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Predictive factors for prolonged survival in acquired immunodeficiency syndrome-associated progressive multifocal
J R Berger1, R M Levy, D Flomenhoft
1Department of Neurology, University of Kentucky College of Medicine, Lexington 40536-0284, USA.
Abstract:
Progressive multifocal leukoencephalopathy (PML) complicating the acquired immunodeficiency syndrome (AIDS) is typically inexorably progressive with death usually occurring within 6 months of symptom onset. Occasional patients have been observed to survive longer than 1 year, often with remission of clinical features. In this study, we identify predictive factors for prolonged survival in patients with biopsy proven, AIDS-associated PML, by comparing 7 patients with survival exceeding 12 months from symptom onset with 45 patients with shorter survivals. PML was the presenting manifestation of AIDS in 5 (71.4%) of 7 long-term survivors compared with 8 (17.8%) of 45 short-term survivors. CD4 T-lymphocyte counts were substantially higher in the long-term survivors, with 3 (42.9%) of 7 having counts exceeding 300 cells/mm3 in comparison with only 1 (4.3%) of 23 short-term survivors. Contrast enhancement on radiographic imaging was observed in 3 (50%) of 6 long-term survivors in comparison with 4 (8.9%) of 45 short-term survivors. Neurological recovery and radiographic improvement were not observed in any short-term survivors but were seen in 5 (71.4%) long-term survivors. There was no association between treatment modalities and survival. Predictors of long-term survival in AIDS patients with PML include PML as the heralding manifestation of AIDS, high CD4 T-lymphocyte count at disease onset, lesion enhancement on computed tomographic scan or magnetic resonance imaging, and evidence of recovery of neurological function.
Insights
Predictors of longer survival in AIDS-associated Progressive Multifocal Leukoencephalopathy (PML) include early diagnosis, higher CD4 counts, and radiographic enhancement. Some patients show neurological recovery.
Area of Science:
- Neuroimmunology
- Viral Pathogenesis
Background:
- Acquired immunodeficiency syndrome (AIDS)-associated Progressive Multifocal Leukoencephalopathy (PML) is typically fatal within six months.
- Long-term survival is rare but occurs in some patients, often with clinical remission.
Observation:
- This study compared 7 patients with AIDS-PML surviving over 12 months to 45 with shorter survivals.
- PML as the initial AIDS manifestation, higher CD4 T-lymphocyte counts, and contrast enhancement on imaging were more frequent in long-term survivors.
- Neurological recovery and radiographic improvement were observed only in long-term survivors.
Findings:
- Predictive factors for prolonged survival in AIDS-PML include PML presenting as the first AIDS symptom.
- Higher CD4 T-lymphocyte counts (>300 cells/mm3) at disease onset are associated with better outcomes.
- Radiographic lesion enhancement on CT or MRI and subsequent neurological recovery predict longer survival.
Implications:
- Identifying these predictors can aid in managing AIDS-PML patients and offer prognostic insights.
- Further research into the mechanisms underlying recovery in long-term survivors is warranted.
- These findings may inform clinical trial design and therapeutic strategies for AIDS-PML.