カイダ　ケンイチ   KAIDA Kenichi   海田　賢一    所属   埼玉医科大学  医学部 総合医療センター　脳神経内科    職種   教授
論文種別	学術雑誌(原著)
言語種別	英語
査読の有無	査読あり
表題	Painful muscle stiffness with markedly elevated serum creatine kinase (CK) levels after twenty weeks of gestation in four patients with myotonic dystrophy type 1 (DM1) and a patient with paramyotonia congenita (PMC).
掲載誌名	正式名：Journal of Neuromuscular Diseases 略　 称：J Neuromuscul Dis
掲載区分	国外
出版社	Sage journals
巻・号・頁	12(4),558-566頁
著者・共著者	Kinoshita M, Yamamoto M, Machida R, Misawa M, Yabe S, Yoshida N, Kikuchi A, Fukaura H, Kaida K, Ohno K.
発行年月	2025/03/28
概要	Four patients with myotonic dystrophy type 1 (DM1) and a patient with paramyotonia congenita (PMC) developed devastating painful muscle stiffness with markedly elevated serum creatine kinase (CK) levels after 20 weeks of gestation. Immediately after delivery, painful muscle stiffness completely disappeared and the CK levels returned to the baseline. In a patient with DM1, muscle biopsy at delivery and skeletal muscle MRI in six days after delivery showed inflammatory changes, which disappeared in MRI on postpartum day 41. Pregnancy-associated aggravation of myotonia has been reported in myotonic disorders, but painful muscle stiffness has been scarcely reported. Inactivation of muscle Cl channel by progesterone that is prominently increased in the middle phase of gestation is likely to be the underlying mechanism of the pregnancy-associated painful muscle stiffness in myotonic disorders.