Chronic inflammatory demyelinating polyneuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an umbrella term that now covers a heterogenous spectrum of clinical presentations of chronic immune determined neuropathies. This includes the prototypical phenotype of a chronic progressive sensorimotor neuropathy with symmetric proximal and distal weakness and areflexia, variants with only focal symptoms, and the exclusive involvement of motor or sensory fibers. The main diagnostic test for CIDP is electrophysiology, based on motor conduction defects indicating patchy demyelination. Other tests that may be helpful are nerve MRI and ultrasound, cerebrospinal fluid testing, and very rarely, in selected cases, nerve biopsy. Current treatment is based on three main first-line therapies: corticosteroids, immunoglobulins, and plasma exchange. It remains important to discern CIDP from relevant mimics that demand different treatment strategies. Important new developments include the discovery of nodal/paranodal pathology and specific antibodies, increased awareness for careful decision making at onset, clinical evaluation using outcome measures, and treatment continuation.