Brain MRI of multiple system atrophy of cerebellar type: a prospective study with implications for diagnosis criteria
Brain MRI of multiple system atrophy of cerebellar type: a prospective study with implications for diagnosis criteria
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DOI：
10.1007/s00415-020-09702-w
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发表时间：
2020-05-01
影响因子：
6
通讯作者：
Tranchant, C.
中科院分区：
文献类型：
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作者：
Carre, G.;Dietemann, J. L.;Tranchant, C.
目的对小脑多系统萎缩型（MSA-C）的MRI诊断提出第二个共识，即脑桥和小脑中脚（MCP）萎缩是MSA-C的MRI表现。然而，其他MRI异常，如MCP高信号，热十字面包征（HCB），壳核低信号和高信号壳核边缘已被描述。目的评价散发性迟发性小脑性共济失调（SLOCA）患者的几种MRI特征对MSA-C的鉴别诊断价值，跟踪其在MSA-C病程中的演变，并寻找这些MRI特征与临床体征之间的相关性。方法回顾性分析2005年1月至2010年12月收治的10例SLOCA患者的临床资料、实验室检查、头颅MRI、DaTscan及随访1年。结果80例患者中，26例为MSA-C型，22例为其他诊断，32例未诊断。基线时，最终诊断为MSA-C的患者中MCP高信号和HCB比其他SLOCA患者更常见（p < 0.0001），与所有其他MRI体征相比，MSA-C诊断的特异性（98.5%）和阳性预测值（91.7%）最高。与HCB征最相关的MRI序列是T2质子密度（DP）加权。所有MRI特征随病程增加而增加。除了小脑蚓部萎缩与CARSIII评分之间的相关性外，任何MRI特征与临床数据或多巴胺能神经元丢失之间均无相关性（p = 0.5008）。结论MCP高信号和HCB征应列入MSA-C的附加特征。MRI信号异常提示MSA-C应在适当的顺序搜索。
Aim The second consensus statement for the diagnosis of multiple system atrophy type cerebellar (MSA-C) includes pons and middle cerebellar peduncle (MCP) atrophy as MRI features. However, other MRI abnormalities such as MCP hyperintensity, hot cross bun sign (HCB), putaminal hypointensity and hyperintense putaminal rim have been described. Objectives To evaluate, in patients with sporadic late-onset cerebellar ataxia (SLOCA), the discriminative value of several MRI features for the diagnosis of MSA-C, to follow their evolution during the course of MSA-C, and to search for correlations between these MRI features and clinical signs. Methods Consecutive patients referred for SLOCA underwent comprehensive clinical evaluation and laboratory investigations, brain MRI, DaTscan and a 1-year follow-up. Results Among 80 patients, 26 had MSA-C, 22 another diagnosis, and 32 no diagnosis at the end of the follow-up. At baseline, MCP hyperintensity and HCB were more frequent in patients finally diagnosed with MSA-C than in other patients with SLOCA (p < 0.0001), and had the highest specificity (98.5%) and positive predictive value (91.7%) for the diagnosis of MSA-C, compared to all other MRI signs. The most relevant MRI sequence regarding HCB sign was the T2-proton density (DP) weighted. All MRI features were more frequent with disease duration. No correlation was found between any MRI feature and neither clinical data, nor dopaminergic neuronal loss (p = 0.5008), except between vermis atrophy and UPDRSIII score. Conclusion MCP hyperintensity and HCB sign should be added into the list of additional features of possible MSA-C. MRI signal abnormalities suggestive of MSA-C should be searched for in suitable sequence.