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Definition (NCI)
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A rare autosomal recessive inherited neurodegenerative disorder caused by mutations in the PLA2G6 gene. It is characterized by the development of swellings called spheroids along the axons of the central nervous system. Signs and symptoms appear early in life and include movement difficulties, muscle hypotonia and spasticity, and dementia.
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Definition (MSH)
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An epileptic syndrome characterized by the triad of infantile spasms, hypsarrhythmia, and arrest of psychomotor development at seizure onset. The majority present between 3-12 months of age, with spasms consisting of combinations of brief flexor or extensor movements of the head, trunk, and limbs. The condition is divided into two forms: cryptogenic (idiopathic) and symptomatic (secondary to a known disease process such as intrauterine infections; nervous system abnormalities; BRAIN DISEASES, METABOLIC, INBORN; prematurity; perinatal asphyxia; TUBEROUS SCLEROSIS; etc.). (From Menkes, Textbook of Child Neurology, 5th ed, pp744-8)
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| Concepts |
Disease or Syndrome
(T047)
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| MSH |
D013036
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| ICD9 |
345.6 |
| ICD10 |
G40.82
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| SnomedCT |
193014002, 193015001, 288197007, 193016000, 155042008, 28055006 |
| English |
Infantile spasms, Spasms, Infantile, Syndrome, West, West Syndrome, Infantile Spasms, Infantile spasms NOS, Infantile spasm, Infant spasms - hypsarrythmia, infantile spasms (diagnosis), infantile spasms, Attack, Lightning, Attacks, Lightning, Lightning Attack, Lightning Attacks, Infantile Spasm, Spasms, Infantile [Disease/Finding], west's syndrome, Seizure;infant spasms, hypsarrhythmia, salaam attacks, salaam seizures, spasm infantile, west syndrome, syndrome west, West syndrome (finding), Infantile spasms NOS (disorder), West's syndrome, Lightning spasms, West syndrome, Infantile spasms - hypsarrythmia, West syndrome (disorder), West, infantile; spasm, lightning; spasm, spasm; infantile, spasm; lightning, infantile spasm |
| Swedish |
Kramp hos spädbarn
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| Japanese |
ﾃﾝﾄｳﾃﾝｶﾝ, ｳｴｽﾄｼｮｳｺｳｸﾞﾝ, 乳児けいれん, ヒプサルスミア, 点頭痙攣, 乳児痙攣, ジャックナイフ痙攣, 点頭けいれん, 点頭発作, ひきつけ-幼児, ヒプスアリスミア, 乳幼児痙攣, 汎性ミオクロニー性れん縮, けいれん-乳幼児, ジャックナイフけいれん, 点頭癲癇, 痙攣-点頭, 癲癇-点頭, 痙攣-乳幼児, ウェスト症候群, てんかん-点頭, 点頭てんかん, ウエスト症候群, ミオクロニー性れん縮-汎性, 幼児ひきつけ, West症候群, けいれん-乳児 |
| Czech |
křeče u dětí, Westův syndrom, Spasmy infantilní |
| Finnish |
Infantiili spasmi
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| Russian |
KIVATEL'NAIA SUDOROGA, EPILEPSIIA DETSKAIA, SUDOROGI U DETEI, GIPSARITMIIA, SALAAMOVY PRIPADKI, SUDOROGI U DETEI EPILEPTICHESKIE, ГИПСАРИТМИЯ, КИВАТЕЛЬНАЯ СУДОРОГА, САЛААМОВЫ ПРИПАДКИ, СУДОРОГИ У ДЕТЕЙ, СУДОРОГИ У ДЕТЕЙ ЭПИЛЕПТИЧЕСКИЕ, ЭПИЛЕПСИЯ ДЕТСКАЯ |
| Italian |
Sindrome di West, Spasmi infantili |
| Polish |
Napady skłonów, Napady zgięciowe, Zespół skurczów zgięciowych, Mioklonie dziecięce masywne, Hipsarytmia, Zespół Westa |
| Hungarian |
Csecsemőkori spasmus, West-syndroma |
| Norwegian |
Wests syndrom, West-syndrom, Infantile spasmer |
| Spanish |
espasmos infantiles, SAI (trastorno), espasmos infantiles, SAI, Crisis Salutatoria, Ataques de Salaam, ataques de cabeceo, ataques salutatorios, espasmos de relampagueo, espasmos infantiles, hipsarritmia, síndrome de West (trastorno), síndrome de West, Espasmos infantiles, Espasmos Infantiles, Síndrome de West |
| Portuguese |
Crises de Salamaleque, Espasmos de Aquiescência, Espasmos Infantis Sintomáticos, Espasmos em Salame, Espasmos infantis, Espasmos Infantis, Síndrome de West |
| Dutch |
bliksem; spasme, infantiel; spasme, spasme; bliksem, spasme; infantiel, infantiele spasmen, Infantiel spasme, Infantiele spasmen, Spasme, infantiel, Spasmen, infantiele, West-syndroom |
| German |
infantile Spasmen, West-Syndrom, Frühkindliche Krampfanfälle, Krampfanfälle, frühkindliche |
| French |
Spasmes infantiles, Spasmes en flexion, Syndrome de West |
