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ORIGINAL ARTICLE
West Syndrome: Clinical Characteristics, Therapeutics, Outcomes and Prognosis
1
Centro Habana Teaching Pediatric Hospital, Havana, CUBA
2
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
KEYWORDS
ABSTRACT
Introduction:
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.
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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