Volume 106, Issue 2, Supplement , Pages S60-S64, 2007
Progressive Multifocal Leukoencephalopathy in an Immunocompetent Taiwanese Patient
Article Outline
Progressive multifocal leukoencephalopathy (PML) is a deadly demyelinating brain disease caused by JC virus (JCV). Genomic analysis of viral isolates in these cases often shows prototype-like JCV and its variants, which is a virulent strain compared to the latent archetype virions mostly found in the kidney. Here, we report a 57-year-old man who suffered from a subacute course of cognitive impairment and multiple neurologic deficits. Neuroimaging, pathology, and virology studies showed multifocal leukoencephalopathy and the presence of JCV deoxyribonucleic acid in the cerebrospinal fluid. The prototype type 1 (Mad-1) strain of JCV was identified on viral genotyping obtained from brain tissue. No immune deficits were found. He responded poorly to a-interferon and antiviral treatment. This case suggests that lack of immune deficiency cannot exclude the possibility of PML as a cause of subacute leukoencephalopathy. Accumulated data with respect to the disease course, pathologic feature, and viral genomic subtyping may pave the way for future treatment against this devastating disease. [J Formos Med Assoc 2007;106(2 Suppl):S60-S64]
Key Words: genotyping , JC virus , magnetic resonance imaging , polymerase chain reaction , progressive multifocal leukoencephalopathy
No full text is available. To read the body of this article, please view the PDF online.
References
- . Human polyomavirus JC virus genome . J Virol . 1984;51:458–469
- . Polyomavirus-induced demyelination . In: Dangond F editors. Disorders of Myelin in the Central and Peripheral Nervous System . Woburn: Butterworth-Heinemann; 2002;p. 293–310
- . Prevalence of antibodies in human sera against JC virus, an isolate from a case of progressive multifocal leukoencephalopathy . J Infect Dis . 1973;127:467–470
- . Traffic of JC virus from sites of initial infection to the brain: the path to progressive multifocal leukoencephalopathy . J Infect Dis . 2002;186:S180–S186
- Different genotypes of human polyomaviruses found in patients with autoimmune diseases in Taiwan . J Med Virol . 1996;48:204–209
- Treatment with cytotoxic immunosuppression agents increases urinary excretion of JCV in patients with autoimmune disease . J Med Virol . 2000;62:505–510
- . Evidence for polyomaviruses in human neurological diseases . Prog Clin Biol Res . 1983;105:183–190
- . An overview: human polyomavirus JC virus and its associated disorders . J Neurovirol . 2003;9(Suppl 1):3–9
- Cultivation of papova-like virus from human brain with progressive multifocal leucoencephalopathy . Lancet . 1971;1:1257–1260
- JC virus regulatory region rearrangements and genotypes in progressive multifocal leukoencephalopathy: two independent aspects of virus variation . J Gen Virol . 1997;78:659–664
- . JC virus regulatory region tandem repeats in plasma and central nervous system isolates correlate with poor clinical outcome in patients with progressive multifocal leukoencephalopathy . J Virol . 2001;75:5672–5676
- Detection of JC virus DNA by polymerase chain reaction in patients with progressive multifocal leukoencephalopathy . J Infect Dis . 1990;162:858–861
- . Advances in the biology of JC virus and induction of progressive multifocal leukoencephalopathy . J Neurovirol . 2003;9:236–246
- . A classification scheme for human polyomavirus JCV variants based on the nucleotide sequence of the noncoding regulatory region . J Neurovirol . 2001;7:280–287
- Autopsy case of the cerebellar form of progressive multifocal leukoencephalopathy without immunodeficiency . Neuropathology . 2002;22:48–56
- Diagnosis of AIDS-related focal brain lesions: a decision-making analysis based on clinical and neuroradiologic characteristics combined with polymerase chain reaction assays in CSF . Neurology . 1997;48:687–694
- . Human fetal astrocytes in culture support the growth of the neurotropic human polyomavirus, JCV . J Neuropathol Exp Neurol . 1989;48:425–436
- Detection of JC virus by anti-VP1 immunohistochemistry in brains with progressive multifocal leukoencephalopathy . Acta Neuropathol (Berl) . 1997;94:226–231
- JCV-DNA and BKV-DNA in the CNS tissue and CSF of AIDS patients and normal subjects. Study of 41 cases and review of the literature . J Acquir Immune Defic Syndr Hum Retrovirol . 1996;12:139–146
- JC virus DNA in cerebrospinal fluid of human immunodeficiency virus-infected patients: predictive value for progressive multifocal leukoencephalopathy . Ann Neurol . 1995;37:395–399
- Progressive multifocal leukoencephalopathy diagnosed by amplification of JC virus-specific DNA from cerebrospinal fluid . AIDS . 1994;8:49–57
PII: S0929-6646(09)60355-7
doi:10.1016/S0929-6646(09)60355-7
© 2007 Formosan Medical Association & Elsevier. Published by Elsevier Inc. All rights reserved.
Volume 106, Issue 2, Supplement , Pages S60-S64, 2007
