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Abstract Details

Diagnostic Criteria for Idiopathic Distal Sensory Polyneuropathy and Idiopathic Small Fiber Polyneuropathy
Neuromuscular and Clinical Neurophysiology (EMG)
P13 - Poster Session 13 (5:30 PM-6:30 PM)
1-010
To develop standardized diagnostic criteria for research and clinical use for two highly prevalent yet underserved neurological disorders. 
No cause is identified for distal sensory polyneuropathy (DSP) and small fiber neuropathy (SFN) in approximately 20-30% of cases. These idiopathic (i) DSPs have become attractive disorders for drug-development, given recent advances in identifying underlying genetic mechanisms in some patients, yet no evidence-based treatments exist.  One major barrier to therapeutic development is the lack of consensus diagnostic criteria. 
The Consortium on Clinical Endpoints and Procedures for Peripheral Neuropathy Trials (CONCEPPT) of the Analgesic, Anesthetic, and Addiction Clinical Trial Translations, Innovations, Opportunities and Networks (ACTTION) public-private partnership with the FDA convened a meeting of experts (i.e., the CONCEPPT DSP working group) to develop consensus diagnostic criteria for iDSP and iSFN.  After a series of background presentations including reviews of existing criteria for DSPs, a collaborative approach was used to develop expert consensus for new iDSP and iSFN criteria.
Diagnostic criteria were developed that included required symptoms, signs, neurophysiological and neuropathological assessments, and possible etiologies to be excluded. Requirements for an iDSP diagnosis include: one of the following symptoms (1) spontaneous or constant pain, (2) allodynia, (3) a non-painful sensory symptom; one of the following signs: (1) abnormal sensory perception (i.e., pinprick, light touch, vibration, or position sense), (2) allodynia, (3) hyperalgesia; abnormalities in sensory nerve conduction studies or distal intra-epidermal nerve fiber density; and absence of specific causes of DSP. Diagnostic criteria for iSFN are similar, except absence of abnormalities in large fiber clinical signs (e.g., vibration perception) and normal sensory nerve conduction studies are required. 

We propose that adoption of these standardized criteria will advance research and clinical trials, spur development of therapies for iDSP and iSFN, and facilitate generalization of research results to the clinic.
Authors/Disclosures
Roy L. Freeman, MD (Beth Israel Deaconess Hosp)
PRESENTER 		Dr. Freeman has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for Cutaneous Diagnostic Life Sciences. Dr. Freeman has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Vertex. Dr. Freeman has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Theravance. Dr. Freeman has received personal compensation in the range of $10,000-$49,999 for serving as an officer or member of the Board of Directors for Inhibikase. Dr. Freeman has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Elsevier. The institution of Dr. Freeman has received research support from NIH. The institution of Dr. Freeman has received research support from Theravance. The institution of Dr. Freeman has received research support from Biohaven. The institution of Dr. Freeman has received research support from Lundbeck. Dr. Freeman has received research support from Regeneron.
Jennifer Gewandter, PhD, MPH (University of Rochester) No disclosure on file
Robert H. Dworkin, PhD (University of Rochester School of Medicine and Dentistry) Robert H. Dworkin, PhD has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Acadia, Akigai, Allay, AM-Pharma, Analgesic Solutions, Beckley, Biogen, Biosplice, Bsense, Cardialen, Chiesi, Clexio, Collegium, CombiGene, Confo, Contineum, Eccogene, Editas, Eli Lilly, Emmes, Endo, Epizon, Exicure, GlaxoSmithKline, Glenmark, Gloriana, JucaBio, Kriya, Mainstay, Merck, Mind Medicine, NeuroBo, Noema, OliPass, Orion, Oxford Cannabinoid Technologies, Pfizer, Q-State, Regenacy, Rho, Sangamo, Semnur, SIMR Biotech, Sinfonia, SK Biopharmaceuticals, Sparian, SPM Therapeutics, SPRIM Health, Tiefenbacher, Validae, Vertex, Viscera, and WCG.
