Long term survival from progressive multifocal leucoencephalopathy after liver transplantation

Abstract

Progressive multifocal leucoencephalopathy (PML) is an infectious disease caused by JC virus that affects the immunocompromised. First described in 1958 in patients with Hodgkins disease and chronic lymphatic leukaemia [ [1] Astrom K.E. Mancall E.L. Richardson E.P. Progressive multifocal leukoencephalopathy: a hitherto unrecognised complication of chronic lymphatic leukaemia and Hodgkin's disease. Brain. 1958; 81: 93-111 Crossref PubMed Scopus (692) Google Scholar ], it was subsequently recognised in patients with other causes of immunodeficiency, most commonly human immunodeficiency virus infection [ [2] Berger J.R. Kaszovitz B. Donovan Post J. Dickinson G. Progressive multifocal encephalopathy associated with human immunodeficiency virus infection. Ann Intern Med. 1987; 107: 78-87 Crossref PubMed Scopus (438) Google Scholar ]. It usually causes rapidly progressive neurological deficits including hemiparesis, visual field defects, cognitive impairment, cerebellar and brain stem signs and death. PML has been reported in patients on immunosuppressant medication after organ transplantation [ 3 Bronster D.J. Lidov M.W. Wolfe D. Schwartz M.E. Miller C.M. Progressive multifocal leukoencephalopathy after orthotopic liver transplantation. Liver Transplant Surg. 1995; 1: 371-372 Crossref PubMed Scopus (26) Google Scholar , 4 Lewis A.R. Kline L.B. Pinkard N.B. Visual loss due to progressive multifocal leukoencephalopathy in a heart transplant patient. J Clin Neuro-Ophthalmol. 1993; 13: 237-241 PubMed Google Scholar , 5 Worthman F. Turker T. Muller A.R. Patt S. Stoltenburg-Didinger G. Progressive multifocal leukoencephalopathy after orthotopic liver transplantation. Transplantation. 1994; 57: 1268-1270 Crossref PubMed Scopus (19) Google Scholar , 6 Egan J.D. Ring B.L. Reding M.J. Wells I.C. Reticulum cell sarcoma and progressive multifocal leukoencephalopathy following renal transplantation. Transplantation. 1980; 29: 84-86 Crossref PubMed Scopus (26) Google Scholar , 7 Embrey J.R. Silva F.G. Helderman J.H. Peters P.C. Sagalowsky A.I. Long-term survival and late development of bladder cancer in renal transplant patient with progressive multifocal leukoencephalopathy. J Urol. 1988; 139: 580-581 PubMed Google Scholar , 8 Saxton C.R. Gailiunas P.J.r. Helderman J.H. Farkas R.A. McCoy R. Diehl J. et al. Progressive multifocal leukoencephalopathy in a renal transplant recipient. Increased diagnostic sensitivity of computorised tomography by double-dose contrast with delayed films. American Journal of Medicine. 1984; 77: 333-337 Abstract Full Text PDF PubMed Scopus (17) Google Scholar , 9 Hall C.D. Dafni U. Simpson D. Clifford D. Wetherill P.E. Cohen B. et al. Failure of cytarabine in progressive multifocal leucoencephalopathy associated with human immunodeficiency virus infection. N Engl J Med. 1999; 338: 1345-1352 Crossref Scopus (295) Google Scholar ]. The prognosis of patients with PML is usually around 2.5–4 months [ [2] Berger J.R. Kaszovitz B. Donovan Post J. Dickinson G. Progressive multifocal encephalopathy associated with human immunodeficiency virus infection. Ann Intern Med. 1987; 107: 78-87 Crossref PubMed Scopus (438) Google Scholar ], although rarely more prolonged survival can occur [ [10] Berger J.R. Mucke L. Prolonged survival and partial recovery in AIDS-associated progressive leukoencephalopathy. N Engl J Med. 1988; 38: 1060-1065 Google Scholar ]. We report the case of a 60-year-old woman who developed PML after liver transplantation, was treated with cytarabine and withdrawal of her immunosuppressants and is alive 18 months after diagnosis.