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[伴有小纤维受累的周围神经病的临床、电生理及皮肤活检研究:34例报告]

[Clinical, electrophysiological and skin biopsy studies of peripheral neuropathy with small fibers involvement: a report of 34 cases].

作者信息

Sun Bo, Li Yifan, Liu Lizhi, Chen Zhaohui, Ling Li, Yang Fei, Liu Jiexiao, Liu Hong, Zhu Wenjia, Huang Xusheng

机构信息

Department of Neurology, Chinese PLA General Hospital, Beijing 100853, China.

Department of Neurology, Chinese PLA General Hospital, Beijing 100853, China. Email:

出版信息

Zhonghua Yi Xue Za Zhi. 2014 Nov 25;94(43):3397-401.

Abstract

OBJECTIVE

To explore the relationship between clinical features, electrophysiology and intraepidermal nerve fiber density (IENFD) in peripheral neuropathy with small fibers involvement and determine the diagnostic value of 13-item small-fiber neuropathy and symptoms inventory questionnaire (SFN-SIQ) and neuropathy symptom score [lower limb] (NSS [LL]) in small fiber neuropathy (SFN).

METHODS

A total of 34 consecutive patients with peripheral neuropathy with symptoms of small fibers were enrolled and divided into two groups of small fiber injury and small and large fiber injury.SFN-SIQ, NSS [LL] and neuropathy disability score [lower limb] (NDS [LL]) were administered.Nerve conduction studies and skin biopsy were conducted in unilateral lower limb. The relationship between IENFD and these scales was assessed by partial correlation.Receiver operating characteristic analysis was applied for evaluating the diagnostic value of SFN-SIQ and NSS [LL] in small fiber injury. Independent sample t test was used to compare various parameters of two groups. And similar statistical method was used for IENFD abnormal and normal groups to detect the clinicoelectrophysiological differences.

RESULTS

According to the international normative reference of IENFD, 13 patients could be diagnosed with peripheral neuropathy with small fibers involvement. IENFD was moderately correlated with SFN-SIQ (r = 0.437, P = 0.012) and marginally correlated with NSS [LL] (r = 0.334, P = 0.062). The diagnostic value of SFN-SIQ and NSS [LL] was moderate for small fiber injury (Az = 0.753, P = 0.012 for SFN-SIQ, Az = 0.712, P = 0.040 for NSS [LL]) and the best diagnostic indicator of each scale was 6. The value of NDS [LL] was apparently elevated in small and large fiber injury group versus small fiber injury group (t = -5.605, P < 0.001). The IENFD abnormal group had a higher NSS [LL] value than that of the IENFD normal group (t = -2.047, P = 0.049). No differences of electrophysiological parameters existed between IENFD abnormal and normal groups.

CONCLUSION

Chinese normative reference of IENFD should be formulated for the diagnosis of small fiber neuropathy. SFN-SIQ and NSS [LL] may screen for small fiber neuropathy and both are convenient during patient follow-ups.Large sample studies are warranted to further evaluate the clinical values of SFN-SIQ and NSS [LL].

摘要

目的

探讨小纤维受累的周围神经病临床特征、电生理与表皮内神经纤维密度(IENFD)之间的关系,并确定13项小纤维神经病及症状量表问卷(SFN-SIQ)和下肢神经病症状评分(NSS[LL])在小纤维神经病(SFN)中的诊断价值。

方法

连续纳入34例有小纤维症状的周围神经病患者,分为小纤维损伤组和小、大纤维损伤组。采用SFN-SIQ、NSS[LL]和下肢神经病残疾评分(NDS[LL])。对单侧下肢进行神经传导研究和皮肤活检。通过偏相关分析评估IENFD与这些量表之间的关系。应用受试者工作特征分析评估SFN-SIQ和NSS[LL]在小纤维损伤中的诊断价值。采用独立样本t检验比较两组的各项参数。对IENFD异常组和正常组采用类似的统计方法检测临床电生理差异。

结果

根据IENFD的国际规范参考标准,13例患者可诊断为小纤维受累的周围神经病。IENFD与SFN-SIQ中度相关(r = 0.437,P = 0.012),与NSS[LL]边缘相关(r = 0.334,P = 0.062)。SFN-SIQ和NSS[LL]对小纤维损伤的诊断价值中等(SFN-SIQ的Az = 0.753,P = 0.012;NSS[LL]的Az = 0.712,P = 0.040),各量表的最佳诊断指标均为6。与小纤维损伤组相比,小、大纤维损伤组的NDS[LL]值明显升高(t = -5.605,P < 0.001)。IENFD异常组的NSS[LL]值高于IENFD正常组(t = -2.047,P = 0.049)。IENFD异常组和正常组之间的电生理参数无差异。

结论

应制定IENFD的中国规范参考标准用于小纤维神经病的诊断。SFN-SIQ和NSS[LL]可用于筛查小纤维神经病,且在患者随访过程中都很方便。需要大样本研究进一步评估SFN-SIQ和NSS[LL]的临床价值。

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