Clinical outcome of long-term survivors of progressive multifocal leukoencephalopathy

dc.creatorLima, Marco A.
dc.creatorBernal-Cano, Francisco
dc.creatorClifford, David B.
dc.creatorGandhi, Rajesh
dc.creatorKoralnik, Igor J.
dc.date2019-12-03T15:07:36Z
dc.date2019-12-03T15:07:36Z
dc.date2010
dc.date.accessioned2023-09-26T20:58:32Z
dc.date.available2023-09-26T20:58:32Z
dc.identifierLIMA, Marco A. et al. Clinical outcome of long-term survivors of progressive multifocal leukoencephalopathy. Journal of Neurology, Neurosurgery and Psychiatry, v. 81, n. 11, p. 1288-1291, Nov. 2010.
dc.identifier0022-3050
dc.identifierhttps://www.arca.fiocruz.br/handle/icict/37549
dc.identifier10.1136/jnnp.2009.179002
dc.identifier1468-330X
dc.identifier.urihttps://repositorioslatinoamericanos.uchile.cl/handle/2250/8867106
dc.descriptionMarco A. Lima. Fundação Oswaldo Cruz. Instituto Nacional de Infectologia Evandro Chagas. Documento produzido em parceria ou por autor vinculado à Fiocruz, mas não consta a informação no documento.
dc.descriptionProgressive 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.
dc.description2020-12-03
dc.formatapplication/pdf
dc.languageeng
dc.publisherBMJ Publishing Group
dc.rightsopen access
dc.subjectProgressive multifocal leukoencephalopathy
dc.subjectClinical outcome
dc.subjectLong-term survivors
dc.titleClinical outcome of long-term survivors of progressive multifocal leukoencephalopathy
dc.typeArticle


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