Cerebral involvement in a patient with Goodpasture's disease due to shortened induction therapy: a case report
IntroductionGoodpasture'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 presentationA 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.
Author: Christoph PreulJens GerthSebastian LangChristoph BergmeierOtto WitteGunter WolfChristoph Terborg
Credits/Source: Journal of Medical Case Reports 2009, 3:120
Published on: 2009-11-12
Copyright by the authors listed above - made available via BioMedCentral (Open Access). Please
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