No disclosure on file
David N. Herrmann, MD, FÂ鶹´«Ã½Ó³»­ (University of Rochester Medical Center) Dr. Herrmann has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Acceleron. Dr. Herrmann has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Guidepoint global. Dr. Herrmann has received personal compensation in the range of $500-$4,999 for serving as a Consultant for GLG. Dr. Herrmann has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Neurogene. Dr. Herrmann has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sarepta. Dr. Herrmann has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Regenacy. Dr. Herrmann has received intellectual property interests from a discovery or technology relating to health care.
Ahmet Hoke, MD, PhD, FÂ鶹´«Ã½Ó³»­ (Johns Hopkins School of Medicine) Dr. Hoke has received personal compensation in the range of $50,000-$99,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Disarm. Dr. Hoke has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Elsevier. Dr. Hoke has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Annals of Clinical and Translational Neurology. Dr. Hoke has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for Burke and Thomas. Dr. Hoke has received stock or an ownership interest from Axoprotego Therapeutics. The institution of Dr. Hoke has received research support from NIH. The institution of Dr. Hoke has received research support from DOD. The institution of Dr. Hoke has received research support from Dr. Miriam and Sheldon G. Adelson Medical Research Foundation. The institution of Dr. Hoke has received research support from Foundation for Peripheral Neuropathy. Dr. Hoke has received intellectual property interests from a discovery or technology relating to health care.
Noah A. Kolb, MD (University of Vermont Medical Center) Dr. Kolb has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Abalone Medical. Dr. Kolb has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Disarm Therapeutics. Dr. Kolb has received personal compensation in the range of $0-$499 for serving as a Consultant for Eisana corporation. Dr. Kolb has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion Pharmaceuticals. Dr. Kolb has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for NINDS. Dr. Kolb has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB. Dr. Kolb has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Locks Law. Dr. Kolb has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for walk up law office . Dr. Kolb has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for ralston, pope, diehl. The institution of Dr. Kolb has received research support from National Cancer Institute.
Giuseppe Lauria, MD No disclosure on file
Rayaz A. Malik, MBBS, PhD (Weill Cornell Medicine) Prof. Malik has nothing to disclose.
Anne Louise Oaklander, MD, PhD, FÂ鶹´«Ã½Ó³»­ (Massachusetts General Hospital) The institution of Dr. Oaklander has received research support from National Institutes of Health.
Amanda C. Peltier, MD, MS (Vanderbilt University) Dr. Peltier has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Alnylam. Dr. Peltier has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for astrazeneca. Dr. Peltier has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for CSL Behring. Dr. Peltier has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Csl Behring. Dr. Peltier has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for argenx. Dr. Peltier has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for alnylam. Dr. Peltier has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for astrazeneca. Dr. Peltier has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Goldman Ismail. The institution of Dr. Peltier has received research support from NIH. The institution of Dr. Peltier has received research support from CSL Behring.
Michael J. Polydefkis, MD, FÂ鶹´«Ã½Ó³»­ (Johns Hopkins University School of Medicine) Dr. Polydefkis has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alnylam Pharmaceuticals. Dr. Polydefkis has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Akcea. Dr. Polydefkis has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Vertex Pharmaceutical . Dr. Polydefkis has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen-Idec. Dr. Polydefkis has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Pfizer.
James W. Russell, MD, MS, FRPC, FÂ鶹´«Ã½Ó³»­ (Department of Neurology) The institution of Dr. Russell has received research support from NIH. Dr. Russell has received publishing royalties from a publication relating to health care.
A. Gordon Smith, MD, FÂ鶹´«Ã½Ó³»­ (VCU Health System Department of Neurology) Dr. Smith has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion. Dr. Smith has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Argenx. Dr. Smith has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Merz. Dr. Smith has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sangamo. Dr. Smith has received personal compensation in the range of $500-$4,999 for serving as a Consultant for US Department of Justice. Dr. Smith has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Janssen. Dr. Smith has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Eidos. Dr. Smith has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Lexicon. Dr. Smith has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Alexion. The institution of Dr. Smith has received research support from NIH (NINDS, NIDDK). Dr. Smith has received publishing royalties from a publication relating to health care. Dr. Smith has received personal compensation in the range of $0-$499 for serving as a Study Section with NIH.
No disclosure on file
No disclosure on file