Braune S, Reinhardt M, Schnitzer R, Riedel A, Lücking C H
Neurologische, Universitätsklinik Freiburg, Germany.
Neurology. 1999 Sep 22;53(5):1020-5. doi: 10.1212/wnl.53.5.1020.
To improve the differential diagnosis between patients with multiple system atrophy (MSA) and idiopathic PD (IPD) with autonomic failure.
Some patients diagnosed with IPD are discovered to have alternative diseases such as MSA, despite the application of stringent diagnostic criteria. This differentiation is particularly difficult if patients with IPD also show symptoms of autonomic failure. In IPD, autonomic failure is caused by damage of the postganglionic part of the autonomic nervous system, whereas in MSA, degeneration of preganglionic and central autonomic neurons is revealed histopathologically.
Scintigraphy with [123I]metaiodobenzylguanidine (MIBG) enables the quantification of postganglionic sympathetic cardiac innervation. Fifteen patients with IPD and 5 patients with MSA underwent standard autonomic function tests and scintigraphy with MIBG.
In all patients, cardiovascular testing showed evidence of autonomic failure of varying severity. In all patients with IPD, the heart-mediastinum (H/M) ratio of MIBG uptake was pathologically impaired, independent of duration and severity of autonomic and parkinsonian symptoms. All patients with MSA had a regular H/M ratio. Each patient could be assigned to the correct diagnostic group based on the results of the MIBG scintigraphy, even if the duration of the disease was only 2 years or less.
This population assessment of the heart-mediastinum ratio of [123I]metaiodobenzylguanidine uptake showed a high sensitivity for the detection of autonomic involvement in patients with idiopathic IPD and also a high specificity for the discrimination between idiopathic PD and MSA.
改善多系统萎缩(MSA)患者与伴有自主神经功能衰竭的特发性帕金森病(IPD)患者之间的鉴别诊断。
尽管应用了严格的诊断标准,但一些被诊断为IPD的患者被发现患有其他疾病,如MSA。如果IPD患者也出现自主神经功能衰竭的症状,这种鉴别尤其困难。在IPD中,自主神经功能衰竭是由自主神经系统节后部分受损引起的,而在MSA中,病理组织学显示节前和中枢自主神经元发生变性。
用[123I]间碘苄胍(MIBG)进行闪烁扫描可对节后交感神经心脏支配进行定量分析。15例IPD患者和5例MSA患者接受了标准的自主神经功能测试和MIBG闪烁扫描。
所有患者的心血管测试均显示出不同程度的自主神经功能衰竭证据。在所有IPD患者中,MIBG摄取的心脏-纵隔(H/M)比值均存在病理性损害,与自主神经和帕金森症状的持续时间及严重程度无关。所有MSA患者的H/M比值均正常。即使疾病病程仅为2年或更短,根据MIBG闪烁扫描结果,每位患者都可被归入正确的诊断组。
对[123I]间碘苄胍摄取的心脏-纵隔比值进行的群体评估显示,其对检测特发性IPD患者自主神经受累具有较高的敏感性,对鉴别特发性PD和MSA也具有较高的特异性。