Progressive multifocal leucoencephalopathy (PML) is a CNS infection of oligodendrocytes by JC virus, which rarely occurs in lupus, and can be mistaken for antiphospholipid antibody syndrome or neuropsychiatric systemic lupus erythematosus (NSLE). This case of PML in a patient with systemic lupus erythematosus on supra-therapeutic doses of methotrexate emphasises that CNS infection is an important diagnostic consideration before empiric treatment with immunosuppresants for NSLE.
2 Ahmed F Aziz T Kaufman LD . Progressive multifocal leukoencephalopathy in a patient with systemic lupus erythematosus.J Rheumatol1999; 26: 1609–1612.
3.
3 Aksamit AJ . Review of progressive multifocal leukoencephalopathy and natalizumab.Neurologist2006; 12: 293–298.
4.
4 Arbusow V Strupp M Pfister HW Seelos KC Bruckmann H Brandt T . Contrast enhancement in progressive multifocal leukoencephalopathy: a predictive factor for long-term survival?J Neurol2000; 247: 306–308.
5.
5 Itoh K Kano T Nagashio C Mimori A Kinoshita M Sumiya M . Progressive multifocal leukoencephalopathy in patients with systemic lupus erythematosus.Arthritis Rheum2006; 54: 1020–1022.
6.
6 Jones HR Hedley-Whyte ET Freidberg SR Kelleher JE Krolikowski J . Primary cerebellopontine progressive multifocal leukoencephalopathy diagnosed premortem by cerebellar biopsy.Ann Neurol1982; 11: 199–202.
7.
7 Kaye BR Neuwelt CM London SS DeArmond SJ . Central nervous system systemic lupus erythematosus mimicking progressive multifocal leucoencephalopathy.Ann Rheum Dis1992; 51: 1152–1156.
8.
8 Kinoshita M Iwana K Shinoura H Aotsuka S Sumiya M . Progressive multifocal leukoencephalopathy resembling central nervous system systemic lupus erythematosus.Clin Exp Rheumatol1998; 16: 313–315.
9.
9 Malas D Weiss S . Progressive multifocal leukoencephalopathy and cryptococcal meningitis with systemic lupus erythematosus and thymoma.Ann Neurol1977; 1: 188–191.
10.
10 Newton P Aldridge RD Lessells AM Best PV . Progressive multifocal leukoencephalopathy complicating systemic lupus erythematosus.Arthritis Rheum1986; 29: 337–343.
11.
11 Reilmann R Imai T Ringelstein EB . Remission of progressive multifocal leucoencephalopathy in SLE after treatment with cidofovir: a 4 year follow up.J Neurol Neurosurg Psychiatry2005; 76: 1304–1305.
12.
12 Salmaggi A Maccagnano E Castagna A . Reversal of CSF positivity for JC virus genome by cidofovir in a patient with systemic lupus erythematosus and progressive multifocal leukoencephalopathy.Neurol Sci2001; 22: 17–20.
13.
13 Tomura N Watanabe M Kato T Nishino K Kowada M . Case report: progressive multifocal leukoencephalopathy with prominent medullary veins on angiogram.Clin Radiol1994; 49: 66–68.
14.
14 Vandecasteele SJ Maes B Claes K Sciot R Vanrenterghem Y . High anti-double-stranded DNA antibodies and progressive multifocal leukoencephalopathy in a patient with systemic lupus erythematosus.Nephrol Dial Transplant2005; 20: 1246–1247.
15.
15 Warnatz K Peter HH Schumacher M . Infectious CNS disease as a differential diagnosis in systemic rheumatic diseases: three case reports and a review of the literature.Ann Rheum Dis2003; 62: 50–57.
16.
16 Weiner LP Herndon RM Narayan O . Isolation of virus related to SV40 from patients with progressive multifocal leukoencephalopathy.N Engl J Med1972; 286: 385–390.
17.
17 Brey RL Holliday SL Saklad AR . Neuropsychiatric syndromes in lupus: prevalence using standardized definitions.Neurology2002; 58: 1214–1220.
18.
18 Sanna G Bertolaccini ML Cuadrado MJ Khamashta MA Hughes GR . Central nervous system involvement in the antiphospholipid (Hughes) syndrome.Rheumatology2003; 42: 200–213.
19.
19 Joseph FG Lammie GA Scolding NJ . CNS lupus: a study of 41 patients.Neurology2007; 69: 644–654.
20.
