Journal of the Formosan Medical Association
Volume 106, Issue 3, Supplement , Pages S24-S28, 2007

Progressive Multifocal Leukoencephalopathy in an AIDS Patient

  • Lih-Shinn Wang

      Affiliations

    • Department of Infection, Tzu-Chi University and Buddhist Tzu-Chi General Hospital, Hualien, Taiwan
    • ,
  • Pao-Sheng Yen

      Affiliations

    • Department of Radiology, Tzu-Chi University and Buddhist Tzu-Chi General Hospital, Hualien, Taiwan

Department of Pathology, Tzu-Chi University and Buddhist Tzu-Chi General Hospital, Hualien, Taiwan

Received 20 October 2005; received in revised form 14 March 2006; accepted 2 May 2006.

Article Outline

Progressive multifocal leukoencephalopathy (PML) is a demyelinating brain disease caused by Jamestown Canyon virus (JCV). This disease is an important cause of morbidity and mortality in acquired immunodeficiency syndrome (AIDS) patients. We report a 34-year-old man infected with HIV-1 who presented with frequent general tonic clonic seizure and left side weakness for 2 months. Clinical features and magnetic resonance imaging (MRI) findings with hyperintensity on T2-weighted imaging and low density on T2 fluid attenuated inversion recovery involving multiple white matter were compatible with PML. He died of sepsis 2 months after diagnosis. Autopsy demonstrated progressive multifocal leukoencephalopathy according to characteristic histopathologic picture with multifocal demyelination, bizarre astrocytes formation and basophilic intranuclear inclusion bodies in the oligodendrocytes. JCV genome was demonstrated in the nucleus of oligodendrocytes using in situ hybridization. In conclusion, in AIDS patients with neurologic signs and typical MRI findings who present with multifocal demyelination lesions, PML should be diagnosed clinically. [J Formos Med Assoc 2007;106(3 Suppl):S24-S28]

Key Words:  acquired immunodeficiency syndrome , Jamestown Canyon virus , progressive multifocal leukoencephalopathy

No full text is available. To read the body of this article, please view the PDF online.

 

Back to Article Outline

References 

  1. Brooks B , Walker D . Progressive mutifocal leukoencephalopathy . Neurol Clin . 1984;2:299–313
  2. Munoz-Marmol AM , Mola G , Fernandez-Vasalo A , et al.   JC virus early protein detection by immunohistochemistry in progressive multifocal leukoencephalopathy: a comparative study with in situ hybridization and polymerase chain reaction . J Neuropathol Exp Neurol . 2004;11:1124–1130
  3. Padgett BL , Walker DL . Prevalence of antibodies in human sera against JC virus, an isolated from a case of progressive multifocal leukoencephalopathy . J Infect Dis . 1973;127:467–470
  4. Tornatore C , Amemiya K , Atwood W , et al.   JC virus-current concepts and controversies in the molecular virology and pathogenesis of progressive multifocal leukoencephalopathy . Rev Med Virol . 1994;4:197–219
  5. Sabath BF , Major EO . Traffic of JC virus from sites of initial infection to the brain: the path to progressive multifocal leukoencephalopathy . J Infect Dis . 2002;186(Suppl 2):S180–S186
  6. Esiri MM , Kennedy PG . Viral diseases . In:  Graham D ,  Lantos P editor. 6th edition. Greenfield's Neuropathology . vol 2: London: Arnold; 1997;p. 42–46
  7. Frosch MP , Anthony DC , De Girolami U . The central nervous system . In:  Robbins SL ,  Cotran RS editor. Pathologic Basis of Disease . 7th edition. Philadelphia: Elsevier; 2004;p. 1376–1377
  8. Aksamit AJ , Sever JL , Major EO . Progressive multifocal leukoencephalopathy: JC virus detection by in situ hybridization compared with immunohistochemistry . Neurology . 1986;36:499–504
  9. Ledoux S , Libman I , Robert F , et al.   Progressive multifocal leukoencephalopathy with gray matter involvement . Can J Neurol Sci . 1989;16:200–202
  10. Mark A , Atlas S . Progressive multifocal leukoencephalopathy in patients with AIDS: appearance on MRI images . Radiology . 1989;173:517–520
  11. Guilleux MH , Steiner RE , Young IR . MR imaging in progressive multifocal leukoencephalopathy . Am J Neuroradiol . 1986;7:1033–1035
  12. Levy JD , Cottingham KL , Campbell RJ , et al.   Progressive multifocal leukoencephalopathy and magnetic resonance imaging . Ann Neurol . 1986;19:399–401
  13. Major EO , Amemiya K , Tornatore CS , et al.   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
  14. Kepes JJ , Chou SM , Price LW . Progressive multifocal leukoencephalopathy with 10 year survival in a patient with nontropical sprue: report of a case with unusual light and electron microscopic features . Neurology . 1975;25:1006–1012
  15. Gray F , Chretien F , Vallat-Decourelaere AV , et al.   The changing pattern of HIV neuropathology in the HAART era . J Neuropathol Exp Neurol . 2003;62:429–440
  16. Roberts MT . AIDS-associated progressive multifocal leukoencephalopathy: current management strategies . CNS Drugs . 2005;19:671–682

PII: S0929-6646(09)60362-4

doi:10.1016/S0929-6646(09)60362-4

Journal of the Formosan Medical Association
Volume 106, Issue 3, Supplement , Pages S24-S28, 2007

Article Tools