Chronic Inflammatory Demyelinating Polyradiculoneuropathy and Its Variants

Kelly Gwathmey, MD Peripheral Nerve and Motor Neuron Disorders p. 1205-1223 October 2020, Vol.26, No.5 doi: 10.1212/CON.0000000000000907
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PURPOSE OF REVIEW Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and its variants comprise a group of immune-mediated neuropathies with distinctive clinical presentations and electrodiagnostic features. Prompt recognition of these treatable disorders is mandatory as delays result in significant disability and morbidity. This article highlights the clinical presentation, pathophysiology, diagnostic evaluation, and treatment approach of these polyneuropathies.

RECENT FINDINGS The spectrum of CIDP is expanding with the recent characterization of neuropathies associated with nodal and paranodal antibodies. These neuropathies are distinguished by their unique presentations and are often refractory to IV immunoglobulin (IVIg) therapy. Subcutaneous immunoglobulins have recently been approved as a treatment option for CIDP and join corticosteroids, IVIg, and plasma exchange as first-line treatment.

SUMMARY CIDP is characterized by progressive symmetric proximal and distal weakness, large fiber sensory loss, and areflexia, with clinical nadir reached more than 8 weeks after symptom onset. Autoimmune demyelinating neuropathies fall on a continuum, with differences in the type of nerve fibers affected and pattern of deficits. Distinguishing between typical CIDP and its variants allows for selection of the most appropriate treatment.

Address correspondence to Dr Kelly Gwathmey, Department of Neurology, Virginia Commonwealth University, 1101 East Marshall St, PO Box 980599, Richmond, VA 23298, [email protected].

RELATIONSHIP DISCLOSURE: Dr Gwathmey has served as a consultant for and received personal compensation for speaking engagements from Alexion Pharmaceuticals, Inc.

UNLABELED USE OF PRODUCTS/INVESTIGATIONAL USE DISCLOSURE: Dr Gwathmey discusses the unlabeled/investigational use of azathioprine, bortezomib, corticosteroids (methylprednisolone, prednisone), cyclophosphamide, cyclosporine, methotrexate, mycophenolate mofetil, and rituximab for the treatment of chronic inflammatory demyelinating polyradiculoneuropathy and its variants.

© 2020 American Academy of Neurology.