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Idiopathic Hemiconvulsion-Hemiplegia Syndrome

IHHS


Presentation

  • Key points • Hemiconvulsion-hemiplegia-epilepsy syndrome is a disorder associated with a specific clinical presentation sequence: initial prolonged hemiconvulsion (at less than 2 years of age) and immediate flaccid hemiplegia (a hemiconvulsion-hemiplegia[medlink.com]
  • Niger: A retrospective case series. ( 30890411 ) 2019 2 Hemiconvulsion-hemiplegia-epilepsy syndrome in a child with pre-existing cerebral pathology. ( 28652222 ) Ponnusamy A....Nv Moosa A. 2017 3 Hemiconvulsion-Hemiplegia-Epilepsy Syndrome in a Girl Presented[malacards.org]
  • […] and a concurrent febrileillness should be present.[myslide.es]
  • A 20-month-old girl presented with left hemiconvulsions and left hemiplegia lasting for a prolonged period. Seizures failed to resolve with various anticonvulsants even after many physician contacts.[casereports.bmj.com]
  • The face is always involved and aphasia is present if the dominant side is affected. These signs distinguish acquired post-convulsive from congenital hemiplegia. Diagnostic procedures Should include CSF examination.[epilepsy.com]
Inflammation
  • 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;[ncbi.nlm.nih.gov]
  • The role of inflammation is hypoth-esized with a vicious circle involving inflammationand seizure activity facilitated by brainmaturationputting them under the concept of acute enceph-alopathy with inflammation-mediated status epi-lepticus.KEY WORDS:[documents.tips]
  • […] 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]
  • We therefore propose to group these conditions under the concept of acute encephalopathy with inflammation-mediated status epilepticus.[plu.mx]
  • Most likely is that there is a synergisticrelationship between inflammation and seizures thatpotentiates status epilepticus and cellular damage.[myslide.es]
Difficulty Walking
  • The most common clinical presentations were difficulty walking (12 patients), cranial nerve deficits (9 pa- tients), and behavior difficulties (5 patients). Intracranial hyper- tension was the initial symptom in only 4 patients.[docslide.net]
Protein S Deficiency
  • S deficiency. ( 16514228 ) Mondal R.K....Das S. 2006 29 Hemiconvulsion-hemiplegia-epilepsy syndrome as a presenting feature of L-2-hydroxyglutaric aciduria. ( 16948946 ) Lee C....Woelfle J. 2006 30 Hemiconvulsion-hemiplegia-epilepsy syndrome: characteristic[malacards.org]
  • Genetic factors, such as channelopathies secondary to mutation within SCN1A ( 10 ) or CACNA1A ( 11 ) or pro-thrombotic states due to the presence of factor V Leiden ( 12 ), protein S deficiency ( 12 , 13 ), or the MTHFR C677T polymorphism ( 12 ), may[frontiersin.org]
Withdrawn
  • Hemiconvulsion-hemiplegia-epilepsy syndrome: current understandings. ( 22341151 ) Auvin S....Boespflug-Tanguy O. 2012 20 Hemiconvulsion-hemiplegia-epilepsy syndrome associated with CACNA1A S218L mutation. ( 21824570 ) Yamazaki S....Adachi Y. 2011 21 WITHDRAWN[malacards.org]
  • VPA was withdrawn due to pancreatitis. Partial seizure remission was achieved by nitrazepam (NT) introduction at the age of 4 years. Episode of acute encephalopathy, lasting for a week, occurred at the age of 6 years.[omicsonline.org]
Behavior Disorder
  • Approximately half of these children with epilepsy display academic difficulties and/or behavioral disorders. Moreoever, it is now believed that a sizable proportion of children with learning disability suffer from undiagnosed epilepsy.[books.google.com]
Learning Difficulties
  • Learning difficulties are probably the rule. Seizures are usually intractable to anti-epileptic medication.[epilepsy.com]
  • Learning difficulties is probably the rule. Acute and immediate treatment of the hemiconvulsion status epilepticus is mandatory.[medlink.com]
  • Clinical manifestations Rolandic epilepsy, being idiopathic, is expected to occur in children who are otherwise normal, though recent studies showed that the presence of excessive spike and wave epileptiform discharges has been linked to learning difficulties[intechopen.com]
Flaccid Paralysis
  • They show, upon recovery from the acute stage, a flaccid paralysis which is gradually replaced by a spastic condition and, months or years later, a chronic epilepsy, which is rarely of the sensory or motor type, but usually of the psychomotor type.[medlink.com]

Workup

  • Metabolic workup during admission, however, was equivocal, and the patient’s presentation likely had a multifactorial etiology.[frontiersin.org]
Burst Suppression
  • Other testing should include EEG, serum glucose, and CSF analysis.CLINICAL CASE, PART II Following resolution of burst suppression the EEG became severely suppressedover the entire left hemisphere.[myslide.es]
  • The last variant, which is analogous to the burst-suppression of OS (see below), is often associated with cerebral malformations (e. g., schizencephaly, Aicardi syndrome, hemimegalen-cephaly) (113).[aibolita.com]
  • Clinical features: Triad: Myoclonic jerks Erratic, fragmented, random, asynchronous, asymmetric Massive axial bilateral myoclonus Focal seizures Tonic seizures Encephalopathy, global developmental delay, hypotonia, progressive microcephaly EEG findings: Burst-suppression[emilytam.com]
Focal Slow-Waves
  • EEG was performed in 17 children and was normal in 15, dem- onstrated focal epileptic discharges in 1, and focal slow waves in 1.[docslide.net]
  • In the nonactive phase, focal seizures occur infrequently; the wake EEG shows focal slow waves, spikes, or spike-waves (temporoparietal in LKS, frontal in non-LKS), and infrequent bursts of bilateral slow spike-waves (absent in many LKS and some non-LKS[aibolita.com]
Rhythmic Slowing
  • Ictal electroencephalography(EEG) shows high amplitude 23 Hz rhythmic slow-waveactivity with low-amplitude fast activity and rhythmicspikes contralateral to the predominating jerks (Chauvel& Dravet, 2005).[documents.tips]
Brain Edema
  • Higuchi T, Inaba Y, Hata Y, Seki C, Ichikawa M (1998) MRI, SPECT and MRS findings in a case of acute hemiplegia syndrome with a marked hemispheric brain edema. No To Hattatsu 30(5):403–409 PubMed Google Scholar 12.[link.springer.com]
  • Radiologic findings indicate that there is arelationship between a sequence of events includingearly repetitive seizures, brain edema, cortical andsubcortical atrophy, and chronic epilepsy in many ofthe cases.4 It is clear from pathologic examinationsthat[myslide.es]

