West Syndrome: Clinical Characteristics, Therapeutics, Outcomes and Prognosis
More details
Hide details
Centro Habana Teaching Pediatric Hospital, Havana, CUBA
Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, BRAZIL
Online publication date: 2020-02-15
Publication date: 2020-02-15
Electron J Gen Med 2020;17(2):em190
West syndrome (WS) is the most severe, devastating and/or catastrophic epileptic encephalopathy during the lactation period. However, until now, there are few comprehensive clinical studies in this regard in Cuba.

To identify clinical characteristics of patients with WS, the related etiology, therapeutics and prognostic factors.

An observational, descriptive and retrospective study of 39 patients with WS from the Centro Habana Teaching Pediatric Hospital, Havana, Cuba, comprising the period between January 2005 and December 2016 was carried out. Clinical data were recorded for each patient by review of clinical history. Statistical analysis was performed with use of the Statistical Package for Social Sciences.

The genetic, structural/metabolic etiology was predominant. Hypoxic ischemic encephalopathy (25.6%) and neurocutaneous syndromes (17.9%) were the cause more frequent of WS. The combined treatment of adrenocorticotropic hormone (ACTH) and vigabatrin (VGB) was used in 25 patients (64.1%). In 18 of them (72%) there was control of infantile spasms or reduction ≥ 50%, while in 14 patients (56%) the hypsarrhythmia disappeared in the first 6 months (p<0.05). All patients that used ACTH had transient hypertension as an adverse effect of this drug. 71.8% of the patients had moderate to severe delay in psychomotor development, 35.9% were diagnosed with Lennox-Gastaut syndrome and 43.6% with other epilepsies. There was an unfavorable evolution in 74.3% of the patients. The most significant prognostic factors for unfavorable evolution were male gender, symptomatic etiology, psychomotor delay and/or abnormality in the neurological examination, epileptic seizures, previous paroxysmal electroencephalogram, poor response to the treatment, persistence of hypsarrhythmia, and combination of more than three factors in 68.9% of the patients with unfavorable evolution (p <0.05).

Our findings showed that the combined treatment with ACTH and VGB may decrease the time to disappearance of infantile spasms and hypsarrhythmia in patients with WS. Moreover, the unfavorable evolution of these patients is related with the etiology and combination of prognostic factors.

