Adrenocorticotropic Hormone Induced Status Dystonicus in a Child with West Syndrome.

IF 1.5
Jasmine Singh, Roosy Aulakh
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引用次数: 1

Abstract

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.

How to cite this article: 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.

Abstract Image

促肾上腺皮质激素诱导的西综合征患儿肌张力障碍。
肌张力障碍是一种运动障碍,其特征是不自主的持续或间歇性的肌肉收缩,引起重复的扭曲运动和异常的姿势。状态失调(SD)是一种病因不明的疾病。我们在此报告一个患有韦斯特综合征(WS)的儿童,他在肌内注射促肾上腺皮质激素(ACTH)后继续发展为SD。一个11个月大的男婴主诉屈肌痉挛2个月。经脑电图(EEG)证实WS诊断为低心律失常。肌内注射促肾上腺皮质激素,并开始口服三己苯酯治疗肌张力障碍。ACTH第7天,患儿出现频繁的体位压迫。制定了IV级SD管理方案。在大鼠蓝斑处注射促肾上腺皮质激素n端已被证明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导的西综合征患儿状态障碍。中华检验医学杂志;2009;26(8):961-962。
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