An acquired demyelinating peripheral neuropathy of presumed autoimmune etiology.
Proximal symmetric weakness is present in most patients. Atypical cases often have proximal asymmetric 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 immune globulin, subcutaneous immune globulin, corticosteroids, and plasma exchange; long-term immunosuppressive therapy may be needed to prevent relapse. Lack of at least a partial response to one or two first-line agents should cast doubt on the diagnosis.
Differentiation from Guillain-Barre syndrome (GBS) is important because corticosteroids may worsen patients with GBS.
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired demyelinating peripheral neuropathy of presumed autoimmune etiology. The course is usually either chronic progressive (over >8 weeks) or relapsing and remitting. The clinical phenotype consists of proximal and distal symmetric weakness, distal sensory loss, and absent reflexes.
History and exam
Key diagnostic factors
- disease progression
- altered sensation
- decreased deep tendon reflexes
Other diagnostic factors
- age 40 to 60 years
- preceding infection
- absence of exposure to neuropathy-causing drugs
- facial weakness
- urinary incontinence
- urinary urgency or hesitancy
- orthostatic hypotension
- vision loss
- male gender
- autoimmune diseases
- monoclonal gammopathy of undetermined significance (MGUS)
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
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
Glenn Lopate, MD
Professor of Neurology
Department of Neurology
Washington University School of Medicine
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.
Todd Levine, MD
Phoenix Neurological Associates Ltd
TL declares that he has no competing interests.
Richard A.C. Hughes, MD, FRCP, FMedSci
Cochrane Neuromuscular Disease Group
MRC Centre for Neuromuscular Disease
National Hospital for Neurology and Neurosurgery
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
Service et Laboratoire de Neurologie
- Guillain-Barre syndrome (GBS)
- Hereditary motor and sensory neuropathy (HMSN)
- Anti-myelin-associated glycoprotein (anti-MAG) neuropathy
- Guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy
- Recommendations on diagnostic strategies for chronic inflammatory demyelinating polyradiculoneuropathy
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