Chronic inflammatory demyelinating polyneuropathy - Symptoms, Causes, Images, and Treatment Options

Diseases

Highlights & Basics

Key Highlights

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired demyelinating peripheral neuropathy of presumed autoimmune etiology.
Proximal and distal symmetric weakness (often without pain) is typical of CIDP. Symptoms of weakness and sensory loss progress beyond 8 weeks. CIDP variants ("atypical" CIDP) can result in proximal or distal asymmetric weakness with sensory loss, predominantly distal weakness with sensory loss, pure motor symptoms, or pure sensory symptoms, sometimes associated with ataxia.
Diagnosis is based on a combination of clinical history, symptoms, exam findings, and electrodiagnostic testing. However, a treatment trial should not be withheld if electrodiagnostic criteria are not met, if history, symptoms, and clinical exam findings are consistent with the diagnosis.
Initial treatment is with intravenous immune globulin, corticosteroids, or plasma exchange. If there is insufficient response to first-line treatment, an alternative agent should be tried, followed by combination therapy. Lack of at least a partial response to one or two first-line agents should lead to reevaluation of the diagnosis; an alternative immunosuppressant may be added to treat confirmed refractory CIDP. Long-term immunosuppressive therapy may be needed to prevent relapse.
Differentiation from Guillain-Barre syndrome (GBS) is important, because treatment and management are different for the two conditions, and corticosteroids may worsen GBS.

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Citations

Key Articles

Van den Bergh PYK, van Doorn PA, Hadden RDM, et al. European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force - second revision. Eur J Neurol. 2021 Nov;28(11):3556-83.[Abstract][Full Text]
Dyck PJB, Tracy JA. History, diagnosis, and management of chronic inflammatory demyelinating polyradiculoneuropathy. Mayo Clin Proc. 2018 Jun;93(6):777-93.[Abstract][Full Text]
Broers MC, Bunschoten C, Drenthen J, et al. Misdiagnosis and diagnostic pitfalls of chronic inflammatory demyelinating polyradiculoneuropathy. Eur J Neurol. 2021 Jun;28(6):2065-73.[Abstract][Full Text]
Oaklander AL, Lunn MP, Hughes RA, et al. Treatments for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): an overview of systematic reviews. Cochrane Database Syst Rev. 2017 Jan 13;(1):CD010369.[Abstract][Full Text]
Mahdi-Rogers M, Brassington R, Gunn AA, et al. Immunomodulatory treatment other than corticosteroids, immunoglobulin and plasma exchange for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev. 2017 May 8;(5):CD003280.[Abstract][Full Text]

Referenced Articles

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