Childhood case of progressive multifocal leukoencephalopathy with improved clinical outcome
Ercan Demir1, Uwe G Liebert, Figen Söylemezoglu
1Department of Pediatric Neurology, Hacettepe University, Ankara, Turkey.
Insights
This case study highlights long-term survival in a child with progressive multifocal leukoencephalopathy (PML) and normal immunity. It shows potential for recovery with treatment, even in rare neurological conditions.
Area of Science:
- Neurology
- Virology
- Pediatric Oncology
Background:
- Progressive multifocal leukoencephalopathy (PML) is a rare, often fatal, demyelinating disease of the central nervous system.
- PML is caused by the JC virus (JCV) and typically affects immunocompromised individuals.
- This case involves a pediatric patient in remission from acute lymphoblastic leukemia (ALL).
Observation:
- A 6-year-old boy, 2.5 years post-ALL remission, presented with neurological symptoms including seizures, hemiparesis, and visual loss.
- Cranial MRI revealed white and gray matter lesions indicative of demyelination.
- Diagnosis was confirmed by detecting JC virus DNA in brain tissue via PCR.
Findings:
- The patient received a combination of anticonvulsants, amantadine, acyclovir, and ganciclovir.
- Partial neurological recovery was observed following treatment.
- This outcome is notable given the patient's normal immunologic parameters.
Implications:
- This case suggests that long-term survival is possible in PML even with normal immune function.
- It underscores the importance of considering PML in pediatric patients with neurological deficits, irrespective of perceived immune status.
- Further research into treatment strategies for PML in immunocompetent individuals may be warranted.
Abstract:
A 6-year-old boy who had been in remission from acute lymphoblastic leukemia for 2.5 years presented with seizures, hemiparesis, visual loss, and white- and gray-matter lesions on cranial magnetic resonance imaging. The diagnosis of progressive multifocal leukoencephalopathy was established on the detection of JC virus DNA by polymerase chain reaction in brain tissue. The patient was administered several anticonvulsants, amantadine, acyclovir, and ganciclovir. He showed partial recovery. This case illustrates the possibility of long-term survival in progressive multifocal leukoencephalopathy with normal immunologic parameters.
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