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CIDP
Jeffrey S. Ross, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Chronic inflammatory demyelinating polyneuropathy (CIDP)
            • Synonyms

              • Chronic inflammatory demyelinating polyradiculoneuropathy
              • Chronic autoimmune inflammatory polyneuropathy
            • Definitions

              • Chronic acquired, immune-mediated demyelinating neuropathy characterized by relapsing or progressive muscle weakness ± sensory loss

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Consider

                                        • Image Interpretation Pearls

                                          Selected References

                                          1. Kuwabara S et al: Chronic inflammatory demyelinating polyneuropathy. Adv Exp Med Biol. 1190:333-43, 2019
                                          2. Allen JA: Chronic demyelinating polyneuropathies. continuum (Minneap Minn). 23(5, Peripheral Nerve and Motor Neuron Disorders):1310-31, 2017
                                          3. Wong AH et al: Autoimmune inflammatory neuropathies: updates in pathogenesis, diagnosis, and treatment. Curr Opin Neurol. 28(5):468-73, 2015
                                          4. Said G et al: Chronic inflammatory demyelinative polyneuropathy. Handb Clin Neurol. 115:403-13, 2013
                                          5. Mahdi-Rogers M et al: Overview of the pathogenesis and treatment of chronic inflammatory demyelinating polyneuropathy with intravenous immunoglobulins. Biologics. 4:45-9, 2010
                                          6. Tracy JA et al: Investigations and treatment of chronic inflammatory demyelinating polyradiculoneuropathy and other inflammatory demyelinating polyneuropathies. Curr Opin Neurol. 23(3):242-8, 2010
                                          7. Vallat JM et al: Chronic inflammatory demyelinating polyradiculoneuropathy: diagnostic and therapeutic challenges for a treatable condition. Lancet Neurol. 9(4):402-12, 2010
                                          8. Van den Bergh PY 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 - first revision. Eur J Neurol. 17(3):356-63, 2010
                                          9. Brannagan TH 3rd: Current treatments of chronic immune-mediated demyelinating polyneuropathies. Muscle Nerve. 39(5):563-78, 2009
                                          10. Laughlin RS et al: Incidence and prevalence of CIDP and the association of diabetes mellitus. Neurology. 73(1):39-45, 2009
                                          11. Tazawa K et al: Spinal nerve root hypertrophy on MRI: clinical significance in the diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy. Intern Med. 47(23):2019-24, 2008
                                          12. Tsuchiya K et al: Demonstration of spinal cord and nerve root abnormalities by diffusion neurography. J Comput Assist Tomogr. 32(2):286-90, 2008
                                          13. Said G: Chronic inflammatory demyelinating polyneuropathy. Neuromuscul Disord. 16(5):293-303, 2006
                                          14. Köller H et al: Chronic inflammatory demyelinating polyneuropathy--update on pathogenesis, diagnostic criteria and therapy. Curr Opin Neurol. 18(3):273-8, 2005
                                          15. Matsuoka N et al: Detection of cervical nerve root hypertrophy by ultrasonography in chronic inflammatory demyelinating polyradiculoneuropathy. J Neurol Sci. 219(1-2):15-21, 2004
                                          16. Cocito D et al: Different clinical, electrophysiological and immunological features of CIDP associated with paraproteinaemia. Acta Neurol Scand. 108(4):274-80, 2003
                                          17. Fee DB et al: Resolution of chronic inflammatory demyelinating polyneuropathy-associated central nervous system lesions after treatment with intravenous immunoglobulin. J Peripher Nerv Syst. 8(3):155-8, 2003
                                          18. Haq RU et al: Chronic inflammatory demyelinating polyradiculoneuropathy in diabetic patients. Muscle Nerve. 27(4):465-70, 2003
                                          19. Magda P et al: Comparison of electrodiagnostic abnormalities and criteria in a cohort of patients with chronic inflammatory demyelinating polyneuropathy. Arch Neurol. 60(12):1755-9, 2003
                                          20. Odaka M et al: Patients with chronic inflammatory demyelinating polyneuropathy initially diagnosed as Guillain-Barre syndrome. J Neurol. 250(8):913-6, 2003
