Chronic inflammatory demyelinating polyneuropathy: Etiology, clinical features, and diagnosis
- Richard Lewis, MD
Richard Lewis, MD
- Cedars-Sinai Medical Center
Chronic inflammatory demyelinating polyneuropathy (CIDP, also known as chronic inflammatory demyelinating polyradiculoneuropathy) is an acquired disorder of peripheral nerves and nerve roots. There have been descriptions of patients with disorders that would now be considered CIDP for over 75 years, and the landmark report of Austin in 1958 described two patients with relapsing disease and glucocorticoid response .
The concept of CIDP as a distinct entity was introduced in 1975 when a review of the clinical, electrodiagnostic, and pathologic findings of 53 patients was presented . While that publication did not include "demyelinating" in the title, subsequent reports have included it, and demyelination is now recognized as a cardinal feature of the disorder.
The classification, etiology, clinical features, and diagnosis of CIDP will be reviewed here. Treatment and prognosis of this disorder are discussed separately. (See "Chronic inflammatory demyelinating polyneuropathy: Treatment and prognosis".)
Whether CIDP is a disease or a syndrome remains controversial. The following neuropathies all have chronicity, demyelination, inflammation, or immune-mediation in common:
- AUSTIN JH. Recurrent polyneuropathies and their corticosteroid treatment; with five-year observations of a placebo-controlled case treated with corticotrophin, cortisone, and prednisone. Brain 1958; 81:157.
- Dyck PJ, Lais AC, Ohta M, et al. Chronic inflammatory polyradiculoneuropathy. Mayo Clin Proc 1975; 50:621.
- Hughes R, Sanders E, Hall S, et al. Subacute idiopathic demyelinating polyradiculoneuropathy. Arch Neurol 1992; 49:612.
- Oh SJ, Kurokawa K, de Almeida DF, et al. Subacute inflammatory demyelinating polyneuropathy. Neurology 2003; 61:1507.
- Saperstein DS, Katz JS, Amato AA, Barohn RJ. Clinical spectrum of chronic acquired demyelinating polyneuropathies. Muscle Nerve 2001; 24:311.
- Dalakas MC, Medscape. Advances in the diagnosis, pathogenesis and treatment of CIDP. Nat Rev Neurol 2011; 7:507.
- Schneider-Hohendorf T, Schwab N, Uçeyler N, et al. CD8+ T-cell immunity in chronic inflammatory demyelinating polyradiculoneuropathy. Neurology 2012; 78:402.
- Peltier AC, Donofrio PD. Chronic inflammatory demyelinating polyradiculoneuropathy: from bench to bedside. Semin Neurol 2012; 32:187.
- Lunn MP, Manji H, Choudhary PP, et al. Chronic inflammatory demyelinating polyradiculoneuropathy: a prevalence study in south east England. J Neurol Neurosurg Psychiatry 1999; 66:677.
- McLeod JG, Pollard JD, Macaskill P, et al. Prevalence of chronic inflammatory demyelinating polyneuropathy in New South Wales, Australia. Ann Neurol 1999; 46:910.
- Chiò A, Cocito D, Bottacchi E, et al. Idiopathic chronic inflammatory demyelinating polyneuropathy: an epidemiological study in Italy. J Neurol Neurosurg Psychiatry 2007; 78:1349.
- Iijima M, Koike H, Hattori N, et al. Prevalence and incidence rates of chronic inflammatory demyelinating polyneuropathy in the Japanese population. J Neurol Neurosurg Psychiatry 2008; 79:1040.
- Rajabally YA, Simpson BS, Beri S, et al. Epidemiologic variability of chronic inflammatory demyelinating polyneuropathy with different diagnostic criteria: study of a UK population. Muscle Nerve 2009; 39:432.
- Laughlin RS, Dyck PJ, Melton LJ 3rd, et al. Incidence and prevalence of CIDP and the association of diabetes mellitus. Neurology 2009; 73:39.
- Lozeron P, Denier C, Lacroix C, Adams D. Long-term course of demyelinating neuropathies occurring during tumor necrosis factor-alpha-blocker therapy. Arch Neurol 2009; 66:490.
- McCombe PA, Pollard JD, McLeod JG. Chronic inflammatory demyelinating polyradiculoneuropathy. A clinical and electrophysiological study of 92 cases. Brain 1987; 110 ( Pt 6):1617.
- Figueroa JJ, Dyck PJ, Laughlin RS, et al. Autonomic dysfunction in chronic inflammatory demyelinating polyradiculoneuropathy. Neurology 2012; 78:702.
- Lewis RA, Sumner AJ, Brown MJ, Asbury AK. Multifocal demyelinating neuropathy with persistent conduction block. Neurology 1982; 32:958.
- Rajabally YA, Chavada G. Lewis-sumner syndrome of pure upper-limb onset: diagnostic, prognostic, and therapeutic features. Muscle Nerve 2009; 39:206.
