Department of Pediatrics, Lady Hardinge Medical College, New Delhi, India. Correspondence: Dr. Suvasini Sharma, Associate Professor, Department of Pediatrics, Lady Hardinge Medical College and Kalawati Saran Children Hospital, New Delhi, India.
Department of Pediatrics, Pandit Bhagwat Dayal Sharma Post Graduate Institute of Medical Sciences, Rohtak, Haryana, India.
Indian Pediatr. 2021 Jan 15;58(1):54-66.
West syndrome is one of the commonest causes of epilepsy in infants and young children and is a significant contributor to neurodevelopmental morbidity. Multiple regimens for treatment are in use.
An expert group consisting of pediatric neurologists and epileptologists was constituted. Experts were divided into focus groups and had interacted on telephone and e-mail regarding their group recommendations, and developed a consensus. The evidence was reviewed, and for areas where the evidence was not certain, the Delphi consensus method was adopted. The final guidelines were circulated to all experts for approval.
Diagnosis should be based on clinical recognition (history/home video recordings) of spasms and presence of hypsarrhythmia or its variants on electroencephalography. A magnetic resonance imaging of the brain is the preferred neuroimaging modality. Other investigations such as genetic and metabolic testing should be planned as per clinico-radiological findings. Hormonal therapy (adrenocorticotropic hormone or oral steroids) should be preferred for cases other than tuberous sclerosis complex and vigabatrin should be the first choice for tuberous sclerosis complex. Both ACTH and high dose prednisolone have reasonably similar efficacy and adverse effect profile for West syndrome. The choice depends on the preference of the treating physician and the family, based on factors of cost, availability of infrastructure and personnel for daily intramuscular injections, and monitoring side effects. Second line treatment options include anti-epileptic drugs (vigabatrin, sodium valproate, topiramate, zonisamide, nitrazepam and clobazam), ketogenic diet and epilepsy surgery.
婴儿痉挛症是婴幼儿期最常见的癫痫综合征之一，也是导致神经发育障碍的重要原因。目前有多种治疗方案。
由儿童神经科医生和癫痫科医生组成专家组。专家们分为焦点小组，通过电话和电子邮件就小组建议进行了互动，并达成了共识。对证据进行了审查，并对证据不明确的领域采用了德尔菲共识方法。最终指南分发给所有专家进行审批。
诊断应基于痉挛的临床特征（病史/家庭录像）和脑电图上出现高度失律或其变异型。首选脑磁共振成像作为神经影像学检查。根据临床-影像学发现，应计划进行其他检查，如基因和代谢检测。除结节性硬化症外，应首选激素治疗（促肾上腺皮质激素或口服类固醇），而对于结节性硬化症，应首选氨己烯酸。促肾上腺皮质激素和大剂量泼尼松龙治疗婴儿痉挛症的疗效和不良反应谱相当，两者的选择取决于治疗医生和患者家庭的偏好，取决于成本、基础设施和人员是否可用于每日肌内注射以及监测不良反应的因素。二线治疗方案包括抗癫痫药物（氨己烯酸、丙戊酸钠、托吡酯、唑尼沙胺、硝西泮和氯巴占）、生酮饮食和癫痫手术。
