Idiopathic distal sensory polyneuropathy: ACTTION diagnostic criteria

Roy Freeman¹, Jennifer S Gewandter², Catharina G Faber², Christopher Gibbons², Simon Haroutounian², Giuseppe Lauria², Todd Levine², Rayaz A Malik², J Robinson Singleton², A Gordon Smith², Josh Bell², Robert H Dworkin², Eva Feldman², David N Herrmann², Ahmet Hoke², Noah Kolb², Heikki Mansikka², Anne Louise Oaklander², Amanda Peltier², Michael Polydefkis², Elissa Ritt², James W Russell², Stephen Sainati², Deborah Steiner², Roi Treister², Nurcan Üçeyler²

Affiliations

¹ 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. rfreeman@bidmc.harvard.edu.
² 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.

PMID: 33055271
PMCID: PMC7734920
DOI: 10.1212/WNL.0000000000010988

Review

Idiopathic distal sensory polyneuropathy: ACTTION diagnostic criteria

Roy Freeman et al. Neurology. 2020.

. 2020 Dec 1;95(22):1005-1014.

doi: 10.1212/WNL.0000000000010988. Epub 2020 Oct 14.

Authors

Affiliations

¹ 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. rfreeman@bidmc.harvard.edu.
² 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.

PMID: 33055271
PMCID: PMC7734920
DOI: 10.1212/WNL.0000000000010988

Abstract

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.

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References

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Idiopathic distal sensory polyneuropathy: ACTTION diagnostic criteria

Affiliations

Idiopathic distal sensory polyneuropathy: ACTTION diagnostic criteria

Authors

Affiliations

Abstract

References

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Grants and funding

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Full Text Sources