West Syndrome Epilepsy

GPTKB entity

Statements (43)
Predicate Object
gptkbp:instanceOf epileptic syndrome
gptkbp:alsoKnownAs infantile spasms
West syndrome
gptkbp:associatedWith gptkb:autism_spectrum_disorder
intellectual disability
gptkbp:canBeIdiopathic yes
gptkbp:cause gptkb:tuberous_sclerosis
gptkb:Down_syndrome
perinatal asphyxia
gptkbp:characterizedBy developmental regression
epileptic spasms
hypsarrhythmia
gptkbp:complication gptkb:Lennox-Gastaut_syndrome
gptkbp:diagnosedBy gptkb:EEG
clinical observation
gptkbp:EEGPattern hypsarrhythmia
gptkbp:firstDescribed 1841
William James West
gptkbp:frequency 1 in 2,500 to 1 in 6,000 live births
gptkbp:gender male predominance
https://www.w3.org/2000/01/rdf-schema#label West Syndrome Epilepsy
gptkbp:ICD-10_code G40.4
gptkbp:MeSH_ID D013580
gptkbp:mortalityRate increased compared to general population
gptkbp:OMIM 308350
gptkbp:onset infancy
gptkbp:parentOrganization International League Against Epilepsy (ILAE) classification
gptkbp:peakOnsetAge 3 to 12 months
gptkbp:prevalence rare
gptkbp:prognosis variable
often poor
gptkbp:relatedTo Epilepsy
gptkbp:riskFactor genetic disorders
metabolic disorders
structural brain abnormalities
gptkbp:symptom clusters of spasms
sudden flexion or extension of limbs
gptkbp:treatment gptkb:vigabatrin
gptkb:adrenocorticotropic_hormone
corticosteroids
antiepileptic drugs
gptkbp:bfsParent gptkb:D004239
gptkbp:bfsLayer 8