Snider Lisa A, Sachdev Vandana, MaCkaronis Julia E, St Peter Marilyn, Swedo Susan E
Pediatric Developmental and Neuropsychiatry Branch, National Institute of Mental Health, National Institutes of Health, Department of Health and Human Services, 10 Center Dr, Room 4N208, MSC 1255, Bethesda, MD 20892-1255, USA.
Pediatrics. 2004 Dec;114(6):e748-51. doi: 10.1542/peds.2004-0308. Epub 2004 Nov 15.
Sydenham's chorea is the neurologic manifestation of rheumatic fever and is a diagnosis of exclusion requiring only the presence of frank chorea in the absence of another neurologic disorder. Two thirds of children with Sydenham's chorea also have rheumatic carditis (pathologic mitral valve regurgitation). Although there are similar neuropsychiatric symptoms and preceding group A beta-hemolytic streptococcal infection associated with both Sydenham's chorea and the PANDAS (pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections) subgroup, it is unknown whether patients in the PANDAS subgroup have any cardiac involvement.
Sixty children meeting the criteria for PANDAS were entered into protocols at National Institute of Mental Health between 1993 and 2002. Doppler and 2-dimensional echocardiograms were performed on these subjects to assess valvular heart disease.
Of these 60 children, no echocardiographic evidence of significant mitral or aortic valve regurgitation was found. One patient was found to have mild mitral regurgitation, and all patients had normal left atrial size and normal left ventricular size and function. Follow-up echocardiograms on 20 children showed no significant valvular regurgitation.
The evidence of a clear lack of rheumatic carditis in these children supports the hypothesis that PANDAS is a distinct neuropsychiatric diagnosis separate from Sydenham's chorea.
Sydenham舞蹈病是风湿热的神经系统表现,是一种排除性诊断,仅需在无其他神经系统疾病的情况下存在明显的舞蹈病即可。三分之二的Sydenham舞蹈病患儿也患有风湿性心脏病(病理性二尖瓣反流)。尽管Sydenham舞蹈病和PANDAS(与链球菌感染相关的小儿自身免疫性神经精神疾病)亚组都有相似的神经精神症状且之前都有A组β溶血性链球菌感染,但尚不清楚PANDAS亚组患者是否有任何心脏受累情况。
1993年至2002年间,60名符合PANDAS标准的儿童进入美国国立精神卫生研究所的研究方案。对这些受试者进行多普勒和二维超声心动图检查以评估瓣膜性心脏病。
在这60名儿童中,未发现二尖瓣或主动脉瓣明显反流的超声心动图证据。发现1例患者有轻度二尖瓣反流,所有患者左心房大小、左心室大小及功能均正常。对20名儿童的随访超声心动图显示无明显瓣膜反流。
这些儿童明显缺乏风湿性心脏病的证据支持了以下假设,即PANDAS是一种与Sydenham舞蹈病不同的独特神经精神诊断。