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.

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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