Ghosh Ritwik, Dubey Souvik, Das Shambaditya, Benito-León Julián
Department of General Medicine, Burdwan Medical College, Burdwan, West Bengal, India.
Department of Neuromedicine, Bangur Institute of Neurosciences (BIN), Kolkata, West Bengal, India.
Am J Trop Med Hyg. 2022 Aug 8;107(3):557-9. doi: 10.4269/ajtmh.22-0020.
Dystonic storm (also called status dystonicus) is a neurological emergency characterized by sustained/intermittent involuntary generalized muscle contractions resulting in repetitive painful twisting movements and abnormal postures. It is commonly documented in patients with diagnosed primary dystonic syndromes or secondary dystonic states (i.e., patients with inborn errors of metabolism, dystonic cerebral palsy, Wilson's disease, pantothenate kinase-associated neurodegeneration, and exposure to drugs). However, viral-induced dystonic storm cases have rarely been reported. We describe the case of an 11-year-old girl from rural West Bengal (India) with a dystonic storm after Japanese encephalitis. Generalized dystonic spasms lasted for about 10-20 minutes and occurred 20-30 times/day. They were associated with extreme pain, fever, exhaustion, sweating, tachycardia, tachypnea, pupillary dilatation, arterial hypertension, and mutism and were precipitated by a full bladder and relieved somewhat during sleep. When dystonic spasms abated, she had high-grade generalized rigidity of all four limbs and fixed cervical and truncal dystonia. She was put on invasive ventilation and deep intravenous sedation with continuous midazolam infusion and other supportive measures and had a good clinical recovery. During the 12 months of follow-up, she did not have any other episode of a dystonic storm. However, axial rigidity and intermittent appendicular (upper limb) dystonic posturing were observed. The authors also have briefly discussed the differential diagnoses and treatment plans for such a neurological emergency.
肌张力障碍风暴（也称为肌张力障碍状态）是一种神经急症，其特征为持续性/间歇性全身性肌肉不自主收缩，导致重复性疼痛性扭转运动和异常姿势。在诊断为原发性肌张力障碍综合征或继发性肌张力障碍状态的患者中（即患有先天性代谢缺陷、肌张力障碍型脑瘫、威尔逊病、泛酸激酶相关神经变性以及接触药物的患者），这种情况较为常见。然而，病毒诱发的肌张力障碍风暴病例鲜有报道。我们描述了一名来自印度西孟加拉邦农村的11岁女孩，在患日本脑炎后出现肌张力障碍风暴的病例。全身性肌张力障碍痉挛持续约10 - 20分钟，每天发作20 - 30次。这些痉挛伴有剧痛、发热、疲惫、出汗、心动过速、呼吸急促、瞳孔散大、动脉高血压和缄默，因膀胱充盈而诱发，睡眠时有所缓解。当肌张力障碍痉挛缓解后，她四肢出现高度全身性强直，颈部和躯干有固定性肌张力障碍。她接受了有创通气和持续输注咪达唑仑的深度静脉镇静及其他支持措施，临床恢复良好。在12个月的随访期间，她未再出现肌张力障碍风暴发作。然而，观察到有轴性强直和间歇性肢体（上肢）肌张力障碍姿势。作者还简要讨论了针对这种神经急症的鉴别诊断和治疗方案。
