The spectrum of chronic inflammatory demyelinating polyneuropathy

J Neurol Sci. 2000 Feb 15;173(2):129-39. doi: 10.1016/s0022-510x(99)00317-2.


Research criteria for the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) were proposed by an Ad Hoc Subcommittee of the American Academy of Neurology (AAN) in 1991, and since then these criteria have been widely used in clinical studies. We have been impressed by the frequent finding of electrophysiological changes of a demyelinating neuropathy in patients whose clinical presentation does not conform to the usually accepted clinical phenotype of CIDP. To determine the clinical spectrum of CIDP, we conducted a retrospective review of patients of the peripheral electrophysiology laboratory of the University of Miami-Jackson Memorial Medical Center. Diagnostic criteria for acquired demyelination of an individual nerve were adapted from the AAN research criteria for the diagnosis of CIDP (1991). Patients were accepted for inclusion when such evidence was demonstrated in at least one motor nerve or at least two sensory nerves. We then reviewed the clinical phenotype and the underlying etiology of the neuropathy in these cases. Eighty-seven patients, 63 male and 24 female, age of onset 4-84 (mean 49.3) years, met these inclusion criteria. Forty-seven patients (54%) had distinct features outside the usual clinical presentation of CIDP. Of these, 15 (17%) had predominantly distal features, 13 (15%) had exclusively sensory polyneuropathy; seven (8%) had markedly asymmetric disease, seven (8%) had associated CNS demyelination, four (5%) had predominant cranial nerve involvement, and one (1%) had only the restless legs syndrome. An associated medical condition that may have been responsible for the acquired demyelinating neuropathy was present in 60% of the patients. We conclude that spectrum of CIDP is broader than would be indicated by the strict application of the AAN research criteria, and that many of the cases meeting more liberal criteria frequently respond to immunosuppressive therapy.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adult
  • Age of Onset
  • Aged
  • Aged, 80 and over
  • Autoimmune Diseases / diagnosis
  • Autoimmune Diseases / drug therapy
  • Autoimmune Diseases / epidemiology
  • Autoimmune Diseases / metabolism
  • Autoimmune Diseases / physiopathology*
  • Biopsy
  • Blood Protein Electrophoresis
  • Cerebrospinal Fluid Proteins / analysis
  • Child
  • Child, Preschool
  • Comorbidity
  • Cranial Nerves / physiopathology
  • Diabetic Neuropathies / complications
  • Electrophysiology
  • Female
  • Florida / epidemiology
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Male
  • Middle Aged
  • Paraproteinemias / complications
  • Peripheral Nerves / pathology
  • Peripheral Nerves / physiopathology
  • Phenotype
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / diagnosis
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / drug therapy
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / epidemiology
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / metabolism
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / physiopathology*
  • Retrospective Studies
  • Spinal Cord / physiopathology
  • Treatment Failure


  • Cerebrospinal Fluid Proteins
  • Immunosuppressive Agents