Chronic Inflammatory Demyelinating Polyradiculoneuropathy

First described in 1890, chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated disorder affecting the peripheral nervous system and nerve roots. Characterized by symmetric weakness in both proximal and distal muscles, CIDP is a subset of chronic acquired demyelinating polyneuropathies (CADP).

CIDP can manifest in various clinical forms, with "typical" or "classical" presentations involving a progressive motor-predominant peripheral neuropathy and sensory impairment, particularly affecting position and vibration sense more than pain and temperature. Notably, CIDP is closely linked to acute inflammatory demyelinating polyradiculoneuropathy (AIDP), the demyelinating variant of Guillain-Barré syndrome (GBS).

Distinguishing between these 2 diseases is aided by assessing the progression timeline and the presence of relapses. Proper diagnosis is crucial for initiating appropriate treatment and managing the condition effectively.

CIDP may have atypical variants with different immunopathogeneses and treatment responses. The condition can be monophasic, relapsing, or progressive, and symptoms must persist for at least 8 weeks to establish the diagnosis. Progressive or relapsing symptoms over this duration, electrodiagnostic studies revealing pathologic evidence of peripheral nerve demyelination, and the responsiveness to immunomodulatory treatments helps establish the diagnosis of CIDP.