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Symptomatic West syndrome

MedGen UID:
140740
•Concept ID:
C0393699
•
Disease or Syndrome
Synonyms: Infantile Spasm, Symptomatic; Infantile Spasms, Symptomatic; Spasm, Symptomatic Infantile; Spasms, Symptomatic Infantile; Symptomatic Infantile Spasm; Symptomatic Infantile Spasms; Symptomatic West Syndrome; Syndrome, Symptomatic West; West Syndrome, Symptomatic
SNOMED CT: Symptomatic West syndrome (230417001)

Professional guidelines

PubMed

Adrenocorticotropic hormone for the treatment of West Syndrome in children.
Shumiloff NA, Lam WM, Manasco KB
Ann Pharmacother 2013 May;47(5):744-54. Epub 2013 Apr 19 doi: 10.1345/aph.1R535. PMID: 23606552
Immunoglobulin treatment for severe childhood epilepsy.
Geva-Dayan K, Shorer Z, Menascu S, Linder I, Goldberg-Stern H, Heyman E, Lerman-Sagie T, Ben Zeev B, Kramer U
Pediatr Neurol 2012 Jun;46(6):375-81. doi: 10.1016/j.pediatrneurol.2012.03.015. PMID: 22633633
Vigabatrin as a first-choice drug in the treatment of West syndrome.
Fejerman N, Cersósimo R, Caraballo R, Grippo J, Corral S, Martino RH, Martino G, Aldao M, Caccia P, Retamero M, Macat MC, Di Blasi MA, Adi J
J Child Neurol 2000 Mar;15(3):161-5. doi: 10.1177/088307380001500304. PMID: 10757471

Recent clinical studies

Etiology

Adrenocorticotropic hormone for the treatment of West Syndrome in children.
Shumiloff NA, Lam WM, Manasco KB
Ann Pharmacother 2013 May;47(5):744-54. Epub 2013 Apr 19 doi: 10.1345/aph.1R535. PMID: 23606552
STXBP1 mutations cause not only Ohtahara syndrome but also West syndrome--result of Japanese cohort study.
Otsuka M, Oguni H, Liang JS, Ikeda H, Imai K, Hirasawa K, Imai K, Tachikawa E, Shimojima K, Osawa M, Yamamoto T
Epilepsia 2010 Dec;51(12):2449-52. Epub 2010 Nov 3 doi: 10.1111/j.1528-1167.2010.02767.x. PMID: 21204804
West syndrome and other infantile epileptic encephalopathies--Indian hospital experience.
Kalra V, Gulati S, Pandey RM, Menon S
Brain Dev 2002 Mar;24(2):130-9. doi: 10.1016/s0387-7604(02)00029-3. PMID: 11891107
West syndrome and other infantile epileptic encephalopathies--Indian hospital experience.
Kalra V, Gulati S, Pandey RM, Menon S
Brain Dev 2001 Nov;23(7):593-602. doi: 10.1016/s0387-7604(01)00288-1. PMID: 11701262
Vigabatrin as a first-choice drug in the treatment of West syndrome.
Fejerman N, Cersósimo R, Caraballo R, Grippo J, Corral S, Martino RH, Martino G, Aldao M, Caccia P, Retamero M, Macat MC, Di Blasi MA, Adi J
J Child Neurol 2000 Mar;15(3):161-5. doi: 10.1177/088307380001500304. PMID: 10757471

Diagnosis

Probability of Remission of the Main Epileptic Syndromes in Childhood.
Ramos-Lizana J, Martínez-Espinosa G, Aguilera-López P, Aguirre-Rodriguez J
J Child Neurol 2022 Jan;37(1):89-97. Epub 2021 Nov 24 doi: 10.1177/08830738211056780. PMID: 34816766
STXBP1 mutations cause not only Ohtahara syndrome but also West syndrome--result of Japanese cohort study.
Otsuka M, Oguni H, Liang JS, Ikeda H, Imai K, Hirasawa K, Imai K, Tachikawa E, Shimojima K, Osawa M, Yamamoto T
Epilepsia 2010 Dec;51(12):2449-52. Epub 2010 Nov 3 doi: 10.1111/j.1528-1167.2010.02767.x. PMID: 21204804
West syndrome and other infantile epileptic encephalopathies--Indian hospital experience.
Kalra V, Gulati S, Pandey RM, Menon S
Brain Dev 2002 Mar;24(2):130-9. doi: 10.1016/s0387-7604(02)00029-3. PMID: 11891107
West syndrome and other infantile epileptic encephalopathies--Indian hospital experience.
Kalra V, Gulati S, Pandey RM, Menon S
Brain Dev 2001 Nov;23(7):593-602. doi: 10.1016/s0387-7604(01)00288-1. PMID: 11701262
Vigabatrin as a first-choice drug in the treatment of West syndrome.
Fejerman N, Cersósimo R, Caraballo R, Grippo J, Corral S, Martino RH, Martino G, Aldao M, Caccia P, Retamero M, Macat MC, Di Blasi MA, Adi J
J Child Neurol 2000 Mar;15(3):161-5. doi: 10.1177/088307380001500304. PMID: 10757471

