Síndrome de West: etiología, opciones terapéuticas, evolución clínica y factores pronósticos

Arce-Portillo, Elena; Rufo-Campos, M; Muñoz-Cabello, Beatriz; Blanco-Martínez, B; Madruga-Garrido, M; Portal, Laura; Fernández-Mensaque, R. Candau

doi:10.33588/rn.5202.2010392

Cited by 8 publications

(29 citation statements)

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“…Such disease represents around 2 to 10% of the epilepsies in childhood and it is the most frequent encephalopathy which occurs before the first year of life (with the exception of the Ohtahara syndrome). A higher prevalence of WS has been observed in males (3,7,8).…”

Section: Introductionmentioning

confidence: 94%

“…WS, moreover, corresponds to an age-dependent epilepsy. The age of onset of this syndrome varies from 4 to 10 months, with a higher occurrence between 5 to 7 months, which can be extended until 2 years (3,7,8). An incidence of 1 case of WS per 4,000 children has been estimated in the literature (3,4,9,10).…”

Section: Introductionmentioning

confidence: 99%

“…In view of this, the findings in anamnesis and the neurological and/or general physical examination (PE) abnormalities can be associated with the etiological diagnosis and the results of the complementary examinations. In this respect, the knowledge about the etiology is of great importance, since this may lead to the optimal treatment, with positive implications on the evolution and prognosis (7,(16)(17)(18).…”

Section: Introductionmentioning

confidence: 99%

“…Most of the cases of WS have an unfavorable evolution, related to different prognostic factors that alone or combined influence the severity of this syndrome (3)(4)(5)7,16). There are evidences about the efficacy of several drugs for the treatment of WS.…”

Section: Introductionmentioning

confidence: 99%

“…Other drugs, such as prednisolone (1), valproic acid (VA), benzodiazepines, vitamin B6 (VB6), topiramate (TPM), levetiracetam (LVT), and zonisamide have also shown efficacy. In addition, the ketogenic diet, intravenous gammaglobulin therapy and, surgical treatment in refractory cases and specific situations are also useful options for the treatment of such disorder, which have been used increasingly in specialized centers for epilepsy (4,5,7,8,14,16,(19)(20)(21)(22)(23)(24)(25)(26)(27)(28)(29)(30)(31).…”

Section: Introductionmentioning

confidence: 99%

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West Syndrome: Clinical Characteristics, Therapeutics, Outcomes and Prognosis

2020

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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. Objective: To identify clinical characteristics of patients with WS, the related etiology, therapeutics and prognostic factors. Methods: 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. Results: 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). Conclusions: 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.

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Section: Introductionmentioning

confidence: 94%