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Progressive multifocal leukoencephalopathy - immune reconstitution inflammatory syndrome (PML-IRIS) in liver
J Avsenik1, A Horvat Ledinek2, K Šurlan Popovič1
1Clinical Radiology Institute, University Medical Centre Ljubljana, Zaloška cesta 7, 1000 Ljubljana, Slovenia.
Abstract:
65-year-old liver transplant recipient presented with progressive neurologic dysfunction. CSF analysis revealed high JC virus load and MRI findings suggested the diagnosis of progressive multifocal leukoencephalopathy (PML). Cidofovir and mirtazapine were initiated and patient's regular immunosuppressants were reduced. Subsequently patient developed left sided hemiplegia, drowsiness and severe neglect syndrome. MRI revealed enlargement of PML lesions with contrast enhancement and worsening of oedema, consistent with immune reconstitution inflammatory syndrome (IRIS). Steroids were initiated and 3 weeks later patient showed moderate neurologic improvement. PML-IRIS after solid organ transplantation is rarely detected and to the best of our knowledge, this is the first reported case of PML-IRIS in a liver transplant recipient.
Insights
This case study reports the first instance of progressive multifocal leukoencephalopathy immune reconstitution inflammatory syndrome (PML-IRIS) in a liver transplant patient. Early diagnosis and treatment led to moderate neurologic improvement, highlighting the importance of recognizing this rare complication.
Area of Science:
- Neuroscience
- Immunology
- Transplantation Medicine
Background:
- Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease caused by the JC virus.
- Solid organ transplant recipients are at increased risk of opportunistic infections, including PML, due to long-term immunosuppression.
Observation:
- A 65-year-old liver transplant recipient presented with neurological decline.
- Cerebrospinal fluid analysis showed a high JC virus load, and MRI confirmed PML.
- Following treatment initiation and immunosuppression reduction, the patient developed worsening neurological symptoms and enlarged PML lesions, indicative of immune reconstitution inflammatory syndrome (IRIS).
Findings:
- The patient was diagnosed with PML-IRIS, a rare complication following solid organ transplantation.
- Treatment with steroids resulted in moderate neurological improvement within three weeks.
- This represents the first documented case of PML-IRIS in a liver transplant recipient.
Implications:
- This case underscores the importance of considering PML-IRIS in transplant patients with neurological deterioration.
- Prompt recognition and management, including immunosuppression modulation and anti-inflammatory treatment, are crucial for patient outcomes.
- Further research is needed to understand the pathogenesis and optimize treatment strategies for PML-IRIS in transplant populations.