Treatment

  • 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]
  • 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]
  • Surgical treatment of delayedepilepsy in hemiconvulsion-hemiplegia-epilepsy syndrome.Neurology 2008;70:21162122.13. Mirsattari S, Wilde N, Pigott S.[myslide.es]
  • While a great number of textbooks have been devoted to various medical aspects of chi- hood epilepsy (diagnosis, genetics, etiology, drug and surgical treatment, etc.), there have been no comprehensive accounts of the cognitive consequences of this condition[books.google.com]
  • Given the rarity of these diseases, the treatments outlined in the abstracts are not always evidence based. The information in the abstracts isnot intended to replace existing local, regional or country specific recommendations and guidelines.[orpha.net]

Prognosis

  • […] 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]
  • Early recognition of the syndrome may help provide patients and families with an accu-rate prognosis regarding the subsequent development of epilepsy.[myslide.es]
  • 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]
  • Course and prognosis Prognosis for both idiopathic childhood occipital epilepsy and idiopathic photosensitive occipital lobe epilepsy is variable. About 50–60% of Gastaut type have remission of seizures within 2–4 years of them starting.[intechopen.com]
  • Early-onset benign childhood occipital epilepsy (Panayiotopoulos type) Half as frequent as BECTS, excellent prognosis Age 1-14 years (typical 5 years) Seizure-types: Autonomic/behavioral disturbances with vomiting, gaze deviation, impaired consciousness[emilytam.com]

Etiology

  • 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.[ncbi.nlm.nih.gov]
  • While a great number of textbooks have been devoted to various medical aspects of chi- hood epilepsy (diagnosis, genetics, etiology, drug and surgical treatment, etc.), there have been no comprehensive accounts of the cognitive consequences of this condition[books.google.com]
  • They are organised into groups, and further divided into clinical, etiological or histopathological sub-types.[orpha.net]
  • 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]
  • HHE syndrome can be dividedinto 2 groups based on known etiologies.[myslide.es]

Epidemiology

  • ., Wegener, HC, and Tauxe, RV Epidemiology of Campylobacter jejuni infections in the United States and other industrialized nations.[books.google.es]
  • Their inci-dence could be underestimated in the literature, since thereare no epidemiologic studies considering these two enti-ties.[documents.tips]
  • Definitions Making the correct diagnosis in epilepsies The ILAE classification of epileptic seizures and epileptic syndromes Epidemiology of epilepsies 2 - Epileptic seizures and their classification The formal definition of epileptic seizures Classification[ci.nii.ac.jp]
  • Epidemiology Berry aneurysms form of aneurysms of the central nervous.[pinterest.fr]
  • Speech progressed well andhe was speaking in sentences, counting, and identify-ing some letters.DISCUSSION Epidemiology. HHE syndrome wasfirst reported by Gastaut and colleagues1 approxi-mately 50 years ago.[myslide.es]
Sex distribution
Age distribution

Pathophysiology

  • The severe impairments associated with HHEsyndrome makes it imperative that continued re-search into the pathophysiology and treatment of thedisorder is performed.[myslide.es]
  • Auvin S, Devisme L, Maurage CA, Soto-Ares G, Cuisset JM, Leclerc F, Vallée L (2007) Neuropathological and MRI findings in an acute presentation of hemiconvulsion–hemiplegia: a report with pathophysiological implications.[link.springer.com]
  • In the laboratory, I use both in vivo and in vitro studies to develop new possible treatments for the control of seizures and seizure-induced brain damage, but also to understand the pathophysiology of such a complex process.[chusj.org]
  • ., 2011) that these entities with differ-ent names might share a common pathophysiology basedon a vicious circle with inflammation inducing seizuresevolving to status and status enhancing and keeping activethe inflammatory pathways (Nabbout et al., 2011[documents.tips]
  • Its cause and pathophysiology ( cause and mechanisms by which damage occurs ) are different from other epilepsies, although the symptoms are very similar to other epileptic syndromes, such as Generalized epilepsy with febrile seizures plus ( GEFS ), Dravet[eng.ichacha.net]

Prevention

  • […] 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]
  • No guidelines exist as to whether children withHHE syndrome should be on chronic anticonvulsantmedication to prevent the remote seizures.[myslide.es]
  • Abuse and Neglect in Healthcare 2.00 ADD COURSE Preventing Allergic Reactions to Natural Rubber Latex in the Workplace 2.00 ADD COURSE Preventing Needlestick Injuries in Health Care Settings 2.00 ADD COURSE Prevention of Hepatitis A Through Active or[ceuprofessoronline.com]
  • Career Summary My research focuses on the mechanisms of seizure-induced brain damage and how to prevent it.[chusj.org]
  • Aggressive anticonvulsive therapy is important to prevent/milden often developing hemiatrophy and permanent epilepsy.[pinterest.pt]

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