Singh Jasmine, Aulakh Roosy
Department of Pediatrics, Government Medical College and Hospital, Chandigarh, India.
Indian J Crit Care Med. 2022 Aug;26(8):961-962. doi: 10.5005/jp-journals-10071-24289.
Dystonia is a movement disorder characterized by involuntary sustained or intermittent muscle contraction causing repetitive twisting movements and abnormal postures. Status dystonicus (SD) is an enigmatic disease of cryptic etiology. We hereby report a child with West syndrome (WS) who went on to develop SD following intramuscular adrenocorticotropic hormone (ACTH) injection. An 11-month-old male child presented with complaints of flexor spasms for 2 months. The diagnosis of WS was confirmed by electroencephalography (EEG), which showed hypsarrhythmia. Intramuscular ACTH was added, and oral trihexyphenidyl was started for dystonia. On day 7 of ACTH, the child developed frequent opisthotonic posturing. Management protocol for grade IV SD was initiated. Administration of N-terminal of ACTH in rat locus coeruleus has been shown to produce human dystonia-like movement and abnormal posturing.
Singh J, Aulakh R. Adrenocorticotropic Hormone Induced Status Dystonicus in a Child with West Syndrome. Indian J Crit Care Med 2022;26(8):961-962.
肌张力障碍是一种运动障碍,其特征为非自愿性持续或间歇性肌肉收缩,导致重复性扭转运动和异常姿势。持续性肌张力障碍(SD)是一种病因不明的神秘疾病。我们在此报告一名患有韦斯特综合征(WS)的儿童,在肌肉注射促肾上腺皮质激素(ACTH)后发展为SD。一名11个月大的男童因屈肌痉挛2个月前来就诊。脑电图(EEG)显示高峰节律紊乱,确诊为WS。加用肌肉注射ACTH,并开始口服苯海索治疗肌张力障碍。在ACTH治疗的第7天,患儿出现频繁的角弓反张姿势。启动了IV级SD的管理方案。已证明在大鼠蓝斑核注射ACTH的N端可产生类似人类肌张力障碍的运动和异常姿势。
Singh J, Aulakh R. 促肾上腺皮质激素诱发韦斯特综合征患儿的持续性肌张力障碍。《印度重症监护医学杂志》2022;26(8):961 - 962。