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In Vitro and In Vivo Assessment of T, B and Myeloid Cells Suppressive Activity and Humoral Responses from Transplant Recipients
Published on: August 12, 2017
Atypical progressive multifocal leukoencephalopathy in a kidney transplant recipient with improving symptoms after
Giorgia Bruno1, Giuseppina Miele1, Gianmarco Abbadessa1
1Second Division of Neurology. Department of Advanced Medical and Surgical Sciences, University of Campania "Luigi Vanvitelli", Naples, Italy.
Abstract:
Progressive multifocal leukoencephalopathy (PML) is a viral infection due to John Cunningham Virus (JCV) resulting in progressive damage of brain white matter, mostly related to HIV infection or hemato-oncological malignancies. PML onset is usually multifocal with rapid neurological progression and poor prognosis. Here we report an atypical case of PML with monofocal onset and a good outcome in a 64-year-old man who received a kidney transplant for end-stage renal disease (ESRD). The applied antirejection immunosuppressive drug regimen included tacrolimus, prednisone and mycophenolic acid. Three years after the transplant, he complained of right-hand tremor and rapidly progressive right hemiparesis, with prominent involvement of the upper limb. Brain magnetic resonance imaging (MRI) showed a significant demyelinating area in the left frontal lobe, without mass effect and contrast enhancement. Real-time PCR analysis revealed the presence of JCV on cerebrospinal fluid. Consequent immunosuppressive drug suspension resulted in a global improvement of neurological symptoms and a favourable evolution of the neuroradiological findings. Subsequent eight-year follow-up MRI confirmed the stability of imaging findings over time. Therefore, early recognition of PML symptoms and MRI sign along with the rapid suspension of immunosuppressive drugs can modify the natural history of this disease after a kidney transplant.
Insights
A rare case of progressive multifocal leukoencephalopathy (PML) in a kidney transplant recipient showed an atypical monofocal onset. Early recognition and immunosuppressive drug suspension led to a good outcome and symptom improvement.
Area of Science:
- Nephrology
- Neurology
- Infectious Diseases
Background:
- Progressive multifocal leukoencephalopathy (PML) is a severe demyelinating brain disease caused by John Cunningham Virus (JCV).
- PML is typically associated with profound immunosuppression, often seen in HIV/AIDS or hematologic malignancies, and usually presents with multifocal neurological deficits and poor prognosis.
Observation:
- A 64-year-old kidney transplant recipient developed an atypical, monofocal presentation of PML three years post-transplant.
- Symptoms included right-hand tremor and progressive right hemiparesis, predominantly affecting the upper limb.
- Brain MRI revealed a solitary demyelinating lesion in the left frontal lobe without mass effect or contrast enhancement.
Findings:
- Cerebrospinal fluid analysis confirmed JCV infection via real-time PCR.
- Discontinuation of immunosuppressive agents (tacrolimus, prednisone, mycophenolic acid) resulted in significant neurological improvement.
- Follow-up MRI over eight years demonstrated stable imaging findings, indicating a favorable disease course.
Implications:
- This case highlights that PML can present atypically after kidney transplantation.
- Early diagnosis of PML, based on clinical suspicion and neuroimaging, is crucial.
- Prompt reduction of immunosuppression is a key strategy to improve outcomes in transplant patients with PML.
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