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PubMed2001

Treatment of non-AIDS progressive multifocal leukoencephalopathy with cytosine arabinoside.

This open label study determined the outcome of non-AIDS progressive multifocal leukoencephalopathy patients treated with a standard dose of intravenous cytosine arabinoside. Nineteen patients with PML proven by brain biopsy or spinal fluid polymerase chain reaction were treated with intravenous cytosine arabinoside 2 mg/kg per day for 5 days and followed for neurologic outcome by neurologic examination and MRI scanning. Seven of 19 PML patients treated with cytosine arabinoside intravenously improved neurologically. The range of follow-up for these patients was 2.0 to 4.5 years. All were left with neurologic deficits but were functionally improved, and 6 of 7 were able to independently carry out the activities of daily living. Twelve PML patients showed no evidence of response and died rapidly of their disease after treatment (range, 8 days to 6 months). All who survived their neurologic disease recovered from treatment-induced pancytopenia. Cytosine arabinoside given intravenously to non-AIDS PML patients in this small study was associated with a 36% chance of developing stabilization at 1 year. Treatment was associated with significant bone marrow toxicity. The improvement in MRI scan changes in those patients who responded took 6 weeks or longer.

Acquired Immunodeficiency Syndrome↗

PubMed2001

JC virus regulatory region rearrangements in the brain of a long surviving patient with progressive multifocal leukoencephalopathy.

JC virus (JCV) infection of oligodendrocytes causes demyelination in brains of patients with with progressive multifocal leukoencephalopathy (PML). Expansion of demyelination throughout the brain is not fully understood. The opportunity was taken to investigate the postmortem brain of a long surviving patient with PML for whom diagnosis was made 4 years before death based on pathological and virological findings of a brain biopsy. Four distinct regulatory sequences in the JCV genome were detected (designated as JW-1 to 4) from various regions of the necropsied brain. All regulatory sequences were rearranged forms that could be produced from the archetype by deletions and duplications. JW-1 and 2 shared some structural features not present in JW-3 and 4 and vice versa. JW-1 was distributed throughout the brain, whereas JW-2, 3, and 4 were restricted to only part of the brain. JW-1 and 2 had been detected in the initial brain biopsy 4 years earlier. These findings suggested that brain lesions in advanced stages were generated not only by expansion of the original variant (JW-1) of JCV but also by delayed growth of two other variants (JW-3 and 4).

Adult↗

PubMed2004

[Neuroradiological study of progressive multifocal leukoencephalopathy associated with acquired immunodeficiency syndrome by MR imaging including diffusion-weighted imaging].

A 27-year-old man presented with subacute progressive left hemiparesis and hemi-sensory loss. Laboratory examinations revealed CD4 count at 14/microl, the HIV antibody positive, and HIV-RNA at 4.3 x 10(4) copies. Brain CT showed hypodence areas without contrast enhancement or mass effect in the right parietooccipital white matter and the right external capsule. On MR imaging, the lesion in the parietooccipital area showed hypointensity without enhancement on T1-weighted images and hyperintensity on T2-weighted images. On FLAIR images, the lesion showed hypointensity surrounded by hyperintensity. Meanwhile, diffusion-weighted images revealed three layers of different intensity. The area that demonstrated hypointensity on FLAIR images showed also hypointensity, while that in the area that demonstrated hyperintensity on FLAIR was divided into two parts; an area of isointense that encircles an hypointense area and a hyperintense area in the margin. The isointense and hypointense areas on diffusion-weighted images showed hyperintensity on apparent diffusion coefficient map. As JCV DNA was detected from his CSF, the diagnosis of PML was made. Based on comparative assessment of these MR images, it was speculated that the PML lesion appeared to be constituted of three parts from the margin to the center: cytotoxic edema, vasogenic edema, and liquefied lesion such as necrosis.

Acquired Immunodeficiency Syndrome↗

PubMed2001

Progressive multifocal leukoencephalopathy in a child with hyperimmunoglobulin E recurrent infection syndrome and review of the literature.

Progressive multifocal leukoencephalopathy (PML) is a fatal demyelinating disease due to infection with polyomavirus JC (JCV). PML occurs almost exclusively in immunocompromised patients, and although it has increased markedly in relation to AIDS, remains exceptional in children. We present the case of an immunocompromised child with hyperimmunoglobulin E recurrent infection syndrome (HIES) and pathologically-proven PML. HIES is a rare congenital immunodeficiency that to our knowledge has never before been reported in association with neurological complications. Following a recurrence of bronchopneumonia, the child's motor and cognitive functions deteriorated progressively in parallel with alterations on cerebral MRI. The neurological onset coincided with lymphocyte subset changes. PCR for JCV DNA did not detect the virus in CSF, and brain biopsy was required to secure the diagnosis. Antiviral treatment with cidofovir produced no benefit. Autopsy revealed the typical neuropathological findings of PML which were associated with inflammatory eosinophilic infiltrate (a marker of HIES). In accordance with the few pediatric PML cases reported and here reviewed, the child died five months after neurological onset.

Brain↗

PubMed1991

Epidemiology of progressive multifocal leukoencephalopathy in the United States: analysis of national mortality and AIDS surveillance data.

We analyzed progressive multifocal leukoencephalopathy (PML) mortality data from 1979 to 1987 and data on persons with acquired immunodeficiency syndrome (AIDS) reported to the Centers for Disease Control (CDC). Based on analyses of multiple-cause-of-death vital statistics, deaths related to PML have increased fourfold from 1.5/10,000,000 persons in 1979 to 6.1/10,000,000 persons in 1987. The increase in the PML annual death rate began in 1984, occurred primarily in men 20 to 49 years of age, and was greatest in states known to have a high incidence of AIDS. In 1987, 56% of death certificates that listed PML as a cause of death also listed human immunodeficiency virus (HIV) infection. Analysis of AIDS case reports to the CDC from 1981 through June 1990 demonstrated that 0.72% of persons with AIDS were reported as having PML. Although most persons with AIDS who had PML were 20 to 49 years of age (84.6%), PML was reported more frequently among persons with AIDS greater than or equal to 50 years old than less than 50 years old. In addition, PML was reported more frequently among persons with AIDS who were exposed to HIV by blood transfusion than those in all other exposure categories. These data demonstrate that the increase in PML mortality from 1979 to 1987 was associated with the large increase in immunosuppressed persons with AIDS.

Acquired Immunodeficiency Syndrome↗

PubMed1995