Abstract
Hemiconvulsion-hemiplegia-epilepsy syndrome (HHES) is a subset of acute encephalopathy characterized by infantile-onset with acute hemiconvulsive febrile status and subsequent unilateral cerebral atrophy and hemiparesis. In the chronic phase, patients with HHES develop epilepsy, typically displayed as intractable focal seizures. The patients are often intractable with antiepileptic drugs and need surgical treatment. Although viral encephalitis and genetic abnormalities are presumed to be the underlying etiology, the pathogenesis remains mostly unknown. We describe three cases of successful functional hemispherotomy for intractable epilepsy in HHES. Patients developed acute asymmetrical convulsive status following viral infections during the ages of 17–30 months. Their seizures were intractable with antiepileptic drugs and required hemispherotomy. On the basis of the pathological findings, all cases were diagnosed as focal cortical dysplasia (FCD) type IIId. The epileptogenic mild cortical malformations may be the cause of HHES.
Highlights
Hemiconvulsion-hemiplegia-epilepsy syndrome (HHES) is a rare condition first described by Gastaut et al in 1960 [1] that starts with virus infection-associated acute encephalopathy in the unilateral hemisphere
In the 2011 International League Against Epilepsy (ILAE) classification, Focal cortical dysplasia (FCD) was classified into type I: dyslamination and disrupted organization of tissue architecture with morphologically normal neurons and glial cells; type II: Hemiconvulsion-Hemiplegia-Epilepsy With FCD-IIId presence of dysplastic, megalocytic neurons mixed with normal neurons; and type III: adjacent to another principal lesion
We reported 3 cases of HHES following virus infection associated-acute encephalopathy
Summary
Hemiconvulsion-hemiplegia-epilepsy syndrome (HHES) is a rare condition first described by Gastaut et al in 1960 [1] that starts with virus infection-associated acute encephalopathy in the unilateral hemisphere. Brain DWI showed reduced diffusivity on subcortical white matter in the right hemisphere He was diagnosed with viral infection associated-acute encephalopathy and treated with methylprednisolone pulse therapy, high dose immunoglobulin and continuous intravenous midazolam. Excessive neuronal cells and glial cells in white matter (FCD type IIId), immature-like in temporal lobe Immature-like neurons Gliosis y, year(s); mo, month(s); d, day(s); mPSL, methylprednisolone; IV, immunoglobline; MDZ, midazolam; CLB, clobazam; LTG, lamotrigine; TPM, topiramate; CBZ, carbamazepine; LEV, levetiracetum; PB, phenobarbital; VPA, valproic acid; PER, perampanel; F, frontal lobe; T, temporal lobe; O, occipital lobe; FCD, focal cortical dysplasia. Brain DWI showed reduced diffusivity in the cortex and subcortical white matter in the right hemisphere, at 5 days after the occurrence (Figure 1A) He was treated with methylprednisolone pulse therapy and high dose immunoglobulin, mannitol, phenobarbital, and continuous intravenous midazolam. Clustering of immature-like neurons was observed in the left amygdala (Figure 2D) and gliosis was observed in the right hippocampus
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