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PubMed1999

Progressive multifocal leukoencephalopathy: unusual MRI findings and prolonged survival in a pregnant woman.

We report a biopsy-proven case of progressive multifocal leukoencephalopathy (PML) in a pregnant woman without obvious underlying immune disorder. MRI showed lesion enhancement and deep gray matter involvement, which are uncommon imaging patterns in PML. The clinical course in this case is also rather atypical, as the patient is alive 16 months after disease onset. The combination of these unusual characteristics in PML is unknown and indicates that the criteria for this disease may need to be redefined.

Adult↗
PubMed1975

Progressive multifocal leukoencephalopathy with 10-year survival in a patient with nontropical sprue. Report of a case with unusual light and electron microscopic features.

A 46-year-old man with nontropical sprue had anemia and hypoproteinemia for several years, until his condition was diagnosed and treated with dietary measures. Within a year after the diagnosis, progressive multifocal leukoencephalopathy developed, and the patient had a slightly fluctuating chronic downhill course until he died 10 years later. It is postulated that this patient's immune deficiency was related to his malabsorption syndrome and hypoglobulinemia, and the course became unusually protracted (longest reported course in the American literature) because of restoration of plasma protein levels. Autopsy showed the classic findings of progressive multifocal leukoencephalopathy, with much tissue loss of subcortical white matter and active perivascular inflammatory foci with numerous eosinophilic granulocytes. On electron microscopy, oligodendrocyte nuclei and cytoplasm were crowded with virions, but many myelin sheaths invested by severely infected oligodendrocytic processes were remarkably well preserved. This fact would argue against a direct cause-and-effect relationship between infection of oligodendrocytes and myelin breakdown in progressive multifocal leukoencephalopathy. The likelihood of an autoimmune mechanism at work in this disease is suggested, and the role of eosinophils and other cells in such process is considered.

Astrocytes↗
PubMed2005

Reduced rate of diagnostic positive detection of JC virus DNA in cerebrospinal fluid in cases of suspected progressive multifocal leukoencephalopathy in the era of potent antiretroviral therapy.

Fifty-nine human immunodeficiency virus (HIV)-infected patients with suspected progressive multifocal leukoencephalopathy and 224 controls were tested for JC virus (JCV) DNA in cerebrospinal fluid by PCR. The diagnostic positive detection rate dropped from 89.5% (95% confidence intervals of 75.5 to 103.5%) in the pre-highly active antiretroviral therapy (HAART) era to 57.5% (95% confidence intervals of 42.1 to 72.9%) in the HAART era; the specificity remained unchanged. Predictors of failure to detect JCV DNA were exposure to HAART at disease onset and higher CD4 counts.

Adult↗
PubMed1991

Amplification of JC virus DNA from brain and cerebrospinal fluid of patients with progressive multifocal leukoencephalopathy.

We have established an assay to detect JC viral DNA in brain tissue and CSF of patients with progressive multifocal leukoencephalopathy (PML) and amplified a 106-base pair segment of the gene encoding JC viral large T antigen by polymerase chain reaction (PCR) from the brains of eight patients with PML and from one of six CSF samples. Brain and CSF of patients without PML did not yield JC virus-specific DNA amplification products. Southern analysis documented that the sequences of the 106-base pair PCR product and JC viral DNA are identical. This assay can be used in the diagnosis of PML and to investigate the biology of JC virus.

Base Sequence↗
PubMed1983

Virologic and serologic studies of progressive multifocal leukoencephalopathy.

The human polyomavirus JCV has been associated with diseased brain tissue from 54 cases of PML. The disease occurred in persons of all ages--the youngest being 5 years old--whose immune mechanisms were depressed for any of a variety of reasons. In six patients the disease ceased progressing and they stabilized or improved. The brains of two of these, who died months or years later, were found to be free of active PML at autopsy. Sera from 21 patients had HAI antibodies against JCV, but none had antibodies against T or the common internal antigens, and only one had IgM antibodies detectable by immunofluorescent staining. Nine CSF were devoid of JCV-related antibodies except for one which gave an HAI titer of 8. Virus has been isolated from 15 PML brains, and preliminary studies indicate that one isolate, while clearly of JCV type, is serologically distinguishable from prototype Mad 1 JCV.

Adolescent↗