Chronic inflammatory demyelinating polyradiculoneuropathy

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired demyelinating peripheral neuropathy of presumed autoimmune aetiology.

Proximal and distal symmetrical 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 asymmetrical 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, examination findings, and electrodiagnostic testing. However, a treatment trial should not be withheld if electrodiagnostic criteria are not met, if history, symptoms, and clinical examination findings are consistent with the diagnosis.

Initial treatment is with intravenous immunoglobulin, 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 re-evaluation 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 aetiology. The course is usually either chronic progressive (>8 weeks), relapsing and remitting, or stepwise. The clinical phenotype is typically characterised by symmetrical proximal and distal weakness, distal large-fibre sensory loss, and absent or reduced reflexes.