Therapy

Adrenocorticotropic hormone for the treatment of West Syndrome in children.
Shumiloff NA, Lam WM, Manasco KB
Ann Pharmacother 2013 May;47(5):744-54. Epub 2013 Apr 19 doi: 10.1345/aph.1R535. PMID: 23606552
Immunoglobulin treatment for severe childhood epilepsy.
Geva-Dayan K, Shorer Z, Menascu S, Linder I, Goldberg-Stern H, Heyman E, Lerman-Sagie T, Ben Zeev B, Kramer U
Pediatr Neurol 2012 Jun;46(6):375-81. doi: 10.1016/j.pediatrneurol.2012.03.015. PMID: 22633633
West syndrome and other infantile epileptic encephalopathies--Indian hospital experience.
Kalra V, Gulati S, Pandey RM, Menon S
Brain Dev 2002 Mar;24(2):130-9. doi: 10.1016/s0387-7604(02)00029-3. PMID: 11891107
West syndrome and other infantile epileptic encephalopathies--Indian hospital experience.
Kalra V, Gulati S, Pandey RM, Menon S
Brain Dev 2001 Nov;23(7):593-602. doi: 10.1016/s0387-7604(01)00288-1. PMID: 11701262
Vigabatrin as a first-choice drug in the treatment of West syndrome.
Fejerman N, Cersósimo R, Caraballo R, Grippo J, Corral S, Martino RH, Martino G, Aldao M, Caccia P, Retamero M, Macat MC, Di Blasi MA, Adi J
J Child Neurol 2000 Mar;15(3):161-5. doi: 10.1177/088307380001500304. PMID: 10757471

Prognosis

Probability of Remission of the Main Epileptic Syndromes in Childhood.
Ramos-Lizana J, Martínez-Espinosa G, Aguilera-López P, Aguirre-Rodriguez J
J Child Neurol 2022 Jan;37(1):89-97. Epub 2021 Nov 24 doi: 10.1177/08830738211056780. PMID: 34816766
Electroencephalographic changes before the onset of symptomatic West syndrome.
Endoh F, Yoshinaga H, Kobayashi K, Ohtsuka Y
Brain Dev 2007 Nov;29(10):630-8. Epub 2007 Jun 6 doi: 10.1016/j.braindev.2007.04.004. PMID: 17555900
West syndrome and other infantile epileptic encephalopathies--Indian hospital experience.
Kalra V, Gulati S, Pandey RM, Menon S
Brain Dev 2002 Mar;24(2):130-9. doi: 10.1016/s0387-7604(02)00029-3. PMID: 11891107
West syndrome and other infantile epileptic encephalopathies--Indian hospital experience.
Kalra V, Gulati S, Pandey RM, Menon S
Brain Dev 2001 Nov;23(7):593-602. doi: 10.1016/s0387-7604(01)00288-1. PMID: 11701262
Vigabatrin as a first-choice drug in the treatment of West syndrome.
Fejerman N, Cersósimo R, Caraballo R, Grippo J, Corral S, Martino RH, Martino G, Aldao M, Caccia P, Retamero M, Macat MC, Di Blasi MA, Adi J
J Child Neurol 2000 Mar;15(3):161-5. doi: 10.1177/088307380001500304. PMID: 10757471

Clinical prediction guides

Clinical features of early myoclonic encephalopathy caused by a CDKL5 mutation.
Takeda K, Miyamoto Y, Yamamoto H, Ishii A, Hirose S, Yamamoto H
Brain Dev 2020 Jan;42(1):73-76. Epub 2019 Sep 3 doi: 10.1016/j.braindev.2019.08.003. PMID: 31492455
Long-term developmental outcome in patients with West syndrome after epilepsy surgery.
Iwatani Y, Kagitani-Shimono K, Tominaga K, Okinaga T, Mohri I, Kishima H, Kato A, Sanefuji W, Yamamoto T, Tatsumi A, Murata E, Taniike M, Nagai T, Ozono K
Brain Dev 2012 Oct;34(9):731-8. Epub 2012 Feb 14 doi: 10.1016/j.braindev.2012.01.008. PMID: 22336751
Electroencephalographic changes before the onset of symptomatic West syndrome.
Endoh F, Yoshinaga H, Kobayashi K, Ohtsuka Y
Brain Dev 2007 Nov;29(10):630-8. Epub 2007 Jun 6 doi: 10.1016/j.braindev.2007.04.004. PMID: 17555900
Development of visual attention in West syndrome.
Guzzetta F, Frisone MF, Ricci D, Randò T, Guzzetta A
Epilepsia 2002 Jul;43(7):757-63. doi: 10.1046/j.1528-1157.2002.34601.x. PMID: 12102680
West syndrome and other infantile epileptic encephalopathies--Indian hospital experience.
Kalra V, Gulati S, Pandey RM, Menon S
Brain Dev 2001 Nov;23(7):593-602. doi: 10.1016/s0387-7604(01)00288-1. PMID: 11701262

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