Sahu Jitendra Kumar, Madaan Priyanka, Prakash Kiran
Pediatric Neurology Unit, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Department of Pediatric Neurology, Amrita Institute of Medical Sciences, Faridabad, India.
Lancet Reg Health Southeast Asia. 2023 Feb 28;12:100170. doi: 10.1016/j.lansea.2023.100170. eCollection 2023 May.
Infantile Epileptic Spasms Syndrome (IESS), commonly known as West syndrome, is the most common cause of infantile-onset epileptic encephalopathy. There is a peculiar epidemiological profile of IESS in South Asia. Specific features identified were a preponderance of acquired structural aetiology, male gender dominance, a long treatment lag, limited availability of adrenocorticotropic hormone (ACTH) and vigabatrin, and use of carboxymethyl cellulose derivative of ACTH. Because of the significant disease burden and limited resources, there are distinctive challenges to the optimal care of children with IESS in the South Asian region. Also, there are unique opportunities to bridge these challenges and improve outcomes. This review provides an overview of the landscape of IESS in South Asia and highlights its peculiarities, various challenges, and way forward.
婴儿痉挛症综合征(IESS),俗称韦斯特综合征,是婴儿期起病的癫痫性脑病最常见的病因。南亚地区的婴儿痉挛症综合征有独特的流行病学特征。已确定的具体特征包括获得性结构性病因占主导、男性居多、治疗延迟时间长、促肾上腺皮质激素(ACTH)和氨己烯酸供应有限,以及使用ACTH的羧甲基纤维素衍生物。由于疾病负担重且资源有限,南亚地区对婴儿痉挛症综合征患儿进行最佳护理面临着独特的挑战。此外,也有独特的机会来克服这些挑战并改善治疗结果。本综述概述了南亚地区婴儿痉挛症综合征的情况,突出了其特殊性、各种挑战以及未来的方向。