                                          21. Oguz B et al: Diffuse spinal and intercostal nerve involvement in chronic inflammatory demyelinating polyradiculoneuropathy: MRI findings. Eur Radiol. 13 Suppl 4:L230-4, 2003
                                          22. Press R et al: Aberrated levels of cerebrospinal fluid chemokines in Guillain-Barre syndrome and chronic inflammatory demyelinating polyradiculoneuropathy. J Clin Immunol. 23(4):259-67, 2003
                                          23. Rodriguez-Casero MV et al: Childhood chronic inflammatory demyelinating polyneuropathy with central nervous system demyelination resembling multiple sclerosis. Neuromuscul Disord. 13(2):158-61, 2003
                                          24. Ropper AH: Current treatments for CIDP. Neurology. 60(8 Suppl 3):S16-22, 2003
                                          25. Saperstein DS et al: Current concepts and controversy in chronic inflammatory demyelinating polyneuropathy. Curr Neurol Neurosci Rep. 3(1):57-63, 2003
                                          26. Toyka KV et al: The pathogenesis of CIDP: rationale for treatment with immunomodulatory agents. Neurology. 60(8 Suppl 3):S2-7, 2003
                                          27. Costello F et al: Childhood-onset chronic inflammatory demyelinating polyradiculoneuropathy with cranial nerve involvement. J Child Neurol. 17(11):819-23, 2002
                                          28. Cros D: Peripheral Neuropathy. 1st ed. Philadelphia: Lippincott Williams & Wilkins: 432, 2001
                                          29. Sabatelli M et al: Pure motor chronic inflammatory demyelinating polyneuropathy. J Neurol. 248(9):772-7, 2001
                                          30. Saperstein DS et al: Clinical spectrum of chronic acquired demyelinating polyneuropathies. Muscle Nerve. 24(3):311-24, 2001
                                          31. Taniguchi N et al: Sonographic detection of diffuse peripheral nerve hypertrophy in chronic inflammatory demyelinating polyradiculoneuropathy. J Clin Ultrasound. 28(9):488-91, 2000
                                          32. Van den Bergh PY et al: Chronic demyelinating hypertrophic brachial plexus neuropathy. Muscle Nerve. 23(2):283-8, 2000
                                          33. Duarte J et al: Hypertrophy of multiple cranial nerves and spinal roots in chronic inflammatory demyelinating neuropathy. J Neurol Neurosurg Psychiatry. 67(5):685-7, 1999
                                          34. Midroni G et al: MRI of the cauda equina in CIDP: clinical correlations. J Neurol Sci. 170(1):36-44, 1999
                                          35. Mizuno K et al: Chronic inflammatory demyelinating polyradiculoneuropathy with diffuse and massive peripheral nerve hypertrophy: distinctive clinical and magnetic resonance imaging features. Muscle Nerve. 21(6):805-8, 1998
                                          36. Kuwabara S et al: Magnetic resonance imaging at the demyelinative foci in chronic inflammatory demyelinating polyneuropathy. Neurology. 48(4):874-7, 1997
                                          37. Simmons Z et al: Chronic inflammatory demyelinating polyradiculoneuropathy in children: I. Presentation, electrodiagnostic studies, and initial clinical course, with comparison to adults. 20(12):1569-75, 1997
                                          38. Van Es HW et al: Magnetic resonance imaging of the brachial plexus in patients with multifocal motor neuropathy. Neurology. 48(5):1218-24, 1997
                                          Related Anatomy
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                                          Related Differential Diagnoses
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                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Top Differential Diagnoses

                                                • Pathology

                                                  • Clinical Issues

                                                    TERMINOLOGY

                                                    • Abbreviations

                                                      • Chronic inflammatory demyelinating polyneuropathy (CIDP)
                                                    • Synonyms

                                                      • Chronic inflammatory demyelinating polyradiculoneuropathy
                                                      • Chronic autoimmune inflammatory polyneuropathy
                                                    • Definitions

