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Vestibular dysfunction in chronic inflammatory demyelinating polyneuropathy

E M Frohman1, R Tusa, A S Mark

  • 1Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.

Annals of Neurology
|April 1, 1996
PubMed
Summary
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Chronic inflammatory demyelinating polyneuropathy (CIDP) can affect cranial nerves. This case study shows immune-mediated vestibular dysfunction synchronized with CIDP, responding to immune therapy.

Area of Science:

  • Neurology
  • Immunology

Background:

  • Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated disorder affecting peripheral nerves.
  • Cranial nerve involvement in CIDP is occasionally reported but often lacks a clear relationship.

Observation:

  • A 45-year-old woman presented with CIDP symptoms, including limb weakness and gait instability.
  • She also exhibited bilateral vestibulopathy, confirmed by clinical and neurophysiological testing, with MRI showing cranial nerve VIII enhancement.
  • An IgG kappa monoclonal gammopathy of undetermined significance was identified.

Findings:

  • The patient experienced a relapsing-remitting course of both CIDP and vestibulopathy over six years.
  • Quantitative testing revealed striking synchronization between the clinical courses of CIDP and vestibulopathy.

Related Experiment Videos

  • Both conditions responded concurrently to immune therapy.

    • Implications:

    • This case suggests that vestibular dysfunction in CIDP can be immune-mediated.
    • The findings highlight the importance of considering immune-mediated mechanisms in cranial neuropathies associated with CIDP.
    • Synchronous clinical courses and treatment responses indicate a shared underlying immune pathogenesis.