chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)
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Introduction
A group of chronic progressive or relapsing inflammatory demyelinating peripheral neuropathies.
Classification
Two major forms of CIDP:
- monoclonal IgM that reacts with myelin-associated glycoprotein (MAG)
- occurs in 1/2 of patients with IgM gammopathy & neuropathy
- more sensory than motor
- slowly progressive
- osteosclerotic myeloma
Etiology
- autoimume inflammatory disorder
- rarely associated with solid tumors
- rarely associated with Waldenstrom's macroglobulinemia, heavy chain disease, or lymphoma
- may occur in association with diabetes mellitus, paraproteinemia or HIV1 infection[2]
Pathology
Clinical manifestations
- insidious, progressive symmetric neuropathy evolving over months
- symptoms stabilize (but may persist) after 3 months
- mixed sensory & motor neuropathy
- symmetric distal & proximal muscle weakness, chronic progressive or relapsing[2]
- symmetric sensory loss, distal sensory paresthesias
- diffuse areflexia
Laboratory
- lumbar puncture with CSF analysis:
- elevation of cerebrospinal fluid protein
- no CSF pleocytosis
- serum protein electrophoresis & CSF protein electrophoresis (high resolution)
- rule out multiple sclerosis
- identify monoclonal gammopathy
- bone marrow biopsy
Diagnostic procedures
- nerve conduction study
- slowed nerve conduction velocities
- including motor nerves
- slowed nerve conduction velocities
- electromyography:
- dispersion of compound muscle action potential
- 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
- 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
- insidious rather than acute time course
- distinguished from Guillain Barre syndrome by
- POEMS syndrome
- lambda monoclonal gammopathy, hepatosplenomegaly & skin changes suggest POEMS syndrome[2]
- multiple sclerosis
- diabetic amyotrophy
Management
- long-term therapy
- glucocorticoids
- plasmapheresis
- IV immunoglobulin
- immunosuppressive therapy
- treat associated malignancy
More general terms
Additional terms
- Guillain-Barre syndrome (GBS)
- multiple sclerosis (MS)
- POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, & Skin changes); Crow-Fukase syndrome
References
- ↑ Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 625-26, 2464
- ↑ 2.0 2.1 2.2 2.3 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
- ↑ Brannagan TH 3rd. Current treatments of chronic immune-mediated demyelinating polyneuropathies. Muscle Nerve. 2009 May;39(5):563-78. PMID: https://www.ncbi.nlm.nih.gov/pubmed/19301378
- ↑ Peltier AC, Donofrio PD. Chronic inflammatory demyelinating polyradiculoneuropathy: from bench to bedside. Semin Neurol. 2012 Jul;32(3):187-95 PMID: https://www.ncbi.nlm.nih.gov/pubmed/23117943
- ↑ 5.0 5.1 Herraets IJT et al. Nerve ultrasound for diagnosing chronic inflammatory neuropathy: A multicenter validation study. Neurology 2020 Jul 16; [e-pub] PMID: https://www.ncbi.nlm.nih.gov/pubmed/32675082 https://n.neurology.org/content/early/2020/07/16/WNL.0000000000010369
- ↑ NINDS Chronic Inflammatory Demyelinating Polyneuropathyv (CIDP) Information Page https://www.ninds.nih.gov/Disorders/All-Disorders/Chronic-Inflammatory-Demyelinating-Polyneuropathy-CIDP-Information-Page
Patient information
chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patient information