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Progressive multifocal leukoencephalopathy in HIV-1-infected children
J R Berger1, G Scott, J Albrecht
1Department of Neurology, University of Miami School of Medicine, Florida 33136.
Objective:
To describe the clinical and pathologic features of two HIV-1-infected children with progressive multifocal leukoencephalopathy (PML).
Design:
Case report.
Setting:
University-affiliated, public-health trust hospital.
Methods:
Two HIV-1-infected children with PML are described. A 13-year-old girl, presumed to be congenitally infected with HIV-1, presented with dysarthria and paresthesias of the tongue and chin that evolved rapidly to dementia, muteness and severe spastic quadriparesis. The other patient, a 10-year-old boy who developed HIV-1 infection from a blood transfusion at the age of 3 years, presented with a facial palsy with subsequent development of right hemiparesis and aphasia.
Results:
Brain biopsy in the first child and autopsy in the second confirmed the diagnosis of PML. In both patients, the CD4 T-lymphocyte count was less than 100 x 10(6)l at the time of neurological presentation.
Conclusion:
Despite seroepidemiological studies suggesting that the majority of individuals are infected with JC virus during childhood, PML is rare in children with impaired cell-mediated immunity. Our patients illustrate that PML is among the neurological complications of HIV-1 infection in children.