Progressive multifocal leukoencephalopathy in an immunocompetent patient

被引:21
作者
Christakis, Panos G. [1 ]
Okin, Daniel [1 ]
Huttner, Anita J. [2 ]
Baehring, Joachim M. [3 ,4 ]
机构
[1] Yale Univ, Sch Med, New Haven, CT USA
[2] Yale Univ, Sch Med, Dept Pathol, New Haven, CT 06510 USA
[3] Yale Univ, Sch Med, Dept Neurol, New Haven, CT 06510 USA
[4] Yale Univ, Sch Med, Dept Neurosurg & Med, New Haven, CT USA
关键词
Progressive multifocal leukoencephalopathy; JC virus; Immunocompetent; Immunosuppression; Mefloquine; Mirtazapine; Neuroimmunology; Neuropathology; IMMUNODEFICIENCY; MEFLOQUINE;
D O I
10.1016/j.jns.2013.01.010
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
A 69-year old man presents with a subacute history of worsening confusion, anxiety and abnormal gait. Brain MRI revealed an extensive non-enhancing signal abnormality of parieto-occipito-temporal white matter. CSF PCR was positive for JC virus, suggestive of progressive multifocal leukoencephalopathy (PML). Extensive workup for occult immunosuppression was negative. Although PML in an immunocompetent patient is exceedingly rare, biopsy confirmed the diagnosis. Mirtazapine and mefloquine therapies were initiated and JCV DNA titre decreased by 100-fold at six months. One year later, his clinical course had stabilized and neuroimaging was improved. Our case suggests that PML can rarely afflict immunocompetent individuals and that serotonin receptor targeting pharmacological therapy may improve the outcome. (c) 2013 Elsevier B.V. All rights reserved.
引用
收藏
页码:107 / 110
页数:4
相关论文
共 14 条
[1]   Prognostic significance of JC virus DNA levels in cerebrospinal fluid of patients with HIV-associated progressive multifocal leukoencephalopathy [J].
Bossolasco, S ;
Calori, G ;
Moretti, F ;
Boschini, A ;
Bertelli, D ;
Mena, M ;
Gerevini, S ;
Bestetti, A ;
Pedale, R ;
Sala, S ;
Sala, S ;
Lazzarin, A ;
Cinque, P .
CLINICAL INFECTIOUS DISEASES, 2005, 40 (05) :738-744
[2]   Progressive multifocal leukoencephalopathy and other forms of JC virus disease [J].
Brew, Bruce J. ;
Davies, Nicholas W. S. ;
Cinque, Paola ;
Clifford, David B. ;
Nath, Avindra .
NATURE REVIEWS NEUROLOGY, 2010, 6 (12) :667-679
[3]   Identification and Characterization of Mefloquine Efficacy against JC Virus In Vitro [J].
Brickelmaier, Margot ;
Lugovskoy, Alexey ;
Kartikeyan, Ramya ;
Reviriego-Mendoza, Marta M. ;
Allaire, Norm ;
Simon, Kenneth ;
Frisque, Richard J. ;
Gorelik, Leonid .
ANTIMICROBIAL AGENTS AND CHEMOTHERAPY, 2009, 53 (05) :1840-1849
[4]   Mirtazapine Use in Human Immunodeficiency Virus-Infected Patients With Progressive Multifocal Leukoencephalopathy [J].
Cettomai, Deanna ;
McArthur, Justin C. .
ARCHIVES OF NEUROLOGY, 2009, 66 (02) :255-258
[5]   DECREASED CELL-MEDIATED-IMMUNITY IN PATIENTS WITH NON-INSULIN-DEPENDENT DIABETES-MELLITUS [J].
CHANG, FY ;
SHAIO, MF .
DIABETES RESEARCH AND CLINICAL PRACTICE, 1995, 28 (02) :137-146
[6]   The human polyomavirus, JCV, uses serotonin receptors to infect cells [J].
Elphick, GF ;
Querbes, W ;
Jordan, JA ;
Gee, GV ;
Eash, S ;
Manley, K ;
Dugan, A ;
Stanifer, M ;
Bhatnagar, A ;
Kroeze, WK ;
Roth, BL ;
Atwood, WJ .
SCIENCE, 2004, 306 (5700) :1380-1383
[7]  
Friedman R, 2011, Neurology Today, V11, P8, DOI [10.1097/01.NT.0000397959.27386.55, DOI 10.1097/01.NT.0000397959.27386.55]
[8]   Progressive multifocal leukoencephalopathy in individuals with minimal or occult immunosuppression [J].
Gheuens, Sarah ;
Pierone, Gerald ;
Peeters, Patrick ;
Koralnik, Igor J. .
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY, 2010, 81 (03) :247-254
[9]   Mefloquine in the treatment of progressive multifocal leukoencephalopathy [J].
Gofton, T. E. ;
Al-Khotani, A. ;
O'Farrell, B. ;
Ang, L. C. ;
McLachlan, R. S. .
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY, 2011, 82 (04) :452-455
[10]   Determinants of survival in progressive multifocal leukoencephalopathy [J].
Marzocchetti, A. ;
Tompkins, T. ;
Clifford, D. B. ;
Gandhi, R. T. ;
Kesari, S. ;
Berger, J. R. ;
Simpson, D. M. ;
Prosperi, M. ;
De Luca, A. ;
Koralnik, I. J. .
NEUROLOGY, 2009, 73 (19) :1551-1558