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Progressive multifocal leukoencephalopathy in an HIV-infected child

Z Liptai1, E Papp, P Barsi

  • 1Department of Pediatrics, Szent László Hospital for Infectious Diseases, Budapest, Hungary. zliptai@laszlokorhaz.hu

Neuropediatrics
|July 4, 2007
PubMed

Insights

Progressive multifocal leukoencephalopathy (PML) is a rare JC virus infection in children. This case highlights a unique presentation in a young HIV-infected boy, emphasizing the need for early diagnosis and treatment in immunocompromised individuals.

Area of Science:

  • Neurology
  • Infectious Diseases
  • Virology

Background:

  • Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease caused by the JC virus, primarily affecting immunocompromised individuals, particularly those with advanced HIV/AIDS.
  • PML is exceptionally uncommon in pediatric populations, making pediatric cases noteworthy for understanding disease presentation and progression.

Observation:

  • A 15-year-old HIV-infected male presented with progressive neurological symptoms including fatigue, apathy, head tilt, diplopia, motor apraxia, and unsteady gait.
  • Cerebrospinal fluid analysis confirmed the presence of JC virus DNA, and Magnetic Resonance Imaging (MRI) revealed a large, non-enhancing lesion in the right cerebellar hemisphere and middle cerebellar peduncle.

Findings:

  • The patient's condition rapidly deteriorated, with MRI demonstrating lesion progression within two weeks.
  • Autopsy confirmed widespread PML lesions in the cerebellar hemispheres and brainstem, consistent with the JC virus infection.

Implications:

  • This case underscores the possibility of PML in young, HIV-infected patients, even with atypical lesion localization and unifocal onset.
  • Early diagnosis through MRI and JC virus DNA detection in CSF is crucial for initiating timely management, primarily immune reconstitution therapy.
  • The unusual presentation in this pediatric case contributes to the understanding of JC virus pathogenesis and PML in the context of pediatric HIV infection.

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