- Sinnreich M, Klein CJ, Daube JR, et al. Chronic immune sensory polyradiculopathy: a possibly treatable sensory ataxia. Neurology 2004; 63:1662.
- Trip SA, Saifee T, Honan W, et al. Chronic immune sensory polyradiculopathy with cranial and peripheral nerve involvement. J Neurol 2012; 259:1238.
- Oh SJ, Joy JL, Kuruoglu R. "Chronic sensory demyelinating neuropathy": chronic inflammatory demyelinating polyneuropathy presenting as a pure sensory neuropathy. J Neurol Neurosurg Psychiatry 1992; 55:677.
- Katz JS, Saperstein DS, Gronseth G, et al. Distal acquired demyelinating symmetric neuropathy. Neurology 2000; 54:615.
- Feasby TE, Hahn AF, Koopman WJ, Lee DH. Central lesions in chronic inflammatory demyelinating polyneuropathy: an MRI study. Neurology 1990; 40:476.
- Harbo T, Andersen H, Jakobsen J. Length-dependent weakness and electrophysiological signs of secondary axonal loss in chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve 2008; 38:1036.
- Prineas JW. Demyelination and remyelination in recurrent idiopathic polyneuropathy. An electron microscope study. Acta Neuropathol 1971; 18:34.
- Hughes RA, Bouche P, Cornblath DR, et al. European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society. Eur J Neurol 2006; 13:326.
- Barohn RJ, Kissel JT, Warmolts JR, Mendell JR. Chronic inflammatory demyelinating polyradiculoneuropathy. Clinical characteristics, course, and recommendations for diagnostic criteria. Arch Neurol 1989; 46:878.
- Research criteria for diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). Report from an Ad Hoc Subcommittee of the American Academy of Neurology AIDS Task Force. Neurology 1991; 41:617.
- Hughes R, Bensa S, Willison H, et al. Randomized controlled trial of intravenous immunoglobulin versus oral prednisolone in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol 2001; 50:195.
- Thaisetthawatkul P, Logigian EL, Herrmann DN. Dispersion of the distal compound muscle action potential as a diagnostic criterion for chronic inflammatory demyelinating polyneuropathy. Neurology 2002; 59:1526.
- Koski CL, Baumgarten M, Magder LS, et al. Derivation and validation of diagnostic criteria for chronic inflammatory demyelinating polyneuropathy. J Neurol Sci 2009; 277:1.
- Joint Task Force of the EFNS and the PNS. European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society--First Revision. J Peripher Nerv Syst 2010; 15:1.
- Rajabally YA, Nicolas G, Piéret F, et al. Validity of diagnostic criteria for chronic inflammatory demyelinating polyneuropathy: a multicentre European study. J Neurol Neurosurg Psychiatry 2009; 80:1364.
- Tackenberg B, Lünemann JD, Steinbrecher A, et al. Classifications and treatment responses in chronic immune-mediated demyelinating polyneuropathy. Neurology 2007; 68:1622.
- Van den Bergh PY, Piéret F. Electrodiagnostic criteria for acute and chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve 2004; 29:565.
- Rajabally YA, Jacob S, Hbahbih M. Optimizing the use of electrophysiology in the diagnosis of chronic inflammatory demyelinating polyneuropathy: a study of 20 cases. J Peripher Nerv Syst 2005; 10:282.
- Molenaar DS, Vermeulen M, de Haan R. Diagnostic value of sural nerve biopsy in chronic inflammatory demyelinating polyneuropathy. J Neurol Neurosurg Psychiatry 1998; 64:84.
- Bosboom WM, van den Berg LH, Franssen H, et al. Diagnostic value of sural nerve demyelination in chronic inflammatory demyelinating polyneuropathy. Brain 2001; 124:2427.
- Vallat JM, Tabaraud F, Magy L, et al. Diagnostic value of nerve biopsy for atypical chronic inflammatory demyelinating polyneuropathy: evaluation of eight cases. Muscle Nerve 2003; 27:478.
- Mathis S, Magy L, Diallo L, et al. Amyloid neuropathy mimicking chronic inflammatory demyelinating polyneuropathy. Muscle Nerve 2012; 45:26.
- Temporal continuum
- CLINICAL MANIFESTATIONS
- Variants of CIDP
- - Lewis-Sumner syndrome
- - Sensory-predominant CIDP
- - Distal acquired demyelinating sensory neuropathy
- - CIDP with CNS involvement
- Differential diagnosis
- Diagnostic criteria
- - Koski criteria
- - EFNS/PNS criteria
- Diagnostic evaluation
- - Electrodiagnostic testing
- - Cerebrospinal fluid analysis
- - Nerve biopsy
- - MRI
- - Laboratory studies
- - Genetic considerations
- SUMMARY AND RECOMMENDATIONS