Focal lesions area feature of chronic inflammatory demyelinating polyneuropathy (CIDP)

Acta Neuropathol. 1998 Dec;96(6):603-9. doi: 10.1007/s004010050941.


In a study designed to identify the neuropathological features typical of chronic inflammatory demyelinating polyneuropathy (CIDP), we reviewed the sural nerve biopsy findings in 105 patients with this disorder. The patients' mean age at biopsy was 49 years. In 65% of patients the disease had a progressive and in 35% a relapsing-remitting course. In 47% of cases the disorder was idiopathic; the remainder had various concurrent conditions. All sural nerve biopsy specimens showed varying amounts of active demyelination associated with onion bulbs (48% of cases), endoneurial edema (55%) and inflammatory infiltrates (25%). The immunopathological hallmarks were T cell infiltration with macrophagic activation and up-regulation of major histocompatibility complex (MHC) class II expression, without B cell infiltration or immunoglobulin deposition on myelin sheaths. In 30% of cases some myelin sheaths showed C3d deposition. Analysis of proinflammatory cytokine expression invariably showed interleukin-1 in perivascular and endoneurial ramified cells and tumor necrosis factor-alpha prevalently in epineurial macrophages, whereas it detected interferon-gamma only in samples with perivascular inflammatory cells. This immunological pattern suggests that the cellular components of immunity play the major role in CIDP. In 19% of cases the neuropathological changes had a focal distribution. This distinctive feature corresponded to more active demyelination, more frequent detection of inflammatory infiltrates and more prominent immunological activation, suggesting that focal involvement is a possible step in the course of the disease.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Biopsy
  • Cerebrospinal Fluid Proteins / analysis
  • Child
  • Child, Preschool
  • Chronic Disease
  • Demyelinating Diseases / cerebrospinal fluid
  • Demyelinating Diseases / pathology*
  • Demyelinating Diseases / physiopathology
  • Female
  • Humans
  • Male
  • Microscopy, Electron
  • Middle Aged
  • Neural Conduction / physiology
  • Neuritis / cerebrospinal fluid
  • Neuritis / pathology*
  • Neuritis / physiopathology
  • Peripheral Nervous System Diseases / cerebrospinal fluid
  • Peripheral Nervous System Diseases / pathology*
  • Peripheral Nervous System Diseases / physiopathology
  • Reaction Time / physiology
  • Sural Nerve / pathology
  • Sural Nerve / physiopathology


  • Cerebrospinal Fluid Proteins