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

N. Rizzuto, M. Morbin, T. Cavallaro, S. Ferrari, M. Fallahi, S. Galiazzo Rizzuto

Research output: Contribution to journalArticle

31 Citations (Scopus)

Abstract

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-α prevalently in epineurial macrophages, whereas it detected interferon-γ 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.

Original languageEnglish
Pages (from-to)603-609
Number of pages7
JournalActa Neuropathologica
Volume96
Issue number6
DOIs
Publication statusPublished - Dec 1998

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Chronic Inflammatory Demyelinating Polyradiculoneuropathy
Sural Nerve
Demyelinating Diseases
Myelin Sheath
Biopsy
Onions
Major Histocompatibility Complex
Interleukin-1
Cellular Immunity
Interferons
Immunoglobulins
Edema
B-Lymphocytes
Up-Regulation
Tumor Necrosis Factor-alpha
Macrophages
Cytokines
T-Lymphocytes

Keywords

  • Cytokines
  • Focal pathological changes
  • Immune-mediated demyelination
  • Inflammation

ASJC Scopus subject areas

  • Clinical Neurology
  • Pathology and Forensic Medicine
  • Neuroscience(all)

Cite this

Rizzuto, N., Morbin, M., Cavallaro, T., Ferrari, S., Fallahi, M., & Galiazzo Rizzuto, S. (1998). Focal lesions area feature of chronic inflammatory demyelinating polyneuropathy (CIDP). Acta Neuropathologica, 96(6), 603-609. https://doi.org/10.1007/s004010050941

Focal lesions area feature of chronic inflammatory demyelinating polyneuropathy (CIDP). / Rizzuto, N.; Morbin, M.; Cavallaro, T.; Ferrari, S.; Fallahi, M.; Galiazzo Rizzuto, S.

In: Acta Neuropathologica, Vol. 96, No. 6, 12.1998, p. 603-609.

Research output: Contribution to journalArticle

Rizzuto, N, Morbin, M, Cavallaro, T, Ferrari, S, Fallahi, M & Galiazzo Rizzuto, S 1998, 'Focal lesions area feature of chronic inflammatory demyelinating polyneuropathy (CIDP)', Acta Neuropathologica, vol. 96, no. 6, pp. 603-609. https://doi.org/10.1007/s004010050941
Rizzuto N, Morbin M, Cavallaro T, Ferrari S, Fallahi M, Galiazzo Rizzuto S. Focal lesions area feature of chronic inflammatory demyelinating polyneuropathy (CIDP). Acta Neuropathologica. 1998 Dec;96(6):603-609. https://doi.org/10.1007/s004010050941
Rizzuto, N. ; Morbin, M. ; Cavallaro, T. ; Ferrari, S. ; Fallahi, M. ; Galiazzo Rizzuto, S. / Focal lesions area feature of chronic inflammatory demyelinating polyneuropathy (CIDP). In: Acta Neuropathologica. 1998 ; Vol. 96, No. 6. pp. 603-609.
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