Goswami Jyotindra Narayan, Roy Shuvendu, Patnaik Saroj Kumar
Department of Pediatrics, Army Hospital (R&R), Delhi, India.
Neurol Clin Pract. 2021 Oct;11(5):e645-e653. doi: 10.1212/CPJ.0000000000000989.
Pediatric dystonic storm is an underrecognized entity. We aimed to evaluate the profiles of children presenting with dystonic storm in a referral hospital. Management schema and treatment responsiveness of this uncommonly reported entity were analyzed.
Retrospective review of all children (up to 18 years) hospitalized with dystonic storm over 39 months in the aforementioned facility.
Twenty-three children whose ages ranged from 2 years 2 months to 14 years 4 months years (median: 6 years 11 months) (males: 13, females: 11) presented with dystonic storm. The annual incidence was 0.4 per 1,000 fresh admissions with an event rate of 0.9 per 1,000 for all admissions. All had Dystonia Severity Action Plan grades 4/5 with identifiable trigger in 13 (50%). Underlying dystonic disorder preexisted in 10 (43.4%); 8 of these had cerebral palsy. Polypharmacotherapy with >4 drugs out of trihexyphenidyl, tetrabenazine, clonazepam, gabapentin, levodopa-carbidopa, trichlorophos, and melatonin was needed. Supportive care and adequate sedation helped in symptom control. All children were managed with midazolam infusion over 2-10 days (median: 5 days). Mechanical ventilation was resorted to in 6 children (3-22 days). Vecuronium and propofol were used in 3/23 (13%) and 4/23 (17%) children, respectively. Deep brain stimulation was curative in 1 child. Hospitalization ranged from 5 to 31 (median: 11) days. Although there were no deaths, rhabdomyolysis was noted in 1 child. Postdischarge, 6 (26%) children relapsed.
Dystonic storm is a medical emergency mandating aggressive multimodal management. Supportive care, antidystonic drugs, and early elective ventilation alongside adequate sedation with benzodiazepines ameliorate complications. Relapses of dystonic storm are not uncommon.
小儿肌张力障碍风暴是一种未得到充分认识的疾病。我们旨在评估一家转诊医院中出现肌张力障碍风暴的儿童的情况。分析了这种报道较少的疾病的管理模式和治疗反应性。
回顾性分析上述机构在39个月内住院的所有（18岁及以下）患有肌张力障碍风暴的儿童。
23名年龄在2岁2个月至14岁4个月（中位数：6岁11个月）的儿童（男13名，女11名）出现了肌张力障碍风暴。年发病率为每1000例新入院患者中有0.4例，所有入院患者的事件发生率为每1000例中有0.9例。所有患者的肌张力障碍严重程度行动计划分级均为4/5级，其中13例（50%）有明确的诱发因素。10例（43.4%）患者先前存在潜在的肌张力障碍疾病；其中8例患有脑瘫。需要使用超过4种药物进行多药联合治疗，这些药物包括苯海索、丁苯那嗪、氯硝西泮、加巴喷丁、左旋多巴-卡比多巴、敌百虫和褪黑素。支持性护理和充分的镇静有助于控制症状。所有儿童均接受了2至10天（中位数：5天）的咪达唑仑静脉输注治疗。6名儿童（3至22天）需要进行机械通气。分别有3/23（13%）和4/23（17%）的儿童使用了维库溴铵和丙泊酚。深部脑刺激对1名儿童有效。住院时间为5至31天（中位数：11天）。虽然没有死亡病例，但有1名儿童出现了横纹肌溶解。出院后，6名（26%）儿童复发。
肌张力障碍风暴是一种需要积极进行多模式管理紧急医疗情况。支持性护理、抗肌张力障碍药物、早期选择性通气以及使用苯二氮䓬类药物进行充分镇静可改善并发症。肌张力障碍风暴复发并不罕见。
