Background

Pure autonomic failure (PAF) and multiple system atrophy (MSA) are rare disorders causing severe autonomic failure. Their initially similar clinical presentation may lead to years of diagnostic difficulties. Improving the differentiation would have an important impact on patients and families in view of better prediction of disease progression.

Objective

To investigate whether several new non-invasive autonomic tests are beneficial in discriminating between PAF and MSA.

Methods

Patients and controls underwent two tests examining the autonomic innervation of the skin (Sudoscan and water-induced skin wrinkling) and one test measuring retinal nerve fiber layer thickness in the eye.

Results

The skin vasomotor tests yielded differences between the disease and control groups, but did not discriminate between PAF and MSA. No differences in retinal nerve fiber layer thickness were found between the groups.

Conclusion

The tests applied in this study may help to confirm autonomic failure but did not support the differential diagnosis between PAF and MSA.

中文翻译：

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新型非侵入性自主神经测试能否帮助区分纯自主神经功能衰竭和多系统萎缩？

背景

纯自主神经功能衰竭 (PAF) 和多系统萎缩 (MSA) 是导致严重自主神经功能衰竭的罕见疾病。它们最初相似的临床表现可能导致多年的诊断困难。鉴于更好地预测疾病进展，改善分化将对患者和家庭产生重要影响。

客观的

研究几种新的非侵入性自主神经测试是否有助于区分 PAF 和 MSA。

方法

患者和对照组接受了两项检查皮肤自主神经支配的测试（Sudoscan 和水引起的皮肤皱纹）和一项测量眼睛视网膜神经纤维层厚度的测试。

结果

皮肤血管舒缩试验在疾病组和对照组之间产生差异，但没有区分 PAF 和 MSA。各组之间未发现视网膜神经纤维层厚度存在差异。

结论

本研究中应用的测试可能有助于确认自主神经功能衰竭，但不支持 PAF 和 MSA 之间的鉴别诊断。