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Nephrol Dial Transplant (2000) 15: 893-896
© 2000 European Renal Association-European Dialysis and Transplant Association

Case Reports

Vasculitic Wallenberg syndrome with detection of anti-proteinase 3 antibodies in the cerebrospinal fluid of a patient with severe Wegener's granulomatosis and only mild kidney involvement

Justus Faust1,, Anke Visbeck2, Sabine Fitzek2, Clemens Fitzek3, Thomas Orth1, Eveline Wandel1 and Werner J. Mayet1

1 Departments of Internal Medicine, 2 Neurology and 3 Neuroradiology, Johannes Gutenberg-Universität Mainz, Mainz, Germany

Keywords: ANCA; proteinase 3; Wallenberg syndrome; Wegener's granulomatosis; vasculitic neurological involvement



   Introduction
 
Neurological involvement of Wegener's granulomatosis (WG) is usually accompanied by affection of upper airways, lungs and kidneys. In a large study of patients with WG [1], neurological manifestation was associated with lung disease in 67%, upper airway disease in 77% and kidney disease in 66%. In patients with peripheral neuropathy the percentage of renal involvement was significantly higher (83%) than in patients showing other neurological manifestations.

We report the case of a patient with vasculitic WG and the clinically-leading involvement of the brainstem (lateral medullary syndrome or Wallenberg syndrome) and peripheral nerves (multiple mononeuropathy).

Moreover, we were able to detect C-ANCA with the target antigen proteinase 3 (PR 3) in the cerebrospinal fluid (CSF). Despite marked involvement of the lungs and upper airways the patient developed only minimal kidney disease.

Because C-ANCA could be detected in the CSF without disturbance of . . . [Full Text of this Article]



   Case
 


   Discussion
 


   Notes
 


   References
 

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