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.
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired demyelinating peripheral neuropathy of presumed immune-related etiology. The course is usually either chronic progressive (>8 weeks), relapsing and remitting, or stepwise. The clinical phenotype is typically characterized by symmetric proximal and distal weakness, distal large-fiber sensory loss, and absent or reduced 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 sex
- autoimmune diseases
- diabetes mellitus
- monoclonal gammopathy of undetermined significance (MGUS)
1st investigations to order
- nerve conduction studies
Investigations to consider
- cerebrospinal fluid (CSF) evaluation
- nerve ultrasound
- MRI spine and plexus with and without contrast
- clinical trial of therapy
- nerve biopsy
- enzyme-linked immunosorbent assay (ELISA) or Western blot to detect autoantibodies
- other tests
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
- Guillain-Barre syndrome (GBS)
- Charcot-Marie-Tooth disease (CMT)
- Anti-myelin-associated glycoprotein (anti-MAG) neuropathy
- 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
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