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Neuromuscular
Review
Advances in diagnosis and management of distal sensory polyneuropathies
  1. Matthew Silsby1,2,
  2. Eva L Feldman3,
  3. Richard D Dortch4,5,6,
  4. Alison Roth4,
  5. Simon Haroutounian7,
  6. Yusuf A Rajabally8,
  7. Steve Vucic2,
  8. Michael E Shy9,
  9. Anne Louise Oaklander10,
  10. Neil G Simon11
  1. 1 Neurology, Westmead Hospital, Westmead, New South Wales, Australia
  2. 2 Brain and Nerve Research Centre, Sydney Medical School, The University of Sydney, New South Wales, Australia
  3. 3 Department of Neurology, University of Michigan, Ann Arbor, Michigan, USA
  4. 4 Division of Neuroimaging Research, Barrow Neurological Institute, Phoenix, Arizona, USA
  5. 5 Department of Radiology and Radiological Sciences, Vanderbilt University Institute of Imaging Science, Nashville, Tennessee, USA
  6. 6 Department of Biomedical Engineering, Vanderbilt University Institute of Imaging Science, Nashville, Tennessee, USA
  7. 7 Department of Anesthesiology, Washington University School of Medicine in Saint Louis, St Louis, Missouri, USA
  8. 8 Inflammatory Neuropathy Clinic, Department of Neurology, University Hospitals Birmingham, Aston Medical School, Aston University, Birmingham, UK
  9. 9 Department of Neurology, Carver College of Medicine, University of Iowa, Iowa City, Iowa, USA
  10. 10 Nerve Unit, Departments of Neurology and Pathology (Neuropathology), Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA
  11. 11 Northern Beaches Clinical School, Macquarie University, Frenchs Forest, New South Wales, Australia
  1. Correspondence to Neil G Simon, Northern Beaches Clinical School, Macquarie University, Frenchs Forest, New South Wales, Australia; neil{at}nbneuro.com.au

Abstract

Distal sensory polyneuropathy (DSP) is characterised by length-dependent, sensory-predominant symptoms and signs, including potentially disabling symmetric chronic pain, tingling and poor balance. Some patients also have or develop dysautonomia or motor involvement depending on whether large myelinated or small fibres are predominantly affected. Although highly prevalent, diagnosis and management can be challenging. While classic diabetes and toxic causes are well-recognised, there are increasingly diverse associations, including with dysimmune, rheumatological and neurodegenerative conditions. Approximately half of cases are initially considered idiopathic despite thorough evaluation, but often, the causes emerge later as new symptoms develop or testing advances, for instance with genetic approaches. Improving and standardising DSP metrics, as already accomplished for motor neuropathies, would permit in-clinic longitudinal tracking of natural history and treatment responses. Standardising phenotyping could advance research and facilitate trials of potential therapies, which lag so far. This review updates on recent advances and summarises current evidence for specific treatments.

  • NEUROGENETICS
  • NEUROIMMUNOLOGY
  • NEUROMUSCULAR
  • NEUROPATHY
  • PERIPHERAL NEUROPATHOLOGY

https://doi.org/10.1136/jnnp-2021-328489

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    Footnotes

    • Twitter @AlisonRoth14

    • Contributors MS: concept, drafting and revising the manuscript. ELF: concept, drafting and revising the manuscript. RDD: concept, drafting and revising the manuscript. AR: concept, drafting and revising the manuscript. SH: concept, drafting and revising the manuscript. YAR: concept, drafting and revising the manuscript. SV: concept, drafting and revising the manuscript. MES: concept, drafting and revising the manuscript. ALO: concept, drafting and revising the manuscript. NGS: concept, drafting and revising the manuscript.

    • Funding RDD reports funding from the US Department of Defence (PR211292), US Department of Health and Human Services, National Institutes of Health, National Centre for Advancing Translational Sciences (R21 TR003312) and the Barrow Neurological Foundation. ELF is supported by the US Department of Health and Human Services National Institutes of Health (R24DK082841, R01DK130913) and the Juvenile Diabetes Research Foundation (5-COE-2019-861-s—B). SH reports funding from US Department of Health and Human Services, National Institutes of Health, National Institute of Neurological Disorders and Stroke (R01NS104500-01) and the US Department of Defence (W81XWH2110736). ALO reports funding from US Department of Health and Human Services National Institutes of Health (R01NS093653), US Department of Defence (GW140169) and the Curvey, Cutler and Mayday Foundations. MES reports funding from US Department of Health and Human Services, National Institutes of Health, National Centre for Advancing Translational Sciences for the Inherited Neuropathy Consortium ((U54NS065712) MES also receives support from the Muscular Dystrophy Association and Charcot-Marie-Tooth Association. MES also receives support from US Department of Health and Human Services, National Institutes of Health, National Institute of Neurological Disorders and Stroke (grant numbers R01NS105755 and U01 NS1094301). SV reports funding support from the Department of Health, Australian Government, National Health and Medical Research Council (project grant numbers 1024915, 2001261).

    • Competing interests MSilsby, ELF, RDD, AR, SV, ALO and NGS reports no competing interests. SH reports personal fees from Rafa Laboratories, Vertex Pharmaceuticals and GW Pharmaceuticals, and research grants from Eli Lilly, outside the scope of this work. YAR has received speaker/consultancy honoraria from LFB, Polyneuron and Argenx and has received educational sponsorships from LFB and CSL Behring and has obtained research grants from LFB and CSL Behring. MShy has no competing interests related to this publication. He serves as a consultant for Applied Therapeutics, DTx Pharma, Mitochondria in Motion, Swan Biosci and Inflectis.

    • Provenance and peer review Commissioned; externally peer reviewed.

    • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.