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Smoldering myeloma presenting as progressive multifocal leukoencephalopathy: a case report
Martina Troppmann1, Roland Büttner, Michael Boewer
1Department of Internal Medicine I, University of Regensburg, Regensburg, Germany. martina.troppmann@klinik.uni-regensburg.de
Introduction:
Progressive multifocal leukoencephalopathy is an opportunistic infection occurring in patients with severe cellular immunodeficiency. This case highlights the role of cellular immunodeficiency in the reactivation of John Cunningham virus in a case of an early stage plasmacytoma.
Case Presentation:
A 76-year-old Caucasian woman presented with progressive left-sided hemiparesis, accompanied by hypoesthesia, hypoalgesia and neuropsychological symptoms. Magnetic resonance imaging demonstrated new hyperattenuating lesions in the right thalamus and left-sided subcortically. A polymerase chain reaction test revealed 4500 copies of John Cunningham virus-deoxyribonucleic acid/ml in cerebrospinal fluid. Human immunodeficiency virus infection was ruled out. A bone marrow biopsy showed an early stage immunoglobulin G-kappa plasmacytoma. Cidofovir (5mg/kg) weekly for three weeks was started. A significant improvement of her neuropsychological symptoms was achieved, but motor system and sensory symptoms did not change.
Conclusions:
This case shows a rapid course of progressive multifocal leukoencephalopathy with severe residual deficits. In the diagnostic workup of all patients with atypical neurologic symptoms or immunodeficiency, progressive multifocal leukoencephalopathy should be included as a differential diagnosis.
Insights
This case study shows progressive multifocal leukoencephalopathy (PML) reactivation due to John Cunningham virus (JCV) in an early plasmacytoma patient. Early diagnosis of PML is crucial for patients with atypical neurological symptoms or immunodeficiency.
Area of Science:
- Neurology
- Infectious Diseases
- Oncology
Background:
- Progressive multifocal leukoencephalopathy (PML) is an opportunistic infection linked to severe cellular immunodeficiency.
- John Cunningham virus (JCV) reactivation can occur in immunocompromised individuals.
- This case involves JCV reactivation in a patient with early-stage plasmacytoma.
Purpose of the Study:
- To present a case of PML in a patient with early-stage plasmacytoma.
- To highlight the role of cellular immunodeficiency in JCV reactivation.
- To emphasize PML as a differential diagnosis in patients with neurological symptoms or immunodeficiency.
Main Methods:
- A 76-year-old woman presented with hemiparesis and neurological deficits.
- MRI revealed hyperattenuating lesions; PCR confirmed high JCV DNA levels in CSF.
- Bone marrow biopsy diagnosed early-stage immunoglobulin G-kappa plasmacytoma.
Main Results:
- Treatment with cidofovir led to significant improvement in neuropsychological symptoms.
- Motor and sensory deficits showed no improvement.
- The case demonstrated a rapid course of PML with severe residual deficits.
Conclusions:
- PML should be considered in the differential diagnosis of patients with atypical neurological symptoms or immunodeficiency.
- Cellular immunodeficiency plays a critical role in JCV reactivation.
- Prompt diagnosis and management are essential for patients presenting with neurological decline and underlying immunodeficiency.
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