The cause of HHE syndrome remains unexplained in present case and HHE may be a causal or chance co‐occurrence. [onlinelibrary.wiley.com]

A consideration must be made for cerebrovascular accident as the cause for a child's neurological condition when one presents with seizures, especially when there are diffusion changes present on imaging. [thejns.org]

After encephalopathy onset, he presented with left hemiparesis, and suffered from left side dominant- or symmetrical tonic spasms. [frontiersin.org]

Another 3-year-old boy former full-term infant delivered vaginally presented with a history of fever followed by seizure and loss of consciousness. [pediatricneurosciences.com]

The disorder is classically characterized by inflammation of one hemisphere and in the acute phase patients may present with focal onset seizures and almost no hemiparesis. [n.neurology.org]

• Fever

  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. [pediatricneurosciences.com]

  Dengue fever triggering hemiconvulsion hemiplegia epilepsy in a child. [neurologyindia.com]

  GABRA1, GABRB2, GABRG2, CYFIP2, and THG1 are the important genes in the present deleted region, which may be responsible for the fever sensitivity and global developmental delay. [onlinelibrary.wiley.com]

  Our cases indicate that the fever associated with the viral infections triggered the seizures in the hemisphere with FCD type IIId and induced epileptic excitotoxicity. [frontiersin.org]

  Berhouma et al. described the case of a previously healthy child younger than 4 years who presented with fever and prolonged status epilepticus with unwitnessed initial seizure onset. [thejns.org]

• Inflammation

  The disorder is classically characterized by inflammation of one hemisphere and in the acute phase patients may present with focal onset seizures and almost no hemiparesis. [n.neurology.org]

  The clinical and histopathological findings of our reported case lend support to the possible role of inflammation in the pathogenesis of HH/HHE syndrome. https://hal.archives-ouvertes.fr/hal-01064293 Contributeur : Laurent Jonchère <> Soumis le : mardi [jeannicod.ccsd.cnrs.fr]

  Several factors might contribute to the pathogenesis of HH/HHE syndrome: 1. prolonged febrile seizure in which inflammation may worsen the level of cell injury; 2. inflammation and prolonged ictal activity that act on blood-brain-barrier permeability; [pubmed.ncbi.nlm.nih.gov]

  […] the pathogenesis of HH/HHE syndrome 1: prolonged seizure of long duration (possibly unnoticed) impairing metabolism of neuronal cells and leading to excitotoxic cell damage prolonged febrile seizure with an aggravation of cell injury by co-existent inflammation [radiopaedia.org]

• Swelling

  Neuroimaging studies demonstrate an acquired atrophy, that often is preceded by swelling and edema of the hemisphere. [journals.sbmu.ac.ir]

  Recently, cytotoxic edematous swelling of one hemisphere was presented as the pathogenesis because high signal intensity was shown in the diffusion image but no remarkable findings were noticed in the FLAIR image and T2WI in two cases presented by Freeman [annchildneurol.org]

  Neuroradiological studies showed unilateral edematous swelling of the epileptic hemisphere at the time of initial status epilepticus (SE). [pubmed.ncbi.nlm.nih.gov]

  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 [neurologyindia.com]

• Unconsciousness

  He remained unconscious during this period and also developed fever after two days of hospital stay. [jpma.org.pk]

• Protein S Deficiency

  Hemiconvulsion, hemiplegia, epilepsy syndrome and inherited protein S deficiency. [link.springer.com]

  Hemiconvulsion, hemiplegia, epilepsy syndrome and inherited protein S deficiency. Indian J Pediatr. 2006 Feb; 73 (2): 157-9. 11. Scantlebury M.H., David M., Carmant L. [epilepsia.su]

  Hemiconvulsion, hemiplegia, epilepsy syndrome and inherited protein S deficiency. Indian J Pediatr, 73 (2006), pp. 157-159 [17.] C. Lee, M. Born, G.S. Salomons, C. Jakobs, J. Woetfle. [elsevier.es]

  Hemiconvulsion, hemiplegia, epilepsy syndrome and inherited protein S deficiency. Mondal RK, Chakravorty D, Das S. Indian J Pediatr. 2006;73:157–159. [PubMed] [Google Scholar] 4. [ncbi.nlm.nih.gov]

• Vascular Disease

  Vascular disease of the nervous system. In Bradley WG, Daroff RB, Fenichel GM, Jankovic J, eds.Neurology in Clinical Practice. 4th edn. Philadelphia, Butterworth Heinemann 2004; 1197–1243. Google Scholar 14. [link.springer.com]

• Muscular Atrophy

  Right-sided muscular atrophy was clearly observed as shown in Figure 1 and Figure 2. Right-sided muscular atrophy. Muscular atrophy over right side compared to the left side. [ncbi.nlm.nih.gov]

• Generalized Tonic-Clonic Seizure

  Secondarily generalized tonic-clonic seizures and often convulsive status epilepticus are common. Learning difficulties are probably the rule. Seizures are usually intractable to anti-epileptic medication. [epilepsy.com]

  It has been reported before as sequel to status epilepticus and few cases following partial seizures or secondary generalized tonic clonic seizures [2]. Dr. Gastaut divided this condition into two types. [ncbi.nlm.nih.gov]

• Hyporeflexia

  We report a case of HHE syndrome in a 3-year-old boy with partial seizures (hemiconvulsion lasting 15–30 minutes) followed by left hemiplegia and hyporeflexia. [journals.viamedica.pl]

• Learning Difficulties

  Learning difficulties are probably the rule. Seizures are usually intractable to anti-epileptic medication. [epilepsy.com]

