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[Cerebral angiitis and Goodpasture's syndrome].

P Garnier1, C Deprele, B Pilonchéry

  • 1Service de Neurologie, Fédération Neuro-Vasculaire, CHU, Saint-Etienne. pierre.garnier@univ-st-etienne.fr

Revue Neurologique
|March 6, 2003
PubMed
Summary

Goodpasture's syndrome, a kidney and lung disease, can rarely affect the brain. This case highlights central nervous system involvement due to cerebral angiitis, successfully treated with cyclophosphamide.

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Area of Science:

  • Nephrology
  • Neurology
  • Immunology

Background:

  • Goodpasture's syndrome is characterized by glomerulonephritis and pulmonary hemorrhage.
  • Antiglomerular basement membrane (anti-GBM) antibodies are the hallmark of this autoimmune condition.
  • Central nervous system (CNS) involvement is an exceptionally rare manifestation.

Observation:

  • A 55-year-old woman presented with fluctuating neurobehavioral symptoms over nine months.
  • Cerebral angiography revealed distal arterial lesions consistent with angiitis.
  • Elevated anti-GBM antibody titers were detected, while anti-neutrophil cytoplasmic antibodies were negative.

Findings:

  • The patient's clinical presentation and diagnostic findings suggest CNS involvement secondary to cerebral angiitis.

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  • The cerebral angiitis is potentially linked to the presence of anti-GBM antibodies.
  • Treatment with cyclophosphamide led to a favorable clinical outcome.

    • Implications:

    • This case underscores the possibility of CNS manifestations in Goodpasture's syndrome.
    • It suggests a potential role for anti-GBM antibodies in the pathogenesis of cerebral angiitis.
    • Further research is warranted to elucidate the mechanisms and management of neuro-Goodpasture's syndrome.