Idiopathic distal sensory polyneuropathy
ACTTION diagnostic criteria
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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.
Glossary
- CCM=
- corneal confocal microscopy;
- iDSP=
- idiopathic distal sensory polyneuropathy;
- IENFD=
- intraepidermal nerve fiber density;
- iLFN=
- idiopathic large fiber sensory neuropathy;
- iSFN=
- idiopathic small fiber sensory neuropathy;
- LF=
- large fiber;
- NCS=
- nerve conduction studies;
- QST=
- quantitative sensory testing;
- SF=
- small fiber
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Roy Freeman and Jennifer S. Gewandter are co–first authors.
↵* These authors contributed equally to this work as co-first authors.
Podcast: NPub.org/3cv4m2
- Received June 10, 2020.
- Accepted in final form September 21, 2020.
- © 2020 American Academy of Neurology
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