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Progressive multifocal leukoencephalopathy in the United States, 1979-1994: increased mortality associated with HIV
R C Holman1, T J Török, E D Belay
1Division of Viral and Rickettsial Diseases, National Center for Infectious Diseases, Atlanta, Ga., USA. rch1@cdc.gov
Abstract:
To examine trends in progressive multifocal leukoencephalopathy (PML) mortality in the United States, we analyzed PML death rates and deaths for 1979 through 1994, using US multiple cause-of-death data. During the 16-year study period 3,894 PML deaths were reported. The age-adjusted death rate increased more than 20-fold, from less than 0.2 per million persons before 1984 to 3.3 per million persons in 1994. The increase was attributable to infection with human immunodeficiency virus (HIV) which was recorded on 2,267 (89.0%) of 2.546 death records from 1991 through 1994. PML age-adjusted death rates increased abruptly for all males beginning in 1984 and for black females in 1990. Only a small increase was observed for white females. In 1994, PML was reported in 2.1% of white males who died with HIV-associated disease compared with 1.2% of white females and 1.0% of black males and females who died of similar causes. The epidemic of PML deaths is increasing in parallel with the AIDS epidemic. The increase in HIV-associated PML deaths, first noted among males, has also become apparent among females and probably reflects the increasing importance of drug use and heterosexual transmission of HIV. The reason for the higher prevalence of PML among white males with HIV infection is unknown.
Insights
Progressive multifocal leukoencephalopathy (PML) mortality surged over 20-fold from 1979-1994, primarily due to human immunodeficiency virus (HIV). This trend mirrors the AIDS epidemic, with increasing deaths observed in both males and females.
Area of Science:
- Neurology
- Epidemiology
- Infectious Diseases
Background:
- Progressive multifocal leukoencephalopathy (PML) is a rare, fatal demyelinating disease of the central nervous system.
- PML is strongly associated with immunosuppression, particularly in individuals with human immunodeficiency virus (HIV) infection.
Purpose of the Study:
- To analyze trends in mortality rates for PML in the United States from 1979 to 1994.
- To investigate the association between PML deaths and HIV infection over the study period.
Main Methods:
- Utilized US multiple cause-of-death data from 1979 to 1994.
- Analyzed age-adjusted death rates and the number of reported PML deaths.
- Examined the co-occurrence of PML and HIV on death records.
Main Results:
- PML deaths increased dramatically, with age-adjusted rates rising over 20-fold from <0.2 to 3.3 per million persons between 1979-1994.
- HIV was listed as a cause in 89% of PML deaths from 1991-1994, indicating a strong link.
- Mortality rates increased significantly for males starting in 1984 and for black females in 1990, while white females showed a smaller increase.
- In 1994, PML was more prevalent in white males dying with HIV-associated disease compared to other demographic groups.
Conclusions:
- The epidemic of PML deaths closely parallels the AIDS epidemic in the US.
- The rise in HIV-associated PML reflects increasing HIV transmission through drug use and heterosexual contact.
- The higher prevalence of PML among white males with HIV warrants further investigation.