特发性远端感觉性多发性神经病：ACTTION 诊断标准。
Idiopathic distal sensory polyneuropathy: ACTTION diagnostic criteria.
机构信息
From the Beth Israel Deaconess Medical Center (R.F., C.G.), Harvard Medical School, MA; University of Rochester Medical Center (J.S.G., R.H.D., D.N.H.), Rochester, NY; Department of Neurology (C.G.F.), School of Mental Health and Neuroscience, Maastricht University Medical Center+, Maastricht, the Netherlands; Department of Anesthesiology (S.H.), Washington University in St. Louis School of Medicine, St. Louis, MO; Neuroalgology Unit (G.L.), Fondazione IRCCS Istituto Neurologico "Carlo Besta," Milan, Italy; Department of Biomedical and Clinical Sciences "Luigi Sacco" (G.L.), University of Milan,Milan, Italy; Phoenix Neurological Associates (T.L.), Phoenix, AZ; Weill Cornell Medicine-Qatar (R.A.M.), Qatar Foundation, Education City, Doha, Qatar; University of Utah (J.R.S.), Salt Lake City, UT; Virginia Commonwealth University (A.G.S.), Richmond, VA; Biogen (J.B.), Cambridge, MA; University of Michigan (E.F.), Ann Arbor, MI; Johns Hopkins School of Medicine (A.H., M.P.), Baltimore, MD; University of Vermont (N.K.), Burlington, VT; Chromocell Corp (H.M.), North Brunswick, NJ; Harvard Medical School (A.L.O.), Boston, MA; Departments of Neurology and Medicine (A.P.), and Vanderbilt Heart and Vascular Institute, Nashville, TN; NuFactor Specialty Pharmacy (E.R.), Temecula, CA; University of Maryland (J.W.R.), Baltimore, MD; Aptinyx (S.S.), INC., Evanston. IL; Amgen (D.S.), Cambridge, MA; University of Haifa (R.T.), Haifa, Israel; and University of Würzburg (N.Ü.), Würzburg, Germany.
出版信息
Neurology. 2020 Dec 1;95(22):1005-1014. doi: 10.1212/WNL.0000000000010988. Epub 2020 Oct 14.
OBJECTIVE
To present standardized diagnostic criteria for idiopathic distal sensory polyneuropathy (iDSP) and its subtypes: idiopathic mixed fiber sensory neuropathy (iMFN), idiopathic small fiber sensory neuropathy (iSFN), and idiopathic large fiber sensory neuropathy (iLFN) for use in research.
METHODS
The Analgesic, Anesthetic, and Addiction Clinical Trial Translations, Innovations, Opportunities and Networks (ACTTION) public-private partnership with the Food and Drug Administration convened a meeting to develop consensus diagnostic criteria for iMFN, iSFN, and iLFN. After background presentations, a collaborative, iterative approach was used to develop expert consensus for new criteria.
RESULTS
An iDSP diagnosis requires at least 1 small fiber (SF) or large fiber (LF) symptom, at least 1 SF or LF sign, abnormalities in sensory nerve conduction studies (NCS) or distal intraepidermal nerve fiber density (IENFD), and exclusion of known etiologies. An iMFN diagnosis requires that at least 1 of the above clinical features is SF and 1 clinical feature is LF with abnormalities in sensory NCS or IENFD. Diagnostic criteria for iSFN require at least 1 SF symptom and at least 1 SF sign with abnormal IENFD, normal sensory NCS, and the absence of LF symptoms and signs. Diagnostic criteria for iLFN require at least 1 LF symptom and at least 1 LF sign with normal IENFD, abnormal sensory NCS, and absence of SF symptoms and signs.
CONCLUSION
Adoption of these standardized diagnostic criteria will advance research and clinical trials and spur development of novel therapies for iDSPs.
目的
提出特发性远端感觉性多发性神经病（iDSP）及其亚型的标准化诊断标准：特发性混合纤维感觉神经病（iMFN）、特发性小纤维感觉神经病（iSFN）和特发性大纤维感觉神经病（iLFN），用于研究。
方法
镇痛、麻醉和成瘾临床试验转化、创新、机遇和网络（ACTION）公私合作伙伴关系与食品和药物管理局召开会议，为 iMFN、iSFN 和 iLFN 制定共识诊断标准。在背景介绍后，采用协作、迭代的方法制定新的标准专家共识。
结果
iDSP 的诊断需要至少 1 个小纤维（SF）或大纤维（LF）症状，至少 1 个 SF 或 LF 体征，感觉神经传导研究（NCS）或远端表皮内神经纤维密度（IENFD）异常，以及排除已知病因。iMFN 的诊断需要至少 1 个上述临床特征为 SF，至少 1 个临床特征为 LF，感觉 NCS 异常和 IENFD。iSFN 的诊断标准需要至少 1 个 SF 症状和至少 1 个 SF 体征，伴有异常的 IENFD、正常的感觉 NCS 和无 LF 症状和体征。iLFN 的诊断标准需要至少 1 个 LF 症状和至少 1 个 LF 体征，伴有正常的 IENFD、异常的感觉 NCS 和无 SF 症状和体征。
结论
采用这些标准化诊断标准将推进 iDSP 的研究和临床试验，并刺激新型疗法的开发。
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