Prior to presenting to us he was admitted and had received treatment for meningitis and status epilepticus in another hospital with workup showing a normal CSF exam and MRI findings as described in the images below. [jpma.org.pk]

• Pleural Effusion on Chest X-Ray

  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. [neurologyindia.com]

• Human Herpesvirus 6

  Human herpesvirus 6 infection, CACNA1A gene mutation and coalition disorders like protein C and S deficiency might be one of the etiological factors. [ncbi.nlm.nih.gov]

  Human herpesvirus 6 was isolated from his pharynx. He was treated with methylprednisolone pulse therapy and continuous intravenous midazolam. [frontiersin.org]

Management options* Immediate seizure control (treating status epilepticus with benzodiazepines) and treatment of the underlying illness are essential. Drug treatment is needed for focal epilepsy. Hemispherectomy is beneficial. [epilepsy.com]

Also, early adequate treatment is required such as immunosuppressive treatment with high dose steroid and intravenous immunoglobulin. [annchildneurol.org]

In the failure of conservative treatment with anticonvulsants, epilepsy surgery may be indicated 7. History and etymology It was first described by the French neurologist Henri Jean Pascal Gastaut (1915-1995) et al. in 1960 4. [radiopaedia.org]

[…] the treatment of cerebral herniation in HHE and advocate close monitoring for development of intracranial hypertension in these patients. [thejns.org]

Steroids and intravenous immunoglobulin are beneficial both in the acute stage as short-term high dose therapies as well as in lower doses for long-term treatment. [n.neurology.org]

Prognosis Depends on cause and speed of effective acute management. Focal seizures appear within 1 to 5 years in 80% of the patients. Secondarily generalized tonic-clonic seizures and often convulsive status epilepticus are common. [epilepsy.com]

[…] subcortical cavitations in the affected hemisphere reduced caliber of ACA, MCA or PCA on the affected side small corpus callosum ipsilateral hemispheric volume loss with a shift of midline structures usually lack hemosiderin on T2*/SWI Treatment and prognosis [radiopaedia.org]

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. [neurologyindia.com]

Etiology Central nervous system (CNS) infection such as herpes encephalitis. A few may be traumatic or vascular. Frequently no cause is found. High family incidence of febrile seizures. [epilepsy.com]

Semiologic, etiologic and prognostic aspects. Bull Acad Natl Med. 1984 Oct-Nov; 168 (7-8): 779-86. 6. Aуvazyan S.O. Syndrome “hemiconvul-sionshemiplegia-epilepsy”. The Journal “Quantum Satis”. 2017; 1 (2): 25-34 (in Russian). 7. Miteff C. [epilepsia.su]

The etiologies and the underlying mechanisms remain to be understood. Using a review of the literature, we summarized the data of the last 20 years. It appears that idiopathic HH/HHE syndrome is the most common reported form. [pubmed.ncbi.nlm.nih.gov]

Late results of HHE are a progressive hemiatrophy of one cerebral hemisphere.14 The etiology of HHE remains poorly defined, although it can be related to a focal cortical malformation of the dyslamination type (focal cortical dysplasia, Type 1), which [thejns.org]

Complicación Convulsión Estudio caso Hemiplejía Lactante Sistema nervioso patología Vacunación Hombre Classification Pascal 002 Biological and medical sciences / 002B Medical sciences / 002B05 Infectious diseases / 002B05A General aspects / 002B05A02 Epidemiology [pascal-francis.inist.fr]

Anticipation and identification in appropriate clinical and epidemiological scenarios can help in prognostication and planning long-term management. Financial support and sponsorship Nil. [neurologyindia.com]

Neuropathological and MRI findings in an acute presentation of hemiconvulsionhemiplegia: a report with pathophysiological implications. Seizure. 2007; 16: 371-376. 4. Yamazaki S., Ikeno K., Abe T. et al. [epilepsia.su]

Chronic epilepsy later develops, typically involving refractory focal seizures; however, the underlying pathophysiology of this epilepsy is not well understood. [x-mol.com]

Neuropathological and MRI findings in an acute presentation of hemiconvulsion-hemiplegia: a report with pathophysiological implications. Seizure. 2007; 16: 371-6. 8. Toldo I, Calderone M, Boniver C, Dravet CH, Guerrini R, Laverda AM. [jpma.org.pk]

Differential Diagnosis: Rasmussen’s Encephalitis Stroke Infections-Meningitis, Encephalitis Neoplasm Congenital brain malformations The pathophysiologic mechanism includes prolonged seizures leading to excitotoxic cell injury or damage to blood-brain [ncbi.nlm.nih.gov]

Neuropathological and MRI findings in an acute presentation of hemiconvulsion-hemiplegia: A report with pathophysiological implications. Seizure 2007;16:371-6. 7. Murthy J. Neurological complications of dengue infection. Neurol India 2010;58:581-4. [neurologyindia.com]

Preventive treatment of longlasting febrile seizures is important, but there is no standard therapy for epilepsy in HHE syndrome, as many different clinical situations may be encountered in this context. [moh-it.pure.elsevier.com]

[…] is better than cure and early and aggressive termination of seizures is considered the most effective way to prevent post-convulsive hemiplegia 9. [radiopaedia.org]

Furthermore, a proper measure to reduce cerebral edema is essential for the prevention of neurologic sequalae and deterioration to epilepsy. Key Words: Hemiconvulsion, Hemiplegia, Epilepsy [annchildneurol.org]

`Early seizure control is also of paramount importance for the prevention of hemiplegia. [jpma.org.pk]

[…] the remote seizures, although three of our four cases required long-term preventive antiepileptic therapy [Table 1]. [pediatricneurosciences.com]