Souza Luciana de Paula, Bermudez Beatriz Bagatin, Bufara Danielle Caldas, Crippa Ana Chrystina de Souza
Paraná Federal University, Curitiba, PR, Brazil.
Universidade Federal do Paraná, Curitiba, PR, Brazil.
Child Neurol Open. 2022 Oct 13;9:2329048X221132639. doi: 10.1177/2329048X221132639. eCollection 2022 Jan-Dec.
West syndrome (WS) is a frequent epileptic encephalopathy associated with Down syndrome (DS). This study evaluated an outpatient protocol for WS in patients with DS who received vigabatrin (VGB) or VGB plus adrenocorticotrophic hormone. We analyzed infants treated in two neuropediatric centers from 2001-2021. We reviewed perinatal and familial history of epilepsy, spasm onset, treatment lag, electroencephalogram, neuroimaging, progression to epilepsy, and other neurological conditions. The outcomes were electroclinical resolution (ECR), relapses, and epilepsy progression. Nineteen infants were included; 57.8% were male. The average spasm onset, follow-up, and treatment lag were 6.4 months, 8.15 years, and 2.33 months, respectively. Almost 74% had ECR after protocol intervention and minor epilepsy progression. Relapses occurred during combined therapy. The treatment protocol, especially combined therapy, was effective for WS in DS, impacting epilepsy progression and indicating the effectiveness of combined therapy to treat WS in patients with trisomy 21.
韦斯特综合征（WS）是一种与唐氏综合征（DS）相关的常见癫痫性脑病。本研究评估了一种针对患有DS且接受vigabatrin（VGB）或VGB加促肾上腺皮质激素治疗的WS患者的门诊治疗方案。我们分析了2001年至2021年在两个神经儿科中心接受治疗的婴儿。我们回顾了围产期和癫痫家族史、痉挛发作、治疗延迟、脑电图、神经影像学、癫痫进展以及其他神经系统疾病。结果包括电临床缓解（ECR）、复发和癫痫进展。纳入了19名婴儿；57.8%为男性。痉挛发作的平均时间、随访时间和治疗延迟分别为6.4个月、8.15年和2.33个月。在方案干预后，近74%的患者实现了ECR，癫痫进展轻微。联合治疗期间出现了复发。该治疗方案，尤其是联合治疗，对DS患者的WS有效，影响癫痫进展，并表明联合治疗对治疗21三体综合征患者的WS有效。
