Department of Neurology and Clinical Neurophysiology, Leiden University Medical Centre, PO Box 9600, 2300 RC Leiden, the Netherlands.
Department of Neurology and Clinical Neurophysiology, St. Antonius Hospital, Nieuwegein, the Netherlands.
Auton Neurosci. 2021 Mar;231:102773. doi: 10.1016/j.autneu.2021.102773. Epub 2021 Jan 26.
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.
To investigate whether several new non-invasive autonomic tests are beneficial in discriminating between PAF and MSA.
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.
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.
The tests applied in this study may help to confirm autonomic failure but did not support the differential diagnosis between PAF and MSA.
单纯自主神经衰竭(PAF)和多系统萎缩(MSA)是引起严重自主神经衰竭的罕见疾病。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,改善鉴别诊断将对患者和家属产生重要影响。
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。
患者和对照组接受了三项测试,分别检查皮肤的自主神经支配(Sudoscan 和水诱导的皮肤起皱)和一项测量眼部视网膜神经纤维层厚度的测试。
皮肤血管运动测试显示疾病组和对照组之间存在差异,但无法区分 PAF 和 MSA。组间视网膜神经纤维层厚度无差异。
本研究中应用的测试有助于确认自主神经衰竭,但不支持 PAF 和 MSA 之间的鉴别诊断。