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Published on: September 1, 2018
Progressive multifocal leukoencephalopathy in transplant recipients
David Shitrit1, Nirit Lev, Ariella Bar-Gil-Shitrit
1Pulmonary Institute, Rabin Medical Center, Beilinson Campus, 49100, Petah Tiqwa, Israel. davariel@zahav.net.il
Abstract:
Progressive multifocal leukoencephalopathy (PML) is a demyelinating infection caused by the JC virus. It is an emerging disease in transplant recipients; however, it remains poorly defined. Twenty-four cases of PML reported in the literature in transplant recipients were reviewed. Of the 24 cases, nine occurred in renal, six in bone marrow, four in liver, three in heart and two in lung transplant recipients. Median time to onset was 17 months; 71% occurred within 24 months of transplantation. PML tended to occur later in the kidney recipients (P=0.04). Seventy-five percent had subacute presentation; hemiparesis (50%), apathy (46%) and confusion (38%) were the most frequently presented features. Treatment included reduction of immunosuppression and chemotherapy, mainly cidofovir. Death occurred within 2.5 months of the onset of symptoms in 17 patients (71%). PML is a unique entity that should be considered in any transplant recipient with neurological symptoms. The outcome is usually fatal, although regression has been reported.
Insights
Progressive multifocal leukoencephalopathy (PML) is a rare JC virus infection in transplant recipients. This review highlights its late onset, common neurological symptoms, and typically fatal outcomes, emphasizing the need for clinical consideration.
Area of Science:
- Neurology
- Infectious Diseases
- Transplantation Immunology
Background:
- Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease caused by the JC virus.
- PML is an emerging opportunistic infection in organ and bone marrow transplant recipients.
- The clinical characteristics and outcomes of PML in transplant populations are not well-defined.
Purpose of the Study:
- To review and define the characteristics of PML in transplant recipients.
- To analyze the incidence, timing, clinical presentation, and outcomes of PML across different transplant types.
- To inform clinical suspicion and management strategies for PML in this vulnerable patient group.
Main Methods:
- A literature review of 24 reported cases of PML in transplant recipients.
- Analysis of transplant type, time to PML onset, presenting neurological symptoms, and treatment modalities.
- Evaluation of patient outcomes, including mortality and reported regressions.
Main Results:
- PML occurred across various transplant types, with a median onset of 17 months post-transplant (71% within 24 months).
- Kidney transplant recipients showed a trend towards later PML onset (P=0.04).
- Common symptoms included hemiparesis (50%), apathy (46%), and confusion (38%); 71% of patients died within 2.5 months.
Conclusions:
- PML is a critical neurological complication to consider in transplant recipients presenting with neurological deficits.
- While often fatal, PML management may involve immunosuppression reduction and antiviral therapy (e.g., cidofovir).
- Further research is needed to better understand and manage PML in the context of transplantation.
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