Thapa Santosh, Raut Ujwal, Shrestha Garima, Shah Sandesh, Helmu Mangal Bahadur
B. P. Koirala Institute of Health Sciences, Dharan.
KIST Medical College and Teaching Hospital, Lalitpur.
Ann Med Surg (Lond). 2024 Feb 9;86(4):2162-2166. doi: 10.1097/MS9.0000000000001798. eCollection 2024 Apr.
Sydenham's chorea (SC), a major neurological manifestation of acute rheumatic fever (ARF), is commonly seen in young children and adolescents. It is characterized by rapid, unpredictable, involuntary, and nonpatterned contractions affecting mostly distal limbs. It can also be associated with clinical or subclinical carditis. SC has been reported as a major manifestation in only 3.87% cases of acute rheumatic fever in Nepal.
The authors report a case of a 12-year-old boy with abnormal movement of his right hand and unsteady gait for 12 days. On examination, he had an abnormal hand grip with difficulty maintaining a tetanic contraction (Milkmaid's grip). Laboratory investigations revealed increased anti-Streptolysin O titre and erythrocyte sedimentation rate. Echocardiography revealed subclinical carditis. After thorough clinical examination and pertinent investigations, the final diagnosis of ARF with SC was made.
SC is a major clinical feature of rheumatic fever according to the revised Jones criteria. It is related to a previous Group A β-haemolytic (GABHS) infection. Approximately 50-65% of the patients with rheumatic fever later develop clinically detectable carditis. Although a self-limiting condition, it might need treatment with antiepileptics, neuroleptics, and phenothiazines.
Any child presenting with a movement disorder should also be considered for SC, necessitating additional testing, including a cardiovascular assessment. It needs to be distinguished from other causes of movement disorders as well as psychiatric conditions. Treatment is necessary for moderate to severe chorea that interfere with daily activities. Compliance with subsequent antibiotic prophylaxis is essential for avoiding future cardiac complications.
Sydenham舞蹈病(SC)是急性风湿热(ARF)的主要神经表现,常见于儿童和青少年。其特征为快速、不可预测、不自主且无规律的收缩,主要影响四肢远端。它还可能与临床或亚临床心脏炎相关。在尼泊尔,SC仅在3.87%的急性风湿热病例中被报告为主要表现。
作者报告了一例12岁男孩,其右手运动异常且步态不稳达12天。检查时,他握力异常,难以维持强直性收缩(挤奶女工握法)。实验室检查显示抗链球菌溶血素O滴度和红细胞沉降率升高。超声心动图显示亚临床心脏炎。经过全面的临床检查和相关调查,最终诊断为ARF伴SC。
根据修订的琼斯标准,SC是风湿热的主要临床特征。它与先前的A组β溶血性(GABHS)感染有关。约50 - 65%的风湿热患者后来会发展为临床可检测到的心脏炎。尽管这是一种自限性疾病,但可能需要用抗癫痫药、抗精神病药和吩噻嗪类药物治疗。
任何出现运动障碍的儿童都应考虑患有SC,需要进行额外检查,包括心血管评估。它需要与其他运动障碍原因以及精神疾病相鉴别。对于干扰日常活动的中度至重度舞蹈病,治疗是必要的。遵守后续的抗生素预防对于避免未来的心脏并发症至关重要。