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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.
Abstract:
Progressive multifocal leukoencephalopathy is a rare, demyelinating disease of the central nervous system caused by JC virus. Fewer than 30 cases have been reported in HIV- and non-infected children. We report the case of a 15-year-old girl with progressive multifocal leukoencephalopathy and AIDS who presented with nystagmus, dysarthria and ataxia. Following combined antiretroviral therapy, she developed immune reconstitution inflammatory syndrome, which proved fatal.
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