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Table of Contents    
Year : 2017  |  Volume : 65  |  Issue : 3  |  Page : 636-638

Dengue fever triggering hemiconvulsion hemiplegia epilepsy in a child

1 Department of Pediatrics, Division of Child Neurology, All India Institute of Medical Sciences, New Delhi, India
2 Department of Radiodiagnosis, Jai Prakash Narayan Apex Trauma Centre, All India Institute of Medical Sciences, New Delhi, India

Date of Web Publication9-May-2017

Correspondence Address:
Sheffali Gulati
Department of Pediatrics, All India Institute of Medical Sciences, New Delhi - 110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/neuroindia.NI_1367_15

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How to cite this article:
Saini L, Chakrabarty B, Pastel H, Israni A, Kumar A, Gulati S. Dengue fever triggering hemiconvulsion hemiplegia epilepsy in a child. Neurol India 2017;65:636-8

How to cite this URL:
Saini L, Chakrabarty B, Pastel H, Israni A, Kumar A, Gulati S. Dengue fever triggering hemiconvulsion hemiplegia epilepsy in a child. Neurol India [serial online] 2017 [cited 2022 Sep 26];65:636-8. Available from: https://www.neurologyindia.com/text.asp?2017/65/3/636/205901


Hemiconvulsion-hemiplegia-epilepsy (HHE) is a rare outcome of prolonged focal status epilepticus that usually occurs in children below 4 years of age.[1] This entity starts with a focal status epilepticus and concurrent febrile illness, which subsequently evolves to ipsilateral hemiparesis of the convulsing side; at follow-up over months to years, two-third of the patients develop epilepsy. Magnetic resonance imaging (MRI) of the brain in the acute stages characteristically shows gross cerebral edema in the contralateral hemisphere, which later turns into atrophy. The possible etiological triggers are vasculopathy, trauma, inflamation, and viral infections. In situ ations where no trigger is identified, it is labelled as idiopathic.[2] Dengue has never been reported as a possible trigger for HHE. The current case, which was initially managed as a seropositive dengue shock syndrome with acute symptomatic seizures, ultimately evolved in terms of clinical and radiological features to HHE and the sequence of events until the follow up evaluation suggested that dengue was just the inciting trigger.

A 3-year old boy presented with a high-grade fever for 4 days, left-sided focal status epilepticus for the last 24 hours, and laboured breathing for 6 hours. On examination, he was hypotensive, comatose, having papilledema with intermittent posturing suggestive of raised intracranial pressure (ICP). He was mechanically ventilated, and measures to counter raised ICP were started. He was also started on the management of status epilepticus as per the protocol, requiring midazolam infusion upto15 μ/kg/min. On the basis of the initial investigations, dengue shock syndrome was a strong possibility. It was the peak season for dengue, and two of his siblings had already suffered from dengue within the last 3 weeks. He had thrombocytopenia and hemoconcentration and there was evidence of third space fluid loss in the form of bilateral pleural effusion on chest X-ray, and a mild hepatomegaly, gall bladder wall edema, and ascites on ultrasound abdomen. Non-structural protein (NS1) antigen testing for dengue infection was positive. Shock was controlled within 24 hours of hospital stay. Serology for all common infections (malaria, Japanese B encephalitis, West Nile virus, Leptospira, and Rickettsia) were negative. The only exception was the presence of dengue serology, which was positive. Cerebrospinal fluid (CSF) examination and Herpes simplex virus (HSV) polymerase chain reaction (PCR) were also negative. His electroencephalogram (EEG) revealed a generalized slow wave activity [Figure 1], and MRI brain showed extensive edema of the right cerebral hemisphere, scalp edema (indicating third space fluid loss in dengue), and evidence of early transtentorial herniation with a normal MR angiogram [Figure 2]. He was also given intravenous immunoglobulin (IVIG) as Rasmussen's encephalitis too was considered as a possibility. Over the next 5 days, his sensorium improved gradually, his seizures became passive, and he was extubated. At that time, a left hemiparesis was noticed. A repeat MRI of the brain after 3 months was suggestive of right cerebral hemisphere atrophy [Figure 3]. Over the next 1 year, he continued to have left hemiparesis and also developed left focal epilepsy with a frequency of 1 to 2 seizures per month with relatively preserved cognition.
Figure 1: Electroencephalogram showing generalized slow wave activity in bipolar children montagea

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Figure 2: T1-weighted (a), T2-weighted (b), and FLAIR (c) axial MR images of the brain at the time of initial presentation show the diffuse swollen gray matter of the right cerebral hemisphere with hyperintense signal changes on T2 and FLAIR. Note the midline shift towards the left

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Figure 3: Follow-up MRI brain of the patient after 5 months. The T1-weighted (a), T2-weighted (b), and FLAIR (c) axial MR images show atrophy of the right cerebral hemisphere with regression of the signal changes

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Gastaut, for the first time, described the clinical, electrographic, radiologic, and pathologic features of HHE.[1] The initial stage is referred to as HH syndrome (HHS) because epilepsy develops later. HHS is characterized by prolonged (in hours), unilateral, clonic convulsions that can be missed initially.[3] The earliest changes in the brain occurs at the time of status epilepticus, which can be appreciated as a unilateral hemispheric swelling that is seen on MRI of the brain.[4] Over weeks-to-months, cerebral atrophy ensues.[5] The current case also had the same clinicoradiological presentation hence, has been labelled as HHE.

