Progressive multifocal leukoencephalopathy in pediatric patients: case report and literature review

Hayden Schwenk1, Lynn Ramirez-Avila, Shu-Hsien Sheu

  • 1From the *Department of Pediatrics, Division of Infectious Diseases, Stanford University School of Medicine, Stanford, CA; †Division of Infectious Diseases, Boston Children's Hospital, Boston, MA; ‡Division of Pediatric Infectious Diseases, David Geffen School of Medicine at UCLA, Mattel Children's Hospital, Los Angeles, CA; §Department of Pathology, Brigham and Women's Hospital; ¶Department of Neurology, Beth Israel Deaconess Medical Center; ‖Department of Neurology, Boston Children's Hospital, Boston, MA; **Department of Pediatrics, Stanford University School of Medicine, Stanford, CA; ††Department of Pathology, Boston Children's Hospital, Boston, MA.

Insights

Progressive multifocal leukoencephalopathy (PML) is a rare JC virus-induced CNS demyelinating disease. This case details a fatal pediatric AIDS case complicated by immune reconstitution inflammatory syndrome after antiretroviral therapy.

Area of Science:

  • Neurology
  • Virology
  • Immunology

Background:

  • Progressive multifocal leukoencephalopathy (PML) is a rare, demyelinating central nervous system (CNS) disease.
  • PML is caused by the JC virus (JCV) and is infrequently reported in children, with fewer than 30 cases documented in both HIV-infected and non-infected pediatric populations.

Observation:

  • A 15-year-old female patient with acquired immunodeficiency syndrome (AIDS) presented with neurological symptoms including nystagmus, dysarthria, and ataxia.
  • These symptoms were indicative of a demyelinating process affecting the CNS.

Findings:

  • The patient was diagnosed with progressive multifocal leukoencephalopathy (PML) in the context of advanced HIV infection.
  • Initiation of combined antiretroviral therapy (cART) led to immune reconstitution inflammatory syndrome (IRIS).

Implications:

  • This case highlights the rare occurrence and severe presentation of PML in pediatric AIDS.
  • The development of fatal IRIS following cART underscores the complex interplay between JCV infection, immune status, and therapeutic interventions in this population.
  • Further research into managing IRIS in pediatric PML cases is warranted.

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