A diagnostic decision tree for adult cerebellar ataxia based on pontine magnetic resonance imaging
A diagnostic decision tree for adult cerebellar ataxia based on pontine magnetic resonance imaging
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DOI：
10.1016/j.jns.2018.02.022
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发表时间：
2018-04-15
影响因子：
4.4
通讯作者：
Yokota, Takanori
中科院分区：
文献类型：
--
作者：
Higashi, Miwa;Ozaki, Kokoro;Yokota, Takanori
小脑共济失调（CA）是异质性条件往往需要鉴别诊断。本研究的目的是建立一个诊断决策树的基础上桥脑MRI表现鉴别CA。将202例共济失调患者临床分为4组：（1）脊髓小脑共济失调（SCA）伴脑干受累（SCA-BSI），（2）单纯小脑SCA，（3）小脑显性多系统萎缩（MSA-c）和（4）其他CA。脑桥信号强度分为3种类型：热十字征（HCBS）、桥脑中线T2高信号（PMH）或正常。测量脑桥基底部与被盖部的距离比，称为“BT比”。HCBS的存在表明特异性为97.7%的MSA-c或SCA 2。当观察到PMH时，BT比率高于3.54强烈表明SCA-BSI，即Machado-Joseph病，SCA 1或齿状核红核-苍白球路易体萎缩，而BT比率低于3.54表明MSA-c或SCA 2。当信号强度正常时，BT比值高于3.52提示SCA-BSI，而BT比值低于3.52提示单纯小脑型SCA或其他CA伴单纯小脑型。该决策树在不同的30例CA患者中被证实是有用的。我们建议结合脑桥MRI信号强度变化和BT比率可以支持CA的鉴别诊断。
Cerebellar ataxias (CAs) are heterogeneous conditions often require differential diagnosis. This study aimed to establish a diagnostic decision tree for differentiating CAs based on pontine MRI findings. Two-hundred and two consecutive ataxia patients were clinically classified into 4 groups: (1) spinocerebellar ataxia (SCA) with brainstem involvement (SCA-BSI), (2) Pure cerebellar SCA, (3) cerebellar dominant multiple system atrophy (MSA-c), and (4) Other CA. Signal intensity in pons was graded into 3 types: hot cross bun sign (HCBS), pontine midline linear T2-hyperintensity (PMH), or normal. The distance ratio of pontine base to tegmentum, named "BT-ratio", was measured. The presence of HCBS indicated either MSA-c with a specificity of 97.7%, or SCA2. When PMH was observed, a BT-ratio above 3.54 strongly indicated SCA-BSI, namely Machado-Joseph disease, SCA1, or dentatorubral-pallidoluysian atrophy, whereas a BT-ratio below 3.54 indicated MSA-c or SCA2. When the signal intensity was normal, a BT-ratio above 3.52 indicated SCA-BSI, whereas a BT-ratio below 3.52 suggested Pure cerebellar SCA or Other CA with pure cerebellar type. The decision tree was confirmed useful in a different 30 CA patients. We propose that differential diagnosis of CAs can be supported by combining pontine MRI signal intensity changes and BT-ratio.