Cerebral Involvement in a Patient with Goodpasture's Disease Due to Shortened Induction Therapy: a Case Report

Overview

Journal J Med Case Rep

Publisher Biomed Central

Specialty General Medicine

Date 2009 Dec 1

PMID 19946538

Citations 1

Authors

Christoph Preul

Jens Gerth

Sebastian Lang

Christoph Bergmeier

Otto W Witte

Gunter Wolf

Christoph Terborg

Affiliations

Soon will be listed here.

Abstract

Introduction: Goodpasture's disease is a rare immunological disease with formation of pathognomonic antibodies against renal and pulmonary basement membranes. Cerebral involvement has been reported in several cases in the literature, yet the pathogenetic mechanism is not entirely clear.

Case Presentation: A 21-year-old Caucasian man with Goodpasture's disease and end-stage renal disease presented with two generalized seizures after a period of mild cognitive disturbance. Blood pressure and routine laboratory tests did not exceed the patient's usual values, and examination of cerebrospinal fluid was unremarkable. Cerebral magnetic resonance imaging (MRI) revealed multiple cortical and subcortical lesions on fluid-attenuated inversion recovery sequences. Since antiglomerular basement membrane antibodies were found to be positive with high titers, plasmapheresis was started. In addition, cyclophosphamide pulse therapy was given on day 13. Encephalopathy and MRI lesions disappeared during this therapy, and antiglomerular basement membrane antibodies were significantly reduced. Previous immunosuppressive therapy was performed without corticosteroids and terminated early after 3 months. The differential diagnostic considerations were cerebral vasculitis and posterior reversible encephalopathy syndrome. Vasculitis could be seen as an extrarenal manifestation of the underlying disease. Posterior reversible encephalopathy syndrome, on the other hand, can be triggered by immunosuppressive therapy and may appear without a hypertensive crisis.

Conclusion: A combination of central nervous system symptoms with a positive antiglomerular basement membrane test in a patient with Goodpasture's disease should immediately be treated as an acute exacerbation of the disease with likely cross-reactivity of antibodies with the choroid plexus. In our patient, a discontinuous strategy of immunosuppressive therapy may have favored recurrence of Goodpasture's disease.

Citing Articles

Hypertensive emergency and seizures in a 30-year-old man with anti-glomerular basement membrane disease.

Mascola M, Karmali R, Mathews J, Obligado S Clin Case Rep. 2020; 8(12):3322-3327.

PMID: 33363926 PMC: 7752327. DOI: 10.1002/ccr3.3361.

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