Summary
- An acquired demyelinating peripheral neuropathy of presumed autoimmune aetiology.
- Proximal symmetrical weakness is present in most patients. Atypical cases often have proximal asymmetrical weakness.
- Nerve conduction studies are the main diagnostic tool, but lack sensitivity; treatment should not be withheld if electrodiagnostic criteria are not met and symptoms are consistent with the diagnosis.
- Treatment includes intravenous immunoglobulin (IVIG), corticosteroids, and plasma exchange; long-term immunosuppressive therapy may be needed to prevent relapse. Lack of at least a partial response to 1 or 2 first-line agents should cast doubt on the diagnosis.
- Differentiation from Guillain-Barre syndrome (GBS) is important because corticosteroids may worsen patients with GBS.
Other related conditions
- Amyloidosis
- Amyotrophic lateral sclerosis
- Aspiration pneumonia
- Assessment of monoclonal gammopathies
- Cryoglobulinaemia
- Diabetic neuropathy
- Glomerulonephritis
- Graft-versus-host disease
- Graves' disease
- Guillain-Barre syndrome
- Hepatitis B
- Hepatitis C
- Irritable bowel syndrome
- Lyme disease
- Monoclonal gammopathy of undetermined significance
- Mononeuritis multiplex
- Multiple myeloma
- Non-Hodgkin's lymphoma
- HIV infection
- Overview of thyroid dysfunction
- Respiratory failure
- Sarcoidosis
- Systemic lupus erythematosus
- Ulcerative colitis
- Waldenström's macroglobulinaemia
Last updated: May 10, 2013
