Chronic Inflammatory Demyelinating Polyneuropathy (Cidp) is an important component in the neurobiology of neurodegenerative diseases. This page provides detailed information about its structure, function, and role in disease processes.
Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is an autoimmune disorder characterized by progressive weakness and impaired sensory function in the arms and legs.[1] It is the chronic counterpart of Guillain-Barré syndrome (GBS) and represents one of the most common treatable causes of peripheral neuropathy.[2]
CIDP is caused by an abnormal immune response that attacks the myelin sheath surrounding peripheral nerves, leading to demyelination and subsequent nerve dysfunction. Unlike GBS, which reaches peak severity within weeks, CIDP progresses over more than 8 weeks and may follow a relapsing-remitting or progressive course.[3]
The pathogenesis of CIDP involves cell-mediated and humoral immune responses targeting peripheral nerve components:[4]
Nerve biopsy reveals characteristic features including:[9]
The clinical presentation of CIDP typically includes:[10]
Motor Symptoms:
Sensory Symptoms:
Several variants of CIDP have been described:[11]
The EFNS/PNS criteria (2021) require:[12]
Clinical criteria:
Electrodiagnostic criteria (must meet 3 of 4):
Supportive criteria:
CIDP must be distinguished from:[13]
Corticosteroids:
Intravenous Immunoglobulin (IVIG):
Plasma Exchange (PLEX):
Approximately 50-80% of patients respond to first-line therapy.[17] Early treatment is associated with better outcomes. Some patients require long-term maintenance therapy.
The prognosis of CIDP varies significantly:[18]
Recent research focuses on:[19]
The study of Chronic Inflammatory Demyelinating Polyneuropathy (Cidp) has evolved significantly over the past decades. Research in this area has revealed important insights into the underlying mechanisms of neurodegeneration and continues to drive therapeutic development.
Historical context and key discoveries in this field have shaped our current understanding and will continue to guide future research directions.
[1] Van den Bergh PYK, Hadden RDM, Bouche P, et al. European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint task force—First revision. J Peripher Nerv Syst. 2010;15(1):1-9.
[2] Rajabally Y, Adams D, Latour P, Attarian S. Phenotypic and genetic spectrum of chronic inflammatory demyelinating polyneuropathy without remission. Neurology. 2020;94(14):e1488-e1495.
[3] Van den Bergh PYK, van Doorn PA, Hadden RDM, et al. European Academy of Neurology/Peripheral Nerve Society Guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy, with emphasis on practice in the context of the COVID-19 pandemic. J Peripher Nerv Syst. 2021;26(2):123-132.
[4] Kieseier BC, Mathey EK, Sommer C, Hartung HP. Immune-mediated neuropathies. Nat Rev Dis Primers. 2018;4(1):31.
[5] Kiefer R, Kieseier BC, Stoll G, Hartung HP. The role of macrophages in immune-mediated damage to the peripheral nervous system. Prog Neurobiol. 2001;64(2):109-127.
[6] Devaux JJ, Miura Y, Fukami Y, et al. Antibodies to neurofascin-155 in chronic inflammatory demyelinating polyneuropathy. Neurology. 2013;80(12):1164-1172.
[7] Monaco S, Bonetti B, Ferrari S, et al. Complement-mediated demyelination in patients with IgM monoclonal gammopathy and polyneuropathy. N Engl J Med. 1990;322(10):649-652.
[8] Dalakas MC, Engel WK. Immunoglobulin and complement deposits in nerves of patients with chronic relapsing polyneuropathy. Arch Neurol. 1980;37(10):637-640.
[9] Laughlin RS, Dyck PJ, Melton LJ, et al. Incidence and prevalence of CIDP and typical peripheral neuropathies. Neurology. 2009;72(6):63-67.
[10] Dyck PJ, Lofgren EP. Chronic inflammatory demyelinating polyradiculoneuropathy. In: Dyck PJ, Thomas PK, eds. Peripheral Neuropathy. 4th ed. Elsevier; 2005:2221-2235.
[11] Rajabally Y, Seror R. Chronic inflammatory demyelinating polyneuropathy variants. Neurology. 2019;93(7):298-304.
[12] Van den Bergh PYK, et al. EFNS/PNS revised diagnostic criteria for CIDP. J Peripher Nerv Syst. 2021.
[13] Latov N. Diagnosis of CIDP. Neurology. 2020;95(7):295-296.
[14] Eftimov F, Winer JB, Vermeulen M, de Haan R, van Schaik IN. Corticosteroids for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev. 2013;(8):CD002200.
[15] van Doorn PA, Ruts L, Jacobs BC. Treatment options for Guillain-Barré syndrome and chronic inflammatory demyelinating polyradiculoneuropathy. Pract Neurol. 2010;10(2):65-74.
[16] Mehndiratta MM, Hughes RAC. Plasma exchange for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev. 2002;(2):CD003906.
[17] Gorson KC, Allam G, Ropper AH. Chronic inflammatory demyelinating polyneuropathy: clinical features and response to treatment in 67 consecutive patients with and without a monoclonal gammopathy. Neurology. 1997;48(2):321-328.
[18] Kuwabara S, Misawa S, Mori M, et al. Long-term prognosis in patients with chronic inflammatory demyelinating polyneuropathy. Brain. 2022;145(3):1101-1112.
[19] Bunschoten C, Jacobs BC, Van den Bergh PYK, Cornblath DR, van Doorn PA. Progress in CIDP: From pathogenesis to treatment. Nat Rev Neurol. 2023;19(11):647-660.