West syndrome (also known as infantile spasm because of its main seizure type) is a rare form of epilepsy that begins during early infancy. Recent guidelines and reviews on West syndrome recommend the use of adrenocorticotropic hormone steroids, or vigabatrin, as the first-line treatment. However, West syndrome remains to be one of the most challenging epilepsies to treat. Here, we systematically reviewed the current literature obtained during the previous decade. This article provides an overview of the current treatment of infantile spasms.
PubMed and EMBASE were searched to retrieve studies on human published during 2005–2015 and to identify patients with clinical diagnosis of infantile spasms. Drug or diet treatments were used as interventions and comparators.
We included 55 studies, of which 1 study was a meta-analysis, 9 were randomized controlled trials, 21 were prospective studies, and 24 were retrospective studies. Topiramate, levetiracetam, zonisamide, and sodium valproate with benzodiazepine (clonazepam or nitrazepam) were found to be potential drugs for treating West syndrome besides adrenocorticotropic hormone, steroids, and vigabatrin. Ketogenic diet and modified Atkins diet were also found to be effective.
To date, data regarding the efficacy of treatments of West syndrome still remain limited. Some treatments, including topiramate and ketogenic diet, seem promising besides adrenocorticotropic hormone, steroids, and vigabatrin. Well-designed trials are warranted to validate the findings.
*Department of Pharmaceutical Medicine and Regulatory Sciences, Colleges of Medicine and Pharmacy, †Department of Pharmacy and Yonsei Institute of Pharmaceutical Sciences, College of Pharmacy, and ‡Department of Pediatrics, College of Medicine, Yonsei University, Incheon; and §Division of Pediatric Neurology, Severance Children's Hospital, Seoul, Republic of Korea.
Address correspondence and reprint requests to Min Jung Chang, PhD, Department of Pharmacy, College of Pharmacy, Yonsei University, 85 Songdogwahak-ro, Yeonsu-gu, Incheon, 21983 Republic of Korea; E-mail: email@example.com; and Se Hee Kim, MD, Department of Pediatrics, Yonsei University College of Medicine, Division of Pediatric Neurology, Severance Children's Hospital, 50-1 Yonsei-ro, Seodaemun-gu, Seoul 03722, Republic of Korea; E-mail: SEHEEKIM@yuhs.ac
Conflicts of Interest and Source of Funding: The authors have no conflicts of interest to declare.
M.J.C. and S.H.K. contributed equally to this study as corresponding authors.
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