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慢性炎症性脱髓鞘性多发性神经病诊断标准的推导与验证

Derivation and validation of diagnostic criteria for chronic inflammatory demyelinating polyneuropathy.

作者信息

Koski C L, Baumgarten M, Magder L S, Barohn R J, Goldstein J, Graves M, Gorson K, Hahn A F, Hughes R A C, Katz J, Lewis R A, Parry G J, van Doorn P, Cornblath D R

机构信息

University of Maryland School of Medicine, Department of Neurology, Baltimore, MD, USA.

出版信息

J Neurol Sci. 2009 Feb 15;277(1-2):1-8. doi: 10.1016/j.jns.2008.11.015. Epub 2008 Dec 16.

DOI:10.1016/j.jns.2008.11.015
PMID:19091330
Abstract

To develop diagnostic criteria for chronic inflammatory demyelinating polyneuropathy (CIDP), a retrospective series of patients' records diagnosed by sexpert consensus as CIDP or other chronic polyneuropathies were analyzed. Classification and regression tree analysis was applied to 150 patients to derive a classification rule. According to the rule, diagnosis of CIDP required that a patient have a chronic non-genetic polyneuropathy, progressive for at least eight weeks, without a serum paraprotein and either 1) recordable compound muscle action potentials in > or =75% of motor nerves and either abnormal distal latency in >50% of nerves or abnormal motor conduction velocity in >50% of nerves or abnormal F wave latency in >50% of nerves; or 2) symmetrical onset of motor symptoms, symmetrical weakness of four limbs, and proximal weakness in > or =1 limb. When validated in 117 patients, the rule had 83% sensitivity (95% confidence interval 69%-93%) and 97% specificity (95% confidence interval 89%-99%) and performed better than published criteria.

摘要

为制定慢性炎性脱髓鞘性多发性神经病(CIDP)的诊断标准,对一系列经专家共识诊断为CIDP或其他慢性多发性神经病的患者记录进行了回顾性分析。对150例患者应用分类与回归树分析得出分类规则。根据该规则,CIDP的诊断要求患者患有慢性非遗传性多发性神经病,病程至少进展8周,无血清副蛋白,且满足以下条件之一:1)在≥75%的运动神经中可记录到复合肌肉动作电位,且在>50%的神经中存在异常远端潜伏期或>50%的神经中存在异常运动传导速度或>50%的神经中存在异常F波潜伏期;或2)运动症状对称起病,四肢对称性无力,且≥1个肢体存在近端无力。在117例患者中进行验证时,该规则的敏感性为83%(95%置信区间69%-93%),特异性为97%(95%置信区间89%-99%),且表现优于已发表的标准。

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