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Kidney Week

Abstract: PO2109

A Case of Unexplained Encephalopathy in a Kidney Transplant Recipient

Session Information

Category: Transplantation

  • 1902 Transplantation: Clinical

Authors

  • Easwar, Anjana, Emory University, Atlanta, Georgia, United States
  • Lakhani, Laila S., Emory University, Atlanta, Georgia, United States
  • Basu, Arpita, Emory University, Atlanta, Georgia, United States
  • Pastan, Stephen O., Emory University, Atlanta, Georgia, United States
Introduction

Progressive multifocal leukoencephalopathy is a fatal demyelinating disease caused by the JC virus. Kidney transplant patients who are immunosuppressed are at higher risk of this infection.

Case Description

A 42 year old woman with CKD5 from FSGS who underwent a living donor kidney transplant 5 months ago, on immunosuppression with tacrolimus, mycophenolate and prednisone, presented with acute confusion, expressive aphasia and gait disturbance. Differentials included structural, infectious, metabolic and nutritional causes. A lumbar puncture and MRI brain were unremarkable. She was treated for suspected thiamine deficiency with intravenous thiamine. She developed myoclonus and hyperreflexia. Benzodiazepines and cyproheptadine were started with concern for serotonin syndrome. Tacrolimus dose was reduced with concern for calcineurin neurotoxicity. On hospital day 10, she developed status epilepticus; but MRI brain was unremarkable and EEG showed generalized encephalopathy. A second lumbar puncture revealed high opening pressures, WBCs and protein concerning for meningoencephalitis with negative gram stain and culture. CNS virus panel including HSV was negative. Empiric treatment with broad spectrum antimicrobials was begun. Tacrolimus was switched to sirolimus without neurologic improvement. A third CSF sample showed pleocytosis and elevated protein. Extended viral panel testing was sent to an outside lab. On day 16, she developed hemodynamic instability with worsening neurological exam, rapidly progressing to fixed dilated pupils and absent cough and gag reflexes. Within hours she developed cardiac arrest and was successfully resuscitated. CT head post arrest demonstrated diffuse cerebral edema and tonsillar herniation indicating devastating neurological injury. She was declared brain dead on hospital day 17. The CSF metagenomic testing panel later returned positive for JC virus.

Discussion

This is a complex and unfortunate case of PML due to JC virus in a newly transplanted patient, highlighting the ability of opportunistic CNS infections to masquerade as a nutritional deficiency/drug toxicity and the challenges of pursuing advanced diagnostic work up beyond standard testing. Immunosuppression in kidney transplant recipients remains a double edged sword where tilting the fine balance in favor of over-immunosuppression can lead to catastrophic infectious complications.