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
Investigations to consider
- nerve biopsy
- MRI spine
- nerve ultrasound
- enzyme-linked immunosorbent assay (ELISA) or Western blot to detect autoantibodies
- clinical trial of immunosuppressant
Treatment algorithm
no significant impact on function and quality of life
significant impact on function and quality of life
partial or no response to initial monotherapy
refractory to combination therapy with 2 initial agents
response to treatment
no response to treatment
Contributors
Authors
Glenn Lopate, MD

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 reviewers
Todd Levine, MD
Partner
Phoenix Neurological Associates Ltd
Phoenix
AZ
Disclosures
TL declares that he has no competing interests.
Richard A.C. Hughes, MD, FRCP, FMedSci
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.
Jean-Michel Vallat, MD
Professor
CHU Dupuytren
Service et Laboratoire de Neurologie
Limoges
France
Disclosures
Not disclosed.
Differentials
- Guillain-Barre syndrome (GBS)
- Hereditary motor and sensory neuropathy (HMSN)
- Anti-myelin-associated glycoprotein (anti-MAG) neuropathy
More DifferentialsGuidelines
- Guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy
- Recommendations on diagnostic strategies for chronic inflammatory demyelinating polyradiculoneuropathy
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