Clinical Background
The human neurotropic polyomavirus (JCV) is the etiologic agent of progressive multifocal leukoencephalopathy (PML).
Epidemiology
- Prevalence
- Seroconversion is 90% by age 20
- Establishes lifelong sub-clinical infection in immunocompetent patients
- Presentation of PML is rare
- Age - no age predisposition
- Sex - M>F
Organism
- JC virus is a nonenveloped DNA virus
- BK virus and SV-40 are also members of this viral family
- JC and BK were named using the initials of the first patients discovered with these diseases
Pathophysiology
- Multiple foci of demyelination caused by lytic infection of the oligodendrocytes
Clinical Presentation
- Progressive Multifocal Leukoencephalopathy (PML)
- Fatal demyelinating disease of the central nervous system (CNS)
- Progressive, fatal within 3-6 months
- Almost exclusively in patients with severely impaired immunity
- Considered an AIDS defining illness
- Hemiplegia, visual disturbances and subcortical dementia
- MRI demonstrates demyelinated lesions in the subcortical matter
Treatment
- No known treatment for virus
- Treatment of underlying disease may help slow the progress
- Disease is routinely fatal in neurologically symptomatic patients
See Also
Diagnosis
Diagnosis
- Laboratory testing
- JC DNA, PCR testing in CSF - positive test with appropriate clinical syndrome strongly supports the diagnosis
- Histology
- Brain biopsy - usually diagnostic
- May not be able to perform on debilitated patients
- Imaging studies
- CT/MRI - patchy or confluent hypodense lesions in the white matter
Tests generally appear in the order most useful for common clinical situations
| Test name: JC Virus by PCR
|
| ARUP #: 0099169 |
| Methodology: Polymerase Chain Reaction
|
| Use: Exclude diagnosis of JC virus |
| Limitations: |
| Follow-up:
|
References
General References
Berger JR. Progressive multifocal leukoencephalopathy. Curr Neurol Neurosci Rep.
2007;
7(
6):
461-469.
Doerries K. Human polyomavirus JC and BK persistent infection. Adv Exp Med Biol.
2006;
577:
102-116.
Drachenberg CB, Papadimitriou JC. Polyomavirus-associated nephropathy: update in diagnosis. Transpl Infect Dis.
2006;
8(
2):
68-75.
Eash S, Manley K, Gasparovic M, Querbes W, Atwood WJ. The human polyomaviruses. Cell Mol Life Sci.
2006;
63(
7-8):
865-876.
Hou J, Major E. Management of infections by the human polyomavirus JC: past, present and future. Expert Rev Anti Infect Ther.
2005;
3(
4):
629-640.
Khalili K, Gordon J, White MK. The polyomavirus, JCV and its involvement in human disease. Adv Exp Med Biol.
2006;
577:
274-287.
Khalili K, White MK. Human demyelinating disease and the polyomavirus JCV. Mult Scler.
2006;
12(
2):
133-142.
Koralnik IJ. Progressive multifocal leukoencephalopathy revisited: Has the disease outgrown its name?. Ann Neurol.
2006;
60(
2):
162-173.
Medical Reviewers
Hillyard, David R., M.D. Medical Director, Molecular Infectious Diseases at ARUP Laboratories; Associate Professor, Pathology, University of Utah
Comprehensive Review: May 2008
Last Update: May 2008
