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https://www.arca.fiocruz.br/handle/icict/37549
Tipo de documento
ArtigoDireito Autoral
Acesso aberto
Data de embargo
2020-12-03
Coleções
- INI - Artigos de Periódicos [3466]
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CLINICAL OUTCOME OF LONG-TERM SURVIVORS OF PROGRESSIVE MULTIFOCAL LEUKOENCEPHALOPATHY
Clinical outcome
Long-term survivors
Afiliação
Harvard Medical School. Beth Israel Deaconess Medical Center. Department of Neurology. Division of Viral Pathogenesis. Boston, MA, USA.
Harvard Medical School. Beth Israel Deaconess Medical Center. Department of Neurology. Division of Viral Pathogenesis. Boston, MA, USA.
Washington University. School of Medicine. Departments of Neurology and Medicine. St. Louis, MO, USA.
Massachusetts General Hospital. Infectious Diseases Division. Boston, MA, USA / Harvard Medical School. Boston, MA, USA / Ragon Institute. Cambridge, MA, USA.
Harvard Medical School. Beth Israel Deaconess Medical Center. Department of Neurology. Division of Viral Pathogenesis. Boston, MA, USA.
Harvard Medical School. Beth Israel Deaconess Medical Center. Department of Neurology. Division of Viral Pathogenesis. Boston, MA, USA.
Washington University. School of Medicine. Departments of Neurology and Medicine. St. Louis, MO, USA.
Massachusetts General Hospital. Infectious Diseases Division. Boston, MA, USA / Harvard Medical School. Boston, MA, USA / Ragon Institute. Cambridge, MA, USA.
Harvard Medical School. Beth Israel Deaconess Medical Center. Department of Neurology. Division of Viral Pathogenesis. Boston, MA, USA.
Resumo em Inglês
Progressive Multifocal Leukoencephalopathy (PML) is a demyelinating disease of the brain caused by the polyomavirus JC (JCV) in immunosuppressed people. There is no cure for PML but one-year survival has increased from 10% to 50% in HIV-infected individuals treated with highly active antiretroviral therapy (HAART). We describe herein the clinical outcome of 24 PML patients whose survival exceeded 5 years, with a mean follow-up of 94.2 months (range 60–188 months). Of all patients, only 2 were females including one who had non-Hodgkin’s lymphoma and was HIV-negative. All 23 HIV-positive patients received HAART, and additional
experimental therapies were not associated with a better clinical outcome. Marked neurological improvement occurred in 4/24(17%) of patients, while 11/24 (46%) had partial improvement and 9/24(37%) remained stable. By the end of the period of observation, 8/24(33%) of patients had no significant disability despite persistent symptoms (modified Rankin disability scale (MRDS) =1), 6/24(25%) had slight disability and were living independently (MRDS=2), 5/24(21%) were moderately disabled, requiring some help during activities of daily
living (MRDS=3) and 5/24(21%) had moderately severe disability, requiring constant help or institutionalization (MRDS=4). Patients with cerebellar lesions tended to have a worse clinical outcome. MRI showed leukomalacia with ventricular enlargement secondary to destruction of the white matter at the site of previous PML lesions, and focal areas of subcortical atrophy with preservation of the cortical ribbon. Of 20 patients tested, 19(95%) had detectable CD8+ cytotoxic T-lymphocytes against JCV in their blood. In absence of a specific treatment, immunotherapies aiming at boosting the cellular immune response against JCV may improve the prognosis of PML.
Palavras-chave em inglês
Progressive multifocal leukoencephalopathyClinical outcome
Long-term survivors
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