20 Lima MA Drislane FW Koralnik IJ . Seizures and their outcome in progressive multifocal leukoencephalopathy.Neurology2006; 66: 262–264.
21.
21 Post MJ Yiannoutsos C Simpson D . Progressive multifocal leukoencephalopathy in AIDS: are there any MR findings useful to patient management and predictive of patient survival? AIDS Clinical Trials Group, 243 Team.AJNR Am J Neuroradiol1999; 20: 1896–1906.
22.
22 Viallard JF Ellie E Lazaro E Lafon ME Pellegrin JL . JC virus meningitis in a patient with systemic lupus erythematosus.Lupus2005; 14: 964–966.
23.
23 Asato R Akiyama Y Ito M . Nuclear magnetic resonance abnormalities of the cerebral white matter in children with acute lymphoblastic leukemia and malignant lymphoma during and after central nervous system prophylactic treatment with intrathecal methotrexate.Cancer1992; 70: 1997–2004.
24.
24 Renard D Westhovens R Vandenbussche E Vandenberghe R . Reversible posterior leucoencephalopathy during oral treatment with methotrexate.J Neurol2004; 251: 226–228.
25.
25 Yokoo H Nakazato Y Harigaya Y Sasaki N Igeta Y Itoh H . Massive myelinolytic leukoencephalopathy in a patient medicated with low-dose oral methotrexate for rheumatoid arthritis: an autopsy report.Acta Neuropathol2007; 114: 425–430.
26.
26 Worthley SG McNeil JD . Leukoencephalopathy in a patient taking low dose oral methotrexate therapy for rheumatoid arthritis.J Rheumatol1995; 22: 335–337.
27.
27 Raghavendra S Nair MD Chemmanam T Krishnamoorthy T Radhakrishnan VV Kuruvilla A . Disseminated necrotizing leukoencephalopathy following low-dose oral methotrexate.Eur J Neurol2007; 14: 309–314.
28.
28 McEvoy GK ed. Methotrexate. In: AHFS drug information 2004. Bethesda, MD: American Society of Health-System Pharmacists; 2006: 1088.
29.
29 Berger JR Levy RM Flomenhoft D Dobbs M . Predictive factors for prolonged survival in acquired immunodeficiency syndrome-associated progressive multifocal leukoencephalopathy.Ann Neurol1998; 44: 341–349.
30.
30 Du Pasquier RA Kuroda MJ Zheng Y Jean-Jacques J Letvin NL Koralnik IJ . A prospective study demonstrates an association between JC virus-specific cytotoxic T lymphocytes and the early control of progressive multifocal leukoencephalopathy.Brain2004; 127: 1970–1978.
31.
31 Van Assche G Van Ranst M Sciot R . Progressive multifocal leukoencephalopathy after natalizumab therapy for Crohn’s disease.N Engl J Med2005; 353: 362–368.
32.
32 Langer-Gould A Atlas SW Green AJ Bollen AW Pelletier D . Progressive multifocal leukoencephalopathy in a patient treated with natalizumab.N Engl J Med2005; 353: 375–381.
33.
33 Kleinschmidt-DeMasters BK Tyler KL . Progressive multifocal leukoencephalopathy complicating treatment with natalizumab and interferon beta-1a for multiple sclerosis.N Engl J Med2005; 353: 369–374.
34.
34 Wang H Xu J Ji X . The abnormal apoptosis of T cell subsets and possible involvement of IL-10 in systemic lupus erythematosus.Cell Immunol2005; 235: 117–121.
35.
35 Aksamit AJ . Treatment of non-AIDS progressive multifocal leukoencephalopathy with cytosine arabinoside.J Neurovirol2001; 7: 386–390.
36.
36 Albrecht H Hoffmann C Degen O . Highly active antiretroviral therapy significantly improves the prognosis of patients with HIV-associated progressive multifocal leukoencephalopathy.AIDS1998; 12: 1149–1154.
37.
37 Garrels K Kucharczyk W Wortzman G Shandling M . Progressive multifocal leukoencephalopathy: clinical and MR response to treatment.AJNR Am J Neuroradiol1996; 17: 597–600.
38.
38 Vulliemoz S Lurati-Ruiz F Borruat FX . Favourable outcome of progressive multifocal leucoencephalopathy in two patients with dermatomyositis.J Neurol Neurosurg Psychiatry2006; 77: 1079–1082.
39.
39 Elphick GF Querbes W Jordan JA . The human polyomavirus, JCV, uses serotonin receptors to infect cells.Science2004; 306: 1380–1383.
