1:418-47.  Back to cited text no. 1
    
2.
Nasir M, Jehangir M, Ahmed RN, Khan E, Ahmad A Hemiconvulsion hemiplegia syndrome in a young boy with developmental delay. J Pak Med Assoc 2018;68:656-9.  Back to cited text no. 2
    
3.
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. 3
    
4.
Toldo I, Calderone M, Boniver C, Dravet Ch, Guerrini R, Laverda AM Hemiconvulsion-hemiplegia-epilepsy syndrome: early magnetic resonance imaging findings and neuroradiological follow-up. Brain Dev 2007;29:109-11.  Back to cited text no. 4
    
5.
Sankhyan N, Sharma S, Kamate M, Subramanian S Teaching neuroimage: Hemiconvulsion-hemiplegia-epilepsy syndrome: sequential MRI follow-up. Neurology 2008;71:e28.  Back to cited text no. 5
    
6.
Yamazaki S, Ikeno K, Abe T, Tohyama J, Adachi Y Hemiconvulsion-hemiplegia-epilepsy syndrome associated with CACNA1A S218L mutation. Pediatr Neurol 2011;45: 193-6.  Back to cited text no. 6
    
7.
Robinson JE, Wolfe SM, Kaiser-Rogers K, Greenwood RS Stroke-like presentation following febrile seizure in a patient with 1q43q44 deletion syndrome. Front Neurol 2016;7:67.  Back to cited text no. 7
    
8.
Auvin S, Devisme L, Maurage CA, Soto-Ares G, Cuisset JM, Leclerc F, et al. Neuropathological and MRI findings in an acute presentation of hemiconvulsion-hemiplegia: a report with pathophysiological implications. Seizure 2007;16:371-6.  Back to cited text no. 8
    
9.
Kim DW, Kim KK, Chu K, Chung CK, Lee SK Surgical treatment of delayed epilepsy in hemiconvulsion-hemiplegia-epilepsy syndrome. Neurology 2008;70:2116-22.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1]



 

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    Abstract
   Introduction
   Case 1
   Case 2
   Case 3
   Case 4
   Discussion
   Conclusion
    References
    Article Figures
    Article Tables

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