Abstract：
OBJECTIVE:To clarify changes in clinical practice for infantile spasms, including West syndrome, in Japan over the past two decades. METHODS:We investigated common treatment strategies for infantile spasms among 157 pediatric neurologists from a designated training facility for pediatric neurology and/or a designated training facility for epilepsy in Japan. A questionnaire was used to investigate use of adrenocorticotropic hormone (ACTH) therapy including daily dose, treatment duration, and tapering off period, and preferred first to fifth-line treatment choices. RESULTS:Among 119 responses (75.8%), 107 enabled analysis of ACTH therapy and 112 were used to determine preferred order of first to fifth-line treatments. Over 80% respondents reported an initial ACTH dose of ≤0.0125 mg/kg/day, with a treatment duration of 14 days and various tapering periods. Following an unfavorable response of seizures to ACTH, 80% respondents increased the dose and/or extended treatment duration. The same ACTH therapy regimen was performed for symptomatic and cryptogenic patients at 95 facilities (88.8%). Preferred orders of therapeutic agents were the same for both symptomatic and cryptogenic patients at 64 facilities (57.1%). Over half the respondents selected vitamin B6 or valproate as the first and second-line treatments instead of ACTH therapy, while ACTH therapy was the most frequently selected third-line treatment. CONCLUSIONS:Current ACTH therapy regimens have lower doses and shorter durations than previously reported. However, treatment strategies for infantile spasms have not changed much in two decades. ACTH therapy should be the first/second-line treatment rather than third-line or later, especially for cryptogenic infantile spasms.
journal_name
Brain Devjournal_title
Brain & developmentauthors
Hamano SI,Nagai T,Matsuura R,Hirata Y,Ikemoto S,Oba A,Hiwatari Edoi
10.1016/j.braindev.2018.04.006subject
Has Abstractpub_date
2018-09-01 00:00:00pages
685-692issue
8eissn
0387-7604issn
1872-7131pii
S0387-7604(18)30163-3journal_volume
40pub_type
杂志文章abstract：BACKGROUND:Leigh syndrome (LS) is a mitochondrial disorder that shows abnormal basal ganglia lesion and psychomotor regression. Although vitamins have been used for LS, we have not found any effective drug. CASE PRESENTATION:A 26-year-old man who showed psychomotor delay and short stature at the age of 1 year was diag...
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pub_type： 杂志文章,评审
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更新日期：1998-10-01 00:00:00
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更新日期：2004-06-01 00:00:00
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journal_title：Brain & development
pub_type： 杂志文章
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更新日期：1981-01-01 00:00:00
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pub_type： 杂志文章
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更新日期：1984-01-01 00:00:00
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更新日期：1990-01-01 00:00:00
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journal_title：Brain & development
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更新日期：2008-01-01 00:00:00
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journal_title：Brain & development
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更新日期：2002-06-01 00:00:00
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更新日期：2011-08-01 00:00:00
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pub_type： 杂志文章,评审
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更新日期：2001-11-01 00:00:00
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更新日期：2000-09-01 00:00:00
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更新日期：2007-10-01 00:00:00
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