                                                      • Chronic acquired, immune-mediated demyelinating neuropathy characterized by relapsing or progressive muscle weakness ± sensory loss

                                                    IMAGING

                                                    • General Features

                                                      • CT Findings

                                                        • MR Findings

                                                          • Ultrasonographic Findings

                                                            • Imaging Recommendations

                                                              DIFFERENTIAL DIAGNOSIS

                                                                PATHOLOGY

                                                                • General Features

                                                                  • Gross Pathologic & Surgical Features

                                                                    • Microscopic Features

                                                                      CLINICAL ISSUES

                                                                      • Presentation

                                                                        • Demographics

                                                                          • Natural History & Prognosis

                                                                            • Treatment

                                                                              DIAGNOSTIC CHECKLIST

                                                                              • Consider

                                                                                • Image Interpretation Pearls

                                                                                  Selected References

                                                                                  1. Kuwabara S et al: Chronic inflammatory demyelinating polyneuropathy. Adv Exp Med Biol. 1190:333-43, 2019
                                                                                  2. Allen JA: Chronic demyelinating polyneuropathies. continuum (Minneap Minn). 23(5, Peripheral Nerve and Motor Neuron Disorders):1310-31, 2017
                                                                                  3. Wong AH et al: Autoimmune inflammatory neuropathies: updates in pathogenesis, diagnosis, and treatment. Curr Opin Neurol. 28(5):468-73, 2015
                                                                                  4. Said G et al: Chronic inflammatory demyelinative polyneuropathy. Handb Clin Neurol. 115:403-13, 2013
                                                                                  5. Mahdi-Rogers M et al: Overview of the pathogenesis and treatment of chronic inflammatory demyelinating polyneuropathy with intravenous immunoglobulins. Biologics. 4:45-9, 2010
                                                                                  6. Tracy JA et al: Investigations and treatment of chronic inflammatory demyelinating polyradiculoneuropathy and other inflammatory demyelinating polyneuropathies. Curr Opin Neurol. 23(3):242-8, 2010
                                                                                  7. Vallat JM et al: Chronic inflammatory demyelinating polyradiculoneuropathy: diagnostic and therapeutic challenges for a treatable condition. Lancet Neurol. 9(4):402-12, 2010
                                                                                  8. Van den Bergh PY 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 - first revision. Eur J Neurol. 17(3):356-63, 2010
                                                                                  9. Brannagan TH 3rd: Current treatments of chronic immune-mediated demyelinating polyneuropathies. Muscle Nerve. 39(5):563-78, 2009
                                                                                  10. Laughlin RS et al: Incidence and prevalence of CIDP and the association of diabetes mellitus. Neurology. 73(1):39-45, 2009
                                                                                  11. Tazawa K et al: Spinal nerve root hypertrophy on MRI: clinical significance in the diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy. Intern Med. 47(23):2019-24, 2008
                                                                                  12. Tsuchiya K et al: Demonstration of spinal cord and nerve root abnormalities by diffusion neurography. J Comput Assist Tomogr. 32(2):286-90, 2008
                                                                                  13. Said G: Chronic inflammatory demyelinating polyneuropathy. Neuromuscul Disord. 16(5):293-303, 2006
                                                                                  14. Köller H et al: Chronic inflammatory demyelinating polyneuropathy--update on pathogenesis, diagnostic criteria and therapy. Curr Opin Neurol. 18(3):273-8, 2005
                                                                                  15. Matsuoka N et al: Detection of cervical nerve root hypertrophy by ultrasonography in chronic inflammatory demyelinating polyradiculoneuropathy. J Neurol Sci. 219(1-2):15-21, 2004
