Progressive Multifocal Leukoencephalopathy in Patients with HIV Infection

Abstract

Progressive multifocal leukoencephalopathy (PML), a formerly rare disease, is estimated to occur in up to 5% of all patients with AIDS. The high prevalence of PML in AIDS patients currently enables a comprehensive evaluation of this disorder. We evaluated the clinical and radiographic features of PML in a large cohort of AIDS patients identified by retrospective chart review from 1981 to 1994. Two hundred and five patients were diagnosed with PML of which 154 met the inclusion criteria. Seventy-two (47%) were pathologically confirmed and the remaining 82 (53%) met clinical and radiographic criteria. There was a 12-fold increase in the frequency of PML between 1981-1984 and 1991-1994. PML affected 136 men and 18 women with AIDS. Eighty-four percent of cases were 20-50 years old (range 5 to 68 years). The most common AIDS risk factors were homosexuality (57%) among men and heterosexual transmission (28%) and intravenous drug abuse (28%) among women. In 27% of patients, PML heralded AIDS. Common manifestations included weakness, gait abnormalities, speech disturbance, cognitive disorders, headache, and visual impairment. The CD4 lymphocyte counts exceeded 200 cells in 11% at the time of presentation. Involvement of posterior fossa structures was evident in 48% of cranial magnetic resonance imaging (MRI) studies, but in only 11% of computed tomographies (CT) of the brain. Contrast enhancement, typically faint and peripheral, was seen in 10% of CT scans and 15% of MRIs. The median survival was 6 months and survival exceeded 1 year in 9%. PML is no longer a rare disease. It often heralds AIDS and may occur in the absence of significant decline in CD4 lymphocytes. Survival is generally poor, although prolonged survival beyond 1 year is not unusual.

Effective use of JC virus PCR for diagnosis of progressive multifocal leukoencephalopathy

“…Our data are consistent with previous observations of the rarity of PML in HIV-negative patients (Berger et al, 1998). However, surprisingly, JCV PCR was positive in only 24 % of patients with radiology findings suggestive of PML (Table 1).…”

Progressive multifocal leukoencephalopathy in rheumatic diseases: Evolving clinical and pathologic patterns of disease

“…In PML, findings of cerebrospinal fluid (CSF) examination are typically unremarkable; in 1 large study (72), the median white blood cell count was 2 cells/l and the mean was 7.7 cells/l. CSF protein levels are normal in ϳ50% of patients.…”

Clinical and Radiological Characterization of Progressive Multifocal Leukoencephalopathy in HIV-Infected Patients: A Retrospective Analysis and Review of the Literature

“…77 Most studies show that the incidence of HIV-associated PML has declined and the prognosis of affected patients has improved after the introduction of HAART. One-year survival rates in HIV patients with PML increased from less than 30% in the pre-HAART era to 38% -62% with HAART.…”