Chronic inflammatory demyelinating polyradiculoneuropathy

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.