Smoldering myeloma presenting as progressive multifocal leukoencephalopathy: a case report

Journal of Medical Case Reports

Bernd Salzberger²  Michael Boewer¹  Roland Büttner²  Martina Troppmann² 

[1] Department of Radiology, Hospital of Deggendorf/Mainkofen, Mainkofen, Germany;Department of Internal Medicine I, University of Regensburg, Regensburg, Germany

关键词: Plasmacytoma;    Cidofovir;    Polyomavirus JC (JCV);    Progressive multifocal leukoencephalopathy (PML);   

Others  :  1195708
DOI  :  10.1186/1752-1947-6-177

 received in 2011-12-23, accepted in 2012-04-25,  发布年份 2012

【 摘 要 】

Introduction

Progressive multifocal leukoencephalopathy is an opportunistic infection occurring in patients with severe cellular immunodeficiency. This case highlights the role of cellular immunodeficiency in the reactivation of John Cunningham virus in a case of an early stage plasmacytoma.

Case presentation

A 76-year-old Caucasian woman presented with progressive left-sided hemiparesis, accompanied by hypoesthesia, hypoalgesia and neuropsychological symptoms. Magnetic resonance imaging demonstrated new hyperattenuating lesions in the right thalamus and left-sided subcortically. A polymerase chain reaction test revealed 4500 copies of John Cunningham virus-deoxyribonucleic acid/ml in cerebrospinal fluid. Human immunodeficiency virus infection was ruled out. A bone marrow biopsy showed an early stage immunoglobulin G-kappa plasmacytoma. Cidofovir (5mg/kg) weekly for three weeks was started. A significant improvement of her neuropsychological symptoms was achieved, but motor system and sensory symptoms did not change.

Conclusions

This case shows a rapid course of progressive multifocal leukoencephalopathy with severe residual deficits. In the diagnostic workup of all patients with atypical neurologic symptoms or immunodeficiency, progressive multifocal leukoencephalopathy should be included as a differential diagnosis.

【 授权许可】

   
2012 Troppmann et al.; licensee BioMed Central Ltd.

【 图 表 】

Figure 1 .

【 参考文献 】

• [1]White MK, Khalili K: Pathogenesis of progressive multifocal leukoencephalopathy-revisited. J Infect Dis 2011, 203:578-586.
• [2]Major EO, Amemiya K, Tornatore CS, Houff SA, Berger JR: Pathogenesis and molecular biology of progressive multifocal leukoencephalopathy, the JC Virus-induced demyelinating disease of the human brain. Clin Microbiol Rev 1992, 5:49-73.
• [3]Whiteman MLH, Post MJD, Berger JR, Tate LG, Bell MD, Limonte LP: Progressive multifocal leukoencephalopathy in 47 HIV-seropositive patients: neuroimaging with clinical and pathologic correlation. Radiology 1993, 187:233-240.
• [4]Taoufink Y, Gasnault J, Karaterki A, Pierre Ferey M, Marchadier E, Goujard C, Lannuzel A, Delfraissy JF, Dussaix E: Prognostic value of JC virus load in cerebrospinal fluid of patients with progressive multifocal leukoencephalopathy. J Infect Dis 1998, 178:1816-1820.
• [5]Happe S, Besselmann M, Matheja P, Rickert CH, Schuierer G, Reichelt D, Schuierer G, Reichelt D, Husstedt IW: Cidofovir (vistide) in therapy of progressive multifocal leukoencephalopathy in AIDS. Review of the literature and report of 2 cases. Nervenarzt 1999, 70:935-943.
• [6]Hall CD, Dafni U, Simpson D, Clifford D, Wetherill PE, Cohen B, McArthur J, Hollander H, Yainnoutsos C, Major E, Millar L, Timpone J: Failure of cytarabine in progressive mulitifocal leukoencephalopathy associated with human immunodeficiency virus infection. AIDS Clinical Trials Group 243 Team. N Engl J Med 1998, 338:1345-1351.
• [7]Geschwind MD, Skolasky RI, Royal WS, McArthur JC: The relative contributions of HAART and alpha interferon for therapy of progressive mulitifocal leukoencephalopathy in AIDS. J Neurovirol 2001, 7:353-357.
• [8]Re D, Bamborschke S, Feiden W, Schröder R, Lehrke R, Diehl V, Tesch H: Progressive multifocal leukoencephalopathy after autologous bone marrow transplantation and alpha-interferon immunoherapy. Bone Marrow Transplant 1999, 23:295-298.