Summary
A female patient with Wegener's granulomatosis developed severe bone marrow depression after two years treatment with cyclophosphamide. Corticosteroids alone could not sufficiently suppress disease activity, therefore additive therapy with Cyclosporin A was started. Four weeks later the patient developed a central nervous system disorder with affective disturbances and progressive somnolence. However, inspite of intensive diagnostic procedures, no definite diagnosis could be established. After another two months she died. Post-mortem-examination showed progressive multifocal leukoencephalopathy. An association between immunosuppressive therapy and reactivation of JC-Virus is suggested.
Similar content being viewed by others
Abbreviations
- PML:
-
Progressive multifokale Leukoenzephalopathie
- WG:
-
Wegener'sche Granulomatose
- CyA:
-
Cyclosporin A
- (C)CT:
-
(Craniale)Computertomographie
- NMR:
-
Nuclear Magnetic Resonance (Kernspintomographic)
- ZNS:
-
Zentralnervensystem
- AIDS:
-
Acquired Immuno-deficiency Syndrome
Literatur
Astroem KE et al. (1958) Progressive multifocal leukoencephalopathy. Brain 81:93–111
Atkinson K et al. (1984) Cyclosporin-associated central nervous system toxicity after allogeneic bone marrow transplantation. Transplantation 38:34–37
Berger JR et al. (1987) Progressive multifocal leukoencephalopathy associated with human immunodeficiency virus infection. Ann Intern Med 107:78–87
Borleffs et al. (1987) Treatment of Wegener's granulomatosis with cyclosporin. Ann Rheum Dis 46:175
Brooks BR, Walker DL (1984) Progressive multifocal leukoencephalopathy. Neurol Clin 2:299–313
Budka H, Shah KV (1983) Papovavirus antigens in paraffin sections of PML brains. In: Sever JL, Madden DL (eds): Polyomaviruses and Human Neurological Disease. Progr Clin Biol Res 105:299–309
Chou S, Norman DJ (1985) Effect of OKT3 Antibody Therapy on Cytomegalovirus Reactivation in Renal Transplant Recipients. Transplant Proc XVII:2755–2756
Fahey JL et al. (1987) Immune Interventions in Disease. Ann Intern Med 106:257–274
Gross WL et al. (1986) Die Wegener'sche Granulomatose — eine primäre Vaskulitis. In: Schilling F (ed) Colloquia rheumatologica 33. Banaschewski, München, S 39–58
Hedley-Whyte ET et al. (1966) Multifocal leukoencephalopathy with remission and five year survival. J Neuropathol 25:107–116
Houff SA et al. (1988) Involvement of JC virus infected mononuclear cells from the bone marrow and spleen in the pathogenesis of progressive multifocal leukoencephalopathy. New Engl J Med 318:301–305
Padgett BL et al. (1971) Cultivation of papovalike virus from human brain with PML. Lancet 1:1257–1260
Padgett BL, Walker DL (1983) Virologic and serologic studies of progressive multifocal leukoencephalopathy. Progr Clin Biol Res 105:107–117
Price RW et al. (1983) Progressive multifocal leukoencephalopathy: a burnt-out case. Ann Neurol 13:485–490
Reznik MJ et al. (1981) Two cases of progressive multifocal leukoencephalopathy after renal transplantation. Acta Neuropathol Suppl 7:189–191
Shillitoe EJ et al. (1974) Immunological features of Wegener's Granulomatosis. Lancet 1:281–284
Van der Woude et al. (1985) Autoantibodies against neutrophils and monocytes: Tool for diagnosis and marker of disease activity in Wegener's Granulomatosis. Lancet 1:425–429
Walker DL (1985) Progressive multifocal leukoencephalopathy. In: Vinken et al. (eds) Handbook of clinical neurology, Vol. 47. Elsevier, Amsterdam, pp 503–524
Wegener F (1981) Wegenersche Granulomatose. In: Hopf Ch et al. (eds) Neurologie in Praxis und Klinik, Bd. 2. Thieme, Stuttgart, S 4.219–4.223
Willoughby E et al. (1980) Progressive multifocal leukoencephalopathy (PML): In vitro cell-mediated immune responses to mitogens and JC virus. Neurology 30:256–262
Zu Rhein GM, Chou SM (1965) Particles resembling papovaviruses in human cerebral demyelinating disease. Science 148:1477–1479
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Ettinger, J., Feiden, W., Hübner, G. et al. Progressive multifokale Leukoenzephalopathie bei Wegener'scher Granulomatose unter Therapie mit Cyclosporin A. Klin Wochenschr 67, 260–264 (1989). https://doi.org/10.1007/BF01717330
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF01717330