A special note on terminology of West syndrome and infantile spasms

Fukuyama, Yukio

doi:10.1016/s0387-7604(01)00328-x

Cited by 8 publications

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“…West syndrome (WS) is an age-specific epileptic encephalopathy (EE), which typically occurs in infants and has poor epilepsy, neurodevelopmental prognosis and high risk of early mortality (Dulac 2001, Fukuyama 2001, Galanopoulou 2013, Galanopoulou & Moshe 2015). WS manifests with at least two of the following features: (a) ictal events of flexion or extension spasms, called infantile spasms (IS) that usually appear in clusters, (b) interictal chaotic high amplitude and multifocal epileptic interictal background (hypsarrhythmia), and (c) intellectual or neurodevelopmental disabilities (Galanopoulou & Moshe 2015).…”

Section: Introductionmentioning

confidence: 99%

Inflammation in Epileptic Encephalopathies

Shandra

Moshé

Galanopoulou

2017

Stress and Inflammation in Disorders

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West syndrome (WS) is an infantile epileptic encephalopathy (EE) that manifests with infantile spasms, hypsarrhythmia (in ~60% of infants) and poor neurodevelopmental outcomes. The etiologies of WS can be structural-metabolic pathologies (~60%), genetic (12–15%) or of unknown origin. The current treatment options include hormonal treatment [adrenocorticotropic hormone (ACTH) and high dose steroids], the GABA aminotransferase inhibitor vigabatrin, while ketogenic diet can be given as add-on treatment in refractory IS. There is a need to identify new therapeutic targets and more effective treatments for WS. Theories about the role of inflammatory pathways in the pathogenesis and treatment of WS have emerged, being supported by both clinical and preclinical data from animal models of WS. Ongoing advances in genetics have revealed numerous genes involved in the pathogenesis of WS, including genes directly or indirectly involved in inflammation. Inflammatory pathways also interact with other signaling pathways implicated in WS, such as the neuroendocrine pathway. Furthermore, seizures may also activate pro-inflammatory pathways raising the possibility that inflammation can be a consequence of seizures and epileptogenic processes. With this targeted review we plan to discuss the evidence pro and against the following key questions. Does activation of inflammatory pathways in the brain cause epilepsy in WS and does it contribute to the associated comorbidities and progression? Can activation of certain inflammatory pathways be a compensatory or protective event? Are there interactions between inflammation and the neuroendocrine system that contribute to the pathogenesis of West syndrome? Does activation of brain inflammatory signaling pathways contribute to the transition of WS to Lennox-Gastaut syndrome? Are there any lead candidates or unexplored targets for future therapy development for WS targeting inflammation?

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Section: Introductionmentioning

confidence: 99%

Inflammation in Epileptic Encephalopathies

Shandra

Moshé

Galanopoulou

2017

Stress and Inflammation in Disorders

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“…A detailed historical review about the different medical nomenclature used to describe spasms in the dominant medical language of the time, English, German and, French, can be found on the review by Yukio Fukiyama published in 2001 in Brain and Development. 10,11…”

Section: Infantile Spasms the Initial Descrip-tions (1841-1960)mentioning

confidence: 99%

Historical Overview of Hypsarrhythmia and Its Association to Epileptic Spasms: A Review of the Medical Literature From 1952 to 1982

Pestana-Knight

Mani

2022

Journal of Clinical Neurophysiology

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Summary:The initial description of infantile spasms and its association to developmental abnormalities was attributed to Dr. Williams J. West in 1841 but the clinical scenario at the time had also been seen by other physicians. French physician Henry Gastaut proposed the eponym of West syndrome in the 9th Colloquium de Marseille in 1960. The description of hypsarrhythmia in 1952 by Gibbs and Gibbs added the EEG component to the triad of infantile spasms. The hypsarrhythmia discovery led to a sudden interest in understanding the etiology and developing treatments for this devastating disease affecting infants and young children. It was in the 1950s when cases of infantile spasms with absence of hypsarrhythmia were initially observed. Also, the treatment with adrenocorticotrophic hormone was initially reported as efficacious for treating infantile spasms and hypsarrhythmia in the late 1950s. Adrenocorticotrophic hormone remains the best treatment option for these epilepsy types. This article will provide a historical review of knowledge developments about hypsarrhythmia and infantile spasms, emphasizing the period 1952 to 1982. The goal of the article was to highlight clinical elements that were discovered then and remain clinically relevant today.

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“…West syndrome is defined by the trial of clusters of axial epileptic flexion or extension tonic spasms first appearing in infants (infantile spasms (IS)), psychomotor impairment, and a classical interictal electroencephalographic pattern called hypsarrhythmia [1, 2]. Electrographically, IS often demonstrate an ictal electrodecremental response (EDR), showing sudden attenuation of the background activities.…”

Section: Introductionmentioning

confidence: 99%

Basic mechanisms of catastrophic epilepsy – Overview from animal models

Galanopoulou

2013

Brain and Development

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Infantile spasms are age-specific seizures of infantile epileptic encephalopathies that are usually associated with poor epilepsy and neurodevelopmental outcomes. The current treatments are not always effective and may be associated with significant side effects. Various mechanisms have been proposed as pathogenic for infantile spasms, including cortical or brainstem dysfunction, disruption of normal cortical-subcortical communications, genetic defects, inflammation, stress, developmental abnormalities. Many of these have been recently tested experimentally, resulting into the emergence of several animal models of infantile spasms. The stress theory of spasms yielded the corticotropin releasing hormone (CRH) induced model, which showed the higher proconvulsant potency of CRH in developing rats, although only limbic seizures were observed. Models of acute induction of infantile spasms in rodents include the N-methyl-D-aspartate (NMDA) model of emprosthotonic seizures, the prenatal betamethasone and prenatal stress variants of the NMDA model, and the γ-butyrolactone induced spasms in a Down’s syndrome mouse model. Chronic rodent models of infantile spasms include the tetrodotoxin model and the multiple-hit models in rats, as well as two genetic mouse models of interneuronopathies with infantile spasms due to loss of function of the aristaless X-linked homeobox related gene (ARX). This review discusses the emerging mechanisms for generation of infantile spasms and their associated chronic epileptic and dyscognitive phenotype as well as the recent progress in identifying pathways to better treat this epileptic encephalopathy.

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A special note on terminology of West syndrome and infantile spasms

Cited by 8 publications

References 0 publications

Inflammation in Epileptic Encephalopathies

Inflammation in Epileptic Encephalopathies

Historical Overview of Hypsarrhythmia and Its Association to Epileptic Spasms: A Review of the Medical Literature From 1952 to 1982

Basic mechanisms of catastrophic epilepsy – Overview from animal models

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