Efficacy of long‐term adrenocorticotropic hormone therapy for West syndrome: A retrospective multicenter case series

Epilepsia Open - Tập 6 Số 2 - Trang 402-412 - 2021
Shimpei Baba1, Tohru Okanishi1, Yoichiro Homma2, Takeshi Yoshida3, Tomohide Goto4, Toshio Fukasawa5, Satoru Kobayashi6, Atsushi Kamei7, Yuji Fujii8, Naomi Hino‐Fukuyo9, Keitaro Yamada10, Atsuro Daida11, Hisashi Kawawaki12, Hideki Hoshino13, Hitoshi Sejima14, Yusuke Ishida15, Tetsuya Okazaki16, Takehiko Inui17, Sotaro Kanai1, Hirotaka Motoi1, Shinji Itamura1, Mitsuyo Nishimura18, Hideo Enoki1, Ayataka Fujimoto19
1Department of Child Neurology, Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital, Hamamatsu, Japan
2General Internal Medicine, Seirei Hamamatsu General Hospital, Hamamatsu, Japan
3Department of Pediatrics, Kyoto University Graduate School of Medicine, Kyoto, Japan
4Department of Neurology, Kanagawa Children's Medical Center, Yokohama, Japan
5Department of Pediatrics, Anjo Kosei Hospital, Anjo, Japan
6Department of Pediatrics, Nagoya City West Medical Center, Nagoya, Japan
7Department of Pediatrics, Iwate Medical University School of Medicine, Shiwa, Japan
8Department of Pediatrics, Hiroshima City Funairi Citizens Hospital, Hiroshima, Japan
9Department of Pediatrics, Tohoku Medical and Pharmaceutical University, Sendai, Japan
10Department of Pediatric Neurology, Aichi Developmental Disability Center Central Hospital, Kasugai, Japan
11Department of Pediatrics, St. Luke’s International Hospital, Tokyo, Japan
12Department of Pediatric Neurology, Osaka City General Hospital, Osaka, Japan
13Department of Pediatrics, Teikyo University School of Medicine, Tokyo, Japan
14Department of Pediatrics, Matsue Red-Cross Hospital, Matsue, Japan
15Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan
16Division of Child Neurology, Department of Brain and Neurosciences, School of Medicine, Tottori University Faculty of Medicine, Yonago, Japan
17Department of Pediatric Neurology, Miyagi Children’s Hospital, Miyagi, Japan
18Division of Clinical Laboratory, Seirei Hamamatsu General Hospital, Hamamatsu, Japan
19Epilepsy and Surgery, Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital, Hamamatsu, Japan

Tóm tắt

AbstractObjectivesLong‐term adrenocorticotropic therapy (LT‐ACTH), which consisted of 2‐4 weeks of daily injections of adrenocorticotropic hormone (ACTH) and subsequent months of weekly injections, was tried for relapsed West syndrome (WS) or other intractable epilepsies in small case reports. Our aim was to explore the efficacy of LT‐ACTH for preventing WS relapse, as well as the prevalence of its adverse events.MethodsThis is a retrospective, nationwide, multicenter case series of patients with WS who underwent LT‐ACTH. Clinical information of the patients and protocol of LT‐ACTH were collected from participating institutes in this study. We defined clinical response to ACTH as achievement of hypsarrhythmia and epileptic spasms resolution. Patients who responded to daily ACTH injections were identified and assessed whether they experienced WS relapse during/after the weekly ACTH injection period. The outcome was measured by the nonrelapse rate at 24 months after daily ACTH injections using the Kaplan‐Meier method.ResultsClinical information of 16 children with WS was analyzed. The median age at LT‐ACTH initiation was 14.5 months (range: 7‐68 months). Thirteen (81%) patients had previously undergone conventional ACTH treatment. The LT‐ACTH regimens comprised a median of 16 days of daily injections (range: 11‐28 days) and 10 months of weekly injections (range: 3‐22 months). Seven patients experienced WS relapse during/after subsequent weekly ACTH period, and the nonrelapse rate at 24 months after daily injections was estimated at 60.6% (95% confidence interval: 32.3%‐80.0%). Height stagnation, hypertension, and irritability were observed; lethal adverse events were not reported.SignificanceOur study firstly explored the efficacy of LT‐ACTH for preventing WS relapse. LT‐ACTH might be a treatment option for patients with relapsed or intractable WS; however, we note that our study is limited by its small sample size and the lack of an appropriate control group.

Từ khóa


Tài liệu tham khảo

10.1111/j.1528-1167.2010.02657.x

10.1111/j.0013-9580.2004.02404.x

10.1111/epi.13057

10.1212/WNL.0b013e318259e2cf

10.1016/S0387-7604(01)00263-7

10.1177/088307389100600412

10.1111/j.1528-1157.1999.tb00773.x

10.1111/j.1528-1167.2008.01688.x

10.1016/S0387-7604(01)00307-2

10.1111/j.1528-1167.2011.03127.x

10.1111/j.1528-1167.2006.00888.x

10.1542/peds.97.3.375

10.1016/j.pediatrneurol.2017.07.008

10.1136/adc.55.9.664

10.1016/S0022-3476(83)80606-4

10.1016/S0022-3476(05)81379-4

10.1016/S0387-7604(99)00053-4

10.1016/j.jpeds.2005.11.041

10.1111/j.1469-8749.1981.tb08446.x

10.1111/epi.13557

10.1111/j.1528-1167.2010.02772.x

10.1111/j.1528-1167.2007.01249.x

10.1016/j.pediatrneurol.2008.02.009

10.1016/j.braindev.2014.07.004

10.1016/j.braindev.2015.10.004

10.3805/eands.9.18

10.1111/epi.13670

10.1111/epi.13709

10.1038/bmt.2012.244

10.1177/088307380001500615

10.1055/s-2008-1059590

10.1016/j.braindev.2015.05.012

10.1212/WNL.43.11.2322

10.1016/j.seizure.2014.05.010

10.1212/WNL.58.1.110

10.1016/S0022-3476(80)80706-2

10.1111/j.1528-1157.1983.tb04875.x

10.1016/S0074-7742(02)49013-7

10.1212/WNL.39.8.1027

10.1016/j.jaad.2016.01.062

10.1177/0883073807310988

Thông tin
Thông tin xuất bản

Epilepsia Open

Tập 6 Số 2

402-412

Thông tin tác giả