D’Alonzo R, Rigante D, Mencaroni E, Esposito S. West Syndrome: a review and guide for paediatricians. Clin Drug Investig 2018;38(2):113-24. https://doi.org/10.1007/s40261... PMid:29086890.
West WJ. On a peculiar form of infantile convulsions. Lancet 1841;35(911):724-5. https://doi.org/10.1016/S0140-....
Pozo Alonso AJ, Pozo Lauzán D, Pozo Alonso D. Síndrome de West: etiología, fisiopatología, aspectos clínicos y pronósticos. Rev Cubana Pediatr 2002;74(2):151-61.
Taghdiri MM, Nemati H. Infantile spasm: a review article. Iran J Child Neurol 2014;8(3):1-5.
Lux AL. Lastest American and European updates on infantile spasms. Curr Neurol Neurosci Rep 2013;13(3):334. https://doi.org/10.1007/s11910... PMid:23341252.
Berg AT, Berkovic SF, Brodie MJ, et al. Revised terminology and concepts for organization of seizures and epilepsies: report of the ILAE Commission on Classification and Terminology, 2005–2009. Epilepsia 2010;51(4):676-85. https://doi.org/10.1111/j.1528... PMid:20196795.
Arce-Portillo E, Rufo-Campos M, Muñoz-Cabello B, et al. Síndrome de West: etiología, opciones terapéuticas, evolución clínica y factores pronósticos. Rev Neurol 2011;52(2):81-9. https://doi.org/10.33588/rn.52... PMid:21271547.
Fejerman N. Síndrome de West: certezas, incógnitas y sorpresas. In: Ruggieri VL, Caraballo RH, Arroyo HA, editors. Temas de neuropediatría. Homenaje al Dr. Natalio Fejerman. Buenos Aires: Editorial Médica Panamericana; 2005:5-41.
Paciorkowski AR, Thio LL, Dobyns WB. Genetic and biologic classification of infantile spasms. Pediatr Neurol 2011;45(6):355-67. https://doi.org/10.1016/j.pedi... PMid:22114996 PMCid:PMC3397192.
Proposal for revised classification of epilepsies and epileptic syndromes. Commission on Classification and Terminology of the International League Against Epilepsy. Epilepsia 1989;30(4):389-99. https://doi.org/10.1111/j.1528... PMid:2502382.
Fejerman N. Diagnósticos diferenciales del síndrome de West. Rev Neurol 2013;57(Supl 1):S125-8. https://doi.org/10.33588/rn.57....
Caraballo RH, Ruggieri V, Gonzalez G, et al. Infantile spams without hypsarrhythmia: a study of 16 cases. Seizure 2011;20(3):197-202. https://doi.org/10.1016/j.seiz... PMid:21167750.
Salar S, Moshé SL, Galanopoulou AS. Metabolic etiologies in West syndrome. Epilepsia Open 2018;3(2):134-66. https://doi.org/10.1002/epi4.1... PMid:29881795 PMCid:PMC5983207.
Dulac O, Lassonde M, Sarnat HB. Handbook of Clinical Neurology. Pediatric Neurology Part I. Elsevier 2013;111:861.
Fisher RS, van Emde Boas W, Blume W, et al. Epileptic seizures and epilepsy: definitions proposed by the International League Against Epilepsy (ILAE) and the International Bureau for Epilepsy (IBE). Epilepsia Epilepsia 2005;46(4):470-2. https://doi.org/10.1111/j.0013... PMid:15816939.
Campistol J, García-Cazorla A. Síndrome de West. Análisis, factores etiológicos y opciones terapéuticas. Rev Neurol 2003;37(4):345-52. https://doi.org/10.33588/rn.37....
Galicchio S, Cersósimo R, Caraballo RH, Yépez-E II, Medina C, Fejerman N. Resonancia magnética cerebral en el estudio del síndrome de West. Rev Neurol 1999;28(7):685-7. https://doi.org/10.33588/rn.28... PMid:10363294.
Lin DDM, Gallagher A. Advances in pediatric epilepsy neuroimaging. Journal of Pediatric Epilepsy 2013;2(01):1-2.
Carmant L. Vigabatrin therapy for infantile spasms: review of major trials in Europe, Canada, and the United States; and recommendations for dosing. Acta Neurol Scand Suppl 2011;124(192):36-47. https://doi.org/10.1111/j.1600... PMid:22061179.
Wheless JW, Gibson PA, Rosbeck KL, et al. Infantile spasms (West syndrome): update and resources for pediatricians and providers to share with parents. BMC Pediatr 2012;12:108. https://doi.org/10.1186/1471-2... PMid:22830456 PMCid:PMC3411499.
