Progressive multifocal leukoencephalopathy in a child with acquired immunodeficiency syndrome (AIDS)

A P Araujo1, H S Pereira, R H Oliveira

  • 1Department of Pediatrics, Universidade Federal do Rio de Janeiro, Brazil.

Insights

Progressive multifocal leukoencephalopathy (PML) is a rare viral disease affecting the brain, often seen in individuals with immunodeficiency. This case highlights PML in a child with AIDS presenting with neurological decline, emphasizing the need for early diagnosis.

Area of Science:

  • Neurology
  • Infectious Diseases
  • Immunology

Background:

  • Progressive multifocal leukoencephalopathy (PML) is a rare, opportunistic infection of the central nervous system.
  • PML is caused by the JC virus (JCV) and primarily affects individuals with compromised immune systems, such as those with Acquired Immunodeficiency Syndrome (AIDS).
  • Demyelination in the brain's white matter is a hallmark of PML, leading to severe neurological deficits.

Observation:

  • A 10-year-old boy diagnosed with AIDS presented with subacute onset of cerebellar dysfunction, cranial nerve palsies, and cognitive decline.
  • Neuroimaging revealed characteristic findings: a focal, non-enhancing hypodense lesion on CT scan and T2 hyperintense lesions on MRI in the cerebellum and white matter.
  • Brain biopsy confirmed the diagnosis by showing typical histopathological features of PML.

Findings:

  • The case demonstrates a rare presentation of PML in a pediatric patient with advanced HIV/AIDS.
  • Cerebellar involvement and multiple cranial nerve deficits were prominent clinical features.
  • Histological confirmation solidified the diagnosis, identifying characteristic viral inclusions and demyelination.

Implications:

  • This case underscores the importance of considering PML in the differential diagnosis of children with AIDS experiencing progressive neurological symptoms.
  • Early recognition and diagnosis of PML in immunocompromised children are crucial for timely management and potentially improving outcomes.
  • Further research into the specific clinical and radiological manifestations of PML in pediatric populations with HIV is warranted.

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