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chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)

A group of chronic progressive or relapsing inflammatory demyelinating peripheral neuropathies. Classification: Two major forms of CIDP: 1) monoclonal IgM that reacts with myelin-associated glycoprotein (MAG) a) occurs in 1/2 of patients with IgM gammopathy & neuropathy b) more sensory than motor c) slowly progressive 2) osteosclerotic myeloma a) monoclonal IgA or IgG that do not react with MAG b) predominantly motor c) often indolent in nature d) may eventually produce severe wasting e) sensory & autonomic phenomena are uncommon Etiology: 1) autoimume inflammatory disorder 2) rarely associated with solid tumors a) breast cancer b) lung cancer c) colon cancer 3) rarely associated with Waldenstrom's macroglobulinemia, heavy chain disease, or lymphoma 4) may occur in association with diabetes mellitus, paraproteinemia or HIV1 infection [2] Pathology: - segmental loss of myelin with preservation of axons Clinical manifestations: 1) insidious, progressive symmetric neuropathy evolving over months 2) symptoms stabilize (but may persist) after 3 months 3) mixed sensory & motor neuropathy a) symmetric distal & proximal muscle weakness, chronic progressive or relapsing [2] b) symmetric sensory loss, distal sensory paresthesias 4) diffuse areflexia Laboratory: 1) lumbar puncture with CSF analysis: a) elevation of cerebrospinal fluid protein b) no CSF pleocytosis 2) serum protein electrophoresis & CSF protein electrophoresis (high resolution) a) rule out multiple sclerosis b) identify monoclonal gammopathy 3) bone marrow biopsy Special laboratory: 1) nerve conduction study - slowed nerve conduction velocities - including motor nerves 2) electromyography: - dispersion of compound muscle action potential 3) sural nerve biopsy may be normal if demyelination is proximal Radiology: - nerve ultrasound is complementary to nerve conduction studies in improving sensitivity for diagnosis of CIDP [5] Differential diagnosis: 1) Guillain Barre syndrome - distinguished from Guillain Barre syndrome by - insidious rather than acute time course - Guillain Barre syndrome reaches a nadir of symptoms within 4 weeks - more prominent involvement of sensory nerves - lack of involvement of autonomic nerves - responsiveness to immunotherapy 2) POEMS syndrome - lambda monoclonal gammopathy, hepatosplenomegaly & skin changes suggest POEMS syndrome [2] 3) multiple sclerosis 4) diabetic amyotrophy Management: 1) long-term therapy 2) glucocorticoids 3) plasmapheresis 4) IV immunoglobulin 5) immunosuppressive therapy a) methotrexate b) cytoxan c) azathiaprine d) cyclosporin 6) efgartigimod alfa (Vyvgart) in combination with hyaluronidase-qvfc (Vyvgart Hytrulo) reduces risk of CIDP relapse [6] 7) treat associated malignancy

Related

Guillain-Barre syndrome (GBS) multiple sclerosis (MS); includes clinically isolated syndrome POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, & Skin changes); Crow-Fukase syndrome

Specific

distal acquired demyelinating symmetric neuropathy (DADS)

General

inflammatory demyelinating polyneuropathy chronic neurologic disease

References

  1. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 625-26, 2464
  2. Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2006, 2009, 2012, 2015, 2018, 2022
  3. Brannagan TH 3rd. Current treatments of chronic immune-mediated demyelinating polyneuropathies. Muscle Nerve. 2009 May;39(5):563-78. PMID: 19301378
  4. Peltier AC, Donofrio PD. Chronic inflammatory demyelinating polyradiculoneuropathy: from bench to bedside. Semin Neurol. 2012 Jul;32(3):187-95 PMID: 23117943
  5. Herraets IJT et al. Nerve ultrasound for diagnosing chronic inflammatory neuropathy: A multicenter validation study. Neurology 2020 Jul 16; [e-pub] PMID: 32675082 https://n.neurology.org/content/early/2020/07/16/WNL.0000000000010369
  6. George J New Treatment Approved for Chronic Inflammatory Demyelinating Polyneuropathy. Efgartigimod injection reduced CIDP relapse risk MedPage Today June 23, 2024 https://www.medpagetoday.com/neurology/generalneurology/110782
  7. NINDS Chronic Inflammatory Demyelinating Polyneuropathyv (CIDP) Information Page https://www.ninds.nih.gov/Disorders/All-Disorders/Chronic-Inflammatory-Demyelinating-Polyneuropathy-CIDP-Information-Page