Chronic inflammatory axonal polyneuropathy

J Neurol Neurosurg Psychiatry. 2020 Nov;91(11):1175-1180. doi: 10.1136/jnnp-2020-323787. Epub 2020 Sep 11.

Abstract

Objectives: Chronic inflammatory axonal polyneuropathy (CIAP) is defined on the basis of the clinical, electrophysiological and nerve biopsy findings and therapeutic responses of 'immunotherapy responding chronic axonal polyneuropathy (IR-CAP)'.

Methods: The diagnosis of IR-CAP was made when all of three of the following mandatory criterion were met: (1) acquired, chronic progressive or relapsing symmetrical or asymmetrical polyneuropathy with duration of progression >2 months; (2) electrophysiological evidence of axonal neuropathy in at least two nerves without any evidence of 'strict criteria of demyelination'; and (3) definite responsiveness to immunotherapy.

Results: Thirty-three patients with IR-CAP showed similar clinical features of chronic inflammatory demyelinating polyneuropathy (CIDP) except 'motor neuropathy subtype'. High spinal fluid protein was found in 27/32 (78%) cases. 'Inflammatory axonal neuropathy' was proven in 14 (45%) of 31 sural nerve biopsies.

Discussions: IR-CAP could well be 'axonal CIDP' in view of clinical similarity, but not proven as yet. Thus, IR-CAP is best described as CIAP, a distinct entity that deserves its recognition in view of responsiveness to immunotherapy.

Conclusion: Diagnosis of CIAP can be made by additional documentation of 'inflammation' by high spinal fluid protein or nerve biopsy in addition to the first two diagnostic criteria of IR-CAP.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Autoimmune Diseases of the Nervous System / classification
  • Autoimmune Diseases of the Nervous System / diagnosis*
  • Autoimmune Diseases of the Nervous System / drug therapy
  • Autoimmune Diseases of the Nervous System / physiopathology
  • Axons / pathology
  • Azathioprine / therapeutic use
  • Biopsy
  • Child
  • Child, Preschool
  • Chronic Disease
  • Cyclophosphamide / therapeutic use
  • Cyclosporine / therapeutic use
  • Electromyography
  • Female
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Immunologic Factors / therapeutic use
  • Inflammation / classification
  • Inflammation / diagnosis*
  • Inflammation / drug therapy
  • Inflammation / physiopathology
  • Male
  • Middle Aged
  • Mycophenolic Acid / therapeutic use
  • Neural Conduction
  • Polyneuropathies / classification
  • Polyneuropathies / diagnosis*
  • Polyneuropathies / drug therapy
  • Polyneuropathies / physiopathology
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / diagnosis*
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / physiopathology
  • Sural Nerve / pathology
  • Young Adult

Substances

  • Immunoglobulins, Intravenous
  • Immunologic Factors
  • Cyclosporine
  • Cyclophosphamide
  • Mycophenolic Acid
  • Azathioprine