多系统萎缩患者143例临床和影像学特征分析

目的 探讨多系统萎缩(MSA)不同亚型的临床和影像学特征及其相关性.方法 对143例符合1999年Gilman诊断标准的MSA患者进行临床分型和诊断分级,根据Horimoto分期对108例影像学出现异常的患者脑桥十字征和壳核裂隙征进行分析,并探讨不同临床亚型及病程与影像学异常的相关性.结果 143例MSA患者男女比例为1.3:1,其中MSA小脑萎缩型(MSA-C)93例,MSA帕金森型(MSA-P)39例,两者同时出现的即为MSA-P+C型11例;很可能的MSA 90例,可能的MSA 53例.108例MSA患者影像学出现异常,其中MSA-C型患者36例(36/76,47%)出现脑桥十字征,10例(10/76,13%)出现壳核裂隙征;MSA-P型患者6例(6/24,25%)出现脑桥十字征,6例(6/24,25%)出现壳核裂隙征.MSA-C型中病程较短的患者脑桥十字征分期较早.结论 本组病例中MSA-C型患者明显多于MSA-P型,可能与种族遗传背景有关.脑桥十字征和壳核裂隙征为MSA患者的显著影像学特征,MSA临床分型与影像学特征具有一定的相关性,其中脑桥十字征在MSA-C型较为显著,壳核裂隙征在MSA-P型较为显著.

Clinical and neuroimaging features of 143 patients with multiple system atrophy

Objective To study the clinical and neuroimaging features of subtypes of multiple system atrophy (MSA) and their correlations. Methods One hundred and forty-three MSA cases fulfilled Gilman diagnostic criteria (1999) were recruited and their clinical subtypes and stages were classified. Using the staging methods of the pontine cross sign and putaminal slit proposed by Horimoto, 108 patients showed abnormalities in MRI and were further evaluated. The relationship between the subtypes of MSA, disease duration, and MRI abnormalities has been analyzed. Results Of 143 MSA patients, the male-to-female ratio is 1.3:1 ; 93 cases are diagnosed with MSA-C, 39 with MSA-P, and 11 with MSA-P + C; 90 cases with probable diagnosis, and 53 with possible diagnosis. Of the 76 MSA-C cases with MRI abnormalities, 36 (47%) show the pontine cross sign and 10 (13%) show the putaminal slit; of the 24 MSA-P cases with MRI abnormalities, 6 (25%) show the pontine cross sign and 6 (25%) show theputaminal slit. In addition, MSA-C cases with shorter disease duration demonstrate earlier stages of the pontine cross sign. Conclusions In this study, the number of MSA-C cases is more than MSA-P, which might be related to the ethnic background. In neuroimaging, both the pontine cross sign and the putaminal slit are the marked features of MSA. To some degree, the subtypes of MSA are related with the features of imaging, that is, MSA-C patients present the pontine cross sign more often than MSA-P, and the putaminal slit is a comparatively common feature among MSA-P cases.