Diagnosis of HHE does not require the presence of any specific EEG findings. In the acute stage, ictal, rhythmic, generalized slow waves (2–3 Hz) with a larger amplitude on the affected side can be appreciated.[3] Spike and slow wave activity can be followed by suppression in the affected hemisphere, whereas in the unaffected hemisphere, epileptic discharges are usually followed by a normal background rhythm.[6] In the index case, generalized slowing was seen in the acute phase, however, there was no asymmetry in terms of amplitude.

Pathological findings described in the literature are spongiosis and disruption of normal cellular architecture, which are postulated to occur secondary to the activation of inflammatory cascade triggered by the various etiological agents,[6] which in the current case was dengue shock syndrome.

The outcome in HHE is variable. After a symptom-free period of months-to-years, approximately two-third of the patients develop intractable epilepsy. The idiopathic ones tend to develop temporal lobe epilepsy, whereas the symptomatic cases have generalized epilepsy. A variable number of patients develop motor deficits as well as cognitive and language impairments in the long run.[2]

The differential diagnoses in the acute phase include stroke, traumatic brain injury, space occupying lesion, infection, demyelination, metabolic disorder, and intracranial malformation.[2] Proper history, clinical examination, neuroimaging findings, and screening for metabolic disorders such as mitochondrial disorders would differentiate HHE from all the above mentioned conditions. In the chronic phase, one needs to rule out Rasmussen encephalitis, which is a disorder of progressive cognitive impairment with epilepsia partialis continua.

Neurological complications occur in 0.5–6% cases of dengue fever, however, HHE has never been described. Three pathogenic mechanisms have been hypothesized: Neurotropism which includes encephalitis, meningitis, and myositis; systemic complications such as stroke and hypokalemic paralysis; and postinfectious immune-mediated mechanism which results in manifestations such as central and peripheral demyelination and opsoclonus myoclonus ataxia.[7] The index case was also possibly due to postinfectious immune-mediated mechanism, which comprises autoimmunity, molecular mimicry, nonspecific activation of autoreactive T cells leading to destruction of the myelin sheath, the presence of a cytokine storm, and activation of the inflammatory cascade. Recently isolated hypoglossal nerve palsy has been described in a patient with dengue.[8] Characteristic imaging findings such as”reverse moustache sign” has been described in dengue encephalitis.[9] In the recent past, pituitary apoplexy has been reported in a 43-year old man with dengue hemorrhagic fever, who had an underlying pituitary macroadenoma.[10]

Thus, the current case unveils a newer etiology leading to HHE as well as a newer neurological complication of dengue virus infection. Anticipation and identification in appropriate clinical and epidemiological scenarios can help in prognostication and planning long-term management.

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There are no conflicts of interest.

 » References Top

Gastaut HPF, Payan H, Salomon G, Toga M, Vigouroux M. HHE syndrome: Hemiconvulsions–hemiplegia–epilepsy. Epilepsia 1960;1:418-7.  Back to cited text no. 1
Tenny JR, Schapiro MB. Child Neurology: Hemiconvulsion-hemiplegia-epilepsy syndrome. Neurology 2012;79:e1-4.  Back to cited text no. 2
Chauvel P, Dravet C. The HHE syndrome. In: Roger J, Bureau M, Dravet C, et al., editors. Epileptic Syndromes in Infancy, Childhood, and Adolescence. Montrouge: John Libbey Eurotext; 2005. pp. 277-93.  Back to cited text no. 3
Toldo I, Calderone M, Boniver C, Dravet Ch, Guerrini R, Laverda AM. Hemiconvulsion hemiplegia-epilepsy syndrome: Early magnetic resonance imaging studies and neuro radiological follow-up. Brain Dev 2007;29:109-11.  Back to cited text no. 4
Sankhyan N, Sharma S, Kamate M, Subramanian S. Hemiconvulsion hemiplegia-epilepsy syndrome: Sequential MRI follow-up. Neurology 2008;71:e28.  Back to cited text no. 5
Auvin S, Devisme L, Maurage CA, Soto-Ares G, Cuisset JM, Leclerc F. 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. 6
Murthy J. Neurological complications of dengue infection. Neurol India 2010;58:581-4.  Back to cited text no. 7
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Jaganathan S, Raman R. Hypoglossal nerve palsy: A rare consequence of dengue fever. Neurol India 2014;62:567-8.  Back to cited text no. 8
[PUBMED]  [Full text]  
Mehta A, Mahale RR, Javali M, Srinivasa R. Diffusion restriction in pons resembling “reverse moustache” in dengue encephalitis. Neurol India 2014;62:683-4.  Back to cited text no. 9
[PUBMED]  [Full text]  
Mishra SS, Panigrahi S, Das S. Dengue hemorrhagic fever: A rare cause of pituitary apoplexy. Neurol India 2014;62:92-3.  Back to cited text no. 10
[PUBMED]  [Full text]  


  [Figure 1], [Figure 2], [Figure 3]

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