Last reviewed:September 2019
Last updated:February  2019

Summary

Definition

History and exam

Key diagnostic factors

  • disease progression
  • weakness
  • altered sensation
  • decreased deep tendon reflexes

Other diagnostic factors

  • incoordination
  • age 40 to 60 years
  • preceding infection
  • absence of exposure to neuropathy-causing drugs
  • dyspnea
  • facial weakness
  • dysarthria
  • dysphagia
  • urinary incontinence
  • urinary urgency or hesitancy
  • impotence
  • orthostatic hypotension
  • papilledema
  • vision loss
  • spasticity
  • ataxia

Risk factors

  • male gender
  • autoimmune diseases
  • diabetes
  • infection
  • monoclonal gammopathy of undetermined significance (MGUS)

Diagnostic investigations

1st investigations to order

  • nerve conduction studies (NCS)
  • cerebrospinal fluid (CSF) evaluation
Full details

Investigations to consider

  • nerve biopsy
  • MRI spine
  • nerve ultrasound
  • enzyme-linked immunosorbent assay (ELISA) or Western blot to detect autoantibodies
  • clinical trial of immunosuppressant
Full details

Treatment algorithm

Contributors

Glenn Lopate

Professor of Neurology

Department of Neurology

Washington University School of Medicine

St. Louis

MO

Disclosures

GL is on the medical advisory board for Alnylam Pharmaceuticals and a consultant for Gerson Lehrman Group. GL is an author of a reference cited in this monograph.

Peer reviewersVIEW ALL

Partner

Phoenix Neurological Associates Ltd

Phoenix

AZ

Disclosures

TL declares that he has no competing interests.

Professor

Cochrane Neuromuscular Disease Group

MRC Centre for Neuromuscular Disease

National Hospital for Neurology and Neurosurgery

London

UK

Disclosures

RACH has held or holds consultancies with companies that produce intravenous immunoglobulin (CSL Behring, LFB, Octapharma, Kedrion, and Talecris) and with other companies that produce immunomodulatory drugs (Biogen-Idec and Ares-Serono). RACH has received grants from the UK Department of Health for the Cochrane Neuromuscular Disease Review Group, Sandoz (now Novartis), and Ares-Serono. RACH is an author of a number of references cited in this monograph.

Professor

CHU Dupuytren

Service et Laboratoire de Neurologie

Limoges

France

Disclosures

Not disclosed.

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