Progressive Multifocal Leukoencephalopathy in HIV-Infected Children: A Case Report and Literature Review

Abstract

We report a case of a perinatally HIV-infected patient aged 9 years, who presented with right-sided hemiplegia. His initial CD4 T-cell was of 0.21% (4 cells/μL) and plasma HIV RNA virus of 185 976 copies/mL (log 5.27). Plasma and CSF samples were subsequently positive for JCV. Twelve days after the initiation of highly active antiretroviral therapy (HAART), the MRI showed progressive white matter lesions with asymmetrical deep and subcortical white matter lesions over the left frontotemporoparietal region and the right frontal lobe. Immune Reconstitution Inflammatory Syndrome (IRIS) was suspected, and the patient was treated with methylprednisolone. His clinical symptoms worsened and despite therapy the patient deteriorated.

Progressive Multifocal Leukoencephalopathy in an Apparently Healthy Child: An Unsuspected Diagnosis

“…As such, it is a rare scenario for PML to be the initial manifestation of HIV, and exceedingly rare in a child. Only 3 other case reports describe pediatric patients presenting with PML and undiagnosed HIV infection [3,4,5]. …”

Progressive Multifocal Leukoencephalopathy in Pediatric Patients

“…Only 3/19 (16%) of patients were newly-diagnosed with HIV at the time of PML presentation. 6, 12, 18 CD4 count was <500 cells/mm 3 in all but one patient. Viral load was reported in 5 cases and ranged from 11,800-292,000 copies/mL.…”

“…45, 46 The effects of antiretroviral drugs on PML outcomes in children are unknown; only 7/19 (37%) of published cases initiated cART at the time of their PML diagnosis. 11-16, 18 …”

Guidelines for the Prevention and Treatment of Opportunistic Infections in HIV-Exposed and HIV-Infected Children

“…It has rarely been seen in reports from large series of HIV-infected children, 13–15 but cases have been reported in children with a wide range of ages and a broad geographical distribution. 16–22 The incidence of PML has decreased from 3.3 cases per 1000 person-years at risk during the era before cART, to 1.3 cases per 1000 person-years after the introduction of cART. 23 During the pre-cART era, survival was extremely poor in adults and children with PML.…”

“…Inflammatory PML should be suspected in cART-treated children with advanced HIV who show acute neurologic deterioration and contrast-enhancing demyelinating lesions on MRI, even if immunological and virological measures show improvement in HIV status. 22 Retrospective data suggest that early and prolonged treatment with steroids may be beneficial for some patients in whom immune reconstitution with ART activates an inflammatory response to JCV. No clinical trial data exist, however, to substantiate the anecdotal evidence.…”