                                                                                  16. Cocito D et al: Different clinical, electrophysiological and immunological features of CIDP associated with paraproteinaemia. Acta Neurol Scand. 108(4):274-80, 2003
                                                                                  17. Fee DB et al: Resolution of chronic inflammatory demyelinating polyneuropathy-associated central nervous system lesions after treatment with intravenous immunoglobulin. J Peripher Nerv Syst. 8(3):155-8, 2003
                                                                                  18. Haq RU et al: Chronic inflammatory demyelinating polyradiculoneuropathy in diabetic patients. Muscle Nerve. 27(4):465-70, 2003
                                                                                  19. Magda P et al: Comparison of electrodiagnostic abnormalities and criteria in a cohort of patients with chronic inflammatory demyelinating polyneuropathy. Arch Neurol. 60(12):1755-9, 2003
                                                                                  20. Odaka M et al: Patients with chronic inflammatory demyelinating polyneuropathy initially diagnosed as Guillain-Barre syndrome. J Neurol. 250(8):913-6, 2003
                                                                                  21. Oguz B et al: Diffuse spinal and intercostal nerve involvement in chronic inflammatory demyelinating polyradiculoneuropathy: MRI findings. Eur Radiol. 13 Suppl 4:L230-4, 2003
                                                                                  22. Press R et al: Aberrated levels of cerebrospinal fluid chemokines in Guillain-Barre syndrome and chronic inflammatory demyelinating polyradiculoneuropathy. J Clin Immunol. 23(4):259-67, 2003
                                                                                  23. Rodriguez-Casero MV et al: Childhood chronic inflammatory demyelinating polyneuropathy with central nervous system demyelination resembling multiple sclerosis. Neuromuscul Disord. 13(2):158-61, 2003
                                                                                  24. Ropper AH: Current treatments for CIDP. Neurology. 60(8 Suppl 3):S16-22, 2003
                                                                                  25. Saperstein DS et al: Current concepts and controversy in chronic inflammatory demyelinating polyneuropathy. Curr Neurol Neurosci Rep. 3(1):57-63, 2003
                                                                                  26. Toyka KV et al: The pathogenesis of CIDP: rationale for treatment with immunomodulatory agents. Neurology. 60(8 Suppl 3):S2-7, 2003
                                                                                  27. Costello F et al: Childhood-onset chronic inflammatory demyelinating polyradiculoneuropathy with cranial nerve involvement. J Child Neurol. 17(11):819-23, 2002
                                                                                  28. Cros D: Peripheral Neuropathy. 1st ed. Philadelphia: Lippincott Williams & Wilkins: 432, 2001
                                                                                  29. Sabatelli M et al: Pure motor chronic inflammatory demyelinating polyneuropathy. J Neurol. 248(9):772-7, 2001
                                                                                  30. Saperstein DS et al: Clinical spectrum of chronic acquired demyelinating polyneuropathies. Muscle Nerve. 24(3):311-24, 2001
                                                                                  31. Taniguchi N et al: Sonographic detection of diffuse peripheral nerve hypertrophy in chronic inflammatory demyelinating polyradiculoneuropathy. J Clin Ultrasound. 28(9):488-91, 2000
                                                                                  32. Van den Bergh PY et al: Chronic demyelinating hypertrophic brachial plexus neuropathy. Muscle Nerve. 23(2):283-8, 2000
                                                                                  33. Duarte J et al: Hypertrophy of multiple cranial nerves and spinal roots in chronic inflammatory demyelinating neuropathy. J Neurol Neurosurg Psychiatry. 67(5):685-7, 1999
                                                                                  34. Midroni G et al: MRI of the cauda equina in CIDP: clinical correlations. J Neurol Sci. 170(1):36-44, 1999
                                                                                  35. Mizuno K et al: Chronic inflammatory demyelinating polyradiculoneuropathy with diffuse and massive peripheral nerve hypertrophy: distinctive clinical and magnetic resonance imaging features. Muscle Nerve. 21(6):805-8, 1998
                                                                                  36. Kuwabara S et al: Magnetic resonance imaging at the demyelinative foci in chronic inflammatory demyelinating polyneuropathy. Neurology. 48(4):874-7, 1997
                                                                                  37. Simmons Z et al: Chronic inflammatory demyelinating polyradiculoneuropathy in children: I. Presentation, electrodiagnostic studies, and initial clinical course, with comparison to adults. 20(12):1569-75, 1997
                                                                                  38. Van Es HW et al: Magnetic resonance imaging of the brachial plexus in patients with multifocal motor neuropathy. Neurology. 48(5):1218-24, 1997