Chudomelova L, Scantlebury MH, Raffo E, Coppola A, Betancourth D, Galanopoulou AS. Modeling new therapies for infantile spasms. Epilepsia 2010;51 Suppl 3:27-33. https://doi.org/10.1111/j.1528... PMid:20618396 PMCid:PMC2909007.
Watemberg N. Infantile spasms: Treatment challenges. Curr Treat Options Neuro 2012;14(4):322-31. https://doi.org/10.1007/s11940... PMid:22581010.
Brunson KL, Avishai-Eliner S, Baram TZ. ACTH treatment of infantile spasms: mechanisms of its effects in modulation of neuronal excitability. Int Rev Neurobiol 2002;49:185-97. https://doi.org/10.1016/S0074-....
Brunson KL, Eghbal-Ahmadi M, Baram TZ. How do the many etiologies of West syndrome lead to excitability and seizures? The corticotropin releasing hormone excess hypothesis. Brain Dev 2001;23(7):533-8. https://doi.org/10.1016/S0387-....
Wray CD, Benke TA. Effect of price increase of adrenocorticotropic hormone on treatment practices of infantile spasms. Pediatr Neurol 2010;43(3):163-6. https://doi.org/10.1016/j.pedi... PMid:20691936 PMCid:PMC3197710.
Fejerman N, Cersósimo R, Caraballo R, et al. Vigabatrin as a first-choice drug in the treatment of West syndrome. J Child Neurol 2000;15(3):161-5. https://doi.org/10.1177/088307... PMid:10757471.
González JE, Martínez A, Avendaño M, et al. Guía práctica clínica. Tratamiento farmacológico del Síndrome de West. Rev Chil Epilepsia 2006;7(1):42-7.
Sakakihara Y. Treatment of West syndrome. Brain Dev 201;33(3):202-6. https://doi.org/10.1016/j.brai... PMid:21196092.
O’Callaghan FJ, Lux AL, Darke K, et al. The effect of lead time to treatment and of age of onset on developmental outcome at 4 years in infantile spasms: evidence from the United Kingdom Infantile Spasms Study. Epilepsia 2011, 52(7):1359-64. https://doi.org/10.1111/j.1528... PMid:21668442.
Nakata M, Kato T, Ide M, et al. Long-term weekly ACTH therapy for relapsed West syndrome in tuberous sclerosis complex: a case report. Brain Dev 2016;38(4):431-4. https://doi.org/10.1016/j.brai... PMid:26482603.
Caraballo RH, Fejerman N. Tratamiento de las epilepsias: Buenos Aires: Editorial Médica Panamericana;2009:292.
Yacubian EMT, Kochen S. Crises epilépticas. São Paulo: Leitura Médica Ltda.;2014:96.
Caraballo RH, Cersósimo RO. Atlas de electroencefalografía en la epilepsia. Buenos Aires: Editorial Médica Panamericana;2010.
Zhu X, Chen O, Zhang D, et al. A prospective study on the treatment of infantile spasms with first-line topiramate followed by low-dose ACTH. Epilepsy Res 2011;93(2-3):149-54. https://doi.org/10.1016/j.eple... PMid:21205578.
Gaily E. Vigabatrin monotherapy for infantile spasms. Expert Rev Neurother 2012;12(3):275-86. https://doi.org/10.1586/ern.12... PMid:22364326.
Hancock EC, Osborne JP, Edwards SW. Treatment of infantile spasms. Cochrane Database Syst Rev 2013;5(6):CD001770. https://doi.org/10.1002/146518....
Durá-Travé T, Yoldi-Petri ME, Hualde-Olascoaga J, Etayo- Etayo V. Epilepsias y síndromes epilépticos durante el primer año de vida. Rev Neurol 2009;48(6):281-4. https://doi.org/10.33588/rn.48... PMid:19291649.
Barbarrosa EP. Características de la epilepsia antes del primer año en el Hospital Pediátrico de Centro Habana durante el período 2004–2009. Rev Cubana Neurol Neurocir 2012;2(2):121-8.
Riikonen R. Recent advances in the pharmacotherapy of infantile spasms. CNS Drugs. 2014; 28(4):279-90. https://doi.org/10.1007/s40263... PMid:24504827.
Farnosova ME, Zharikova TF, Gamirova RG, Sivkova SN. Timing of treatment initiation in West’s syndrome. Int J Risk Saf Med 2015;27(1):S55-S6. https://doi.org/10.3233/JRS-15... PMid:26639711.
Kelley SA, Knupp KG. Infantile Spasms-Have We Made Progress? Curr Neurol Neurosci Rep 2018;18(5):27. https://doi.org/10.1007/s11910... PMid:29671077.
Journals System - logo
Scroll to top