Toxic Encephalopathy and Associations with Calcineurin Inhibitors

Document Type

Abstract

Location

Virtual

Start Date

24-4-2026 9:00 AM

End Date

24-4-2026 3:00 PM

Description

Introduction: Immunosuppressive encephalopathy is a syndrome of acute altered mentation often associated with fevers, focal deficits, and aphasia. Common causes include anti-neoplastic agents and calcineurin inhibitors. Laboratory findings include pleocytosis and elevated protein on lumbar puncture with a negative infectious workup. Pertinent imaging includes T2/FLAIR hyperintensities in white matter particularly in the frontal and parietal lobes and corpus callosum. Case: This is a 39-year-old female with systemic lupus erythematosus, bilateral blindness due to lupus, and chronic kidney disease who presented with altered mentation and fevers following an arteriovenous fistula (AVF) procedure. Pertinent medications include voclosporin, azathioprine, hydroxychloroquine, and steroids. Concern was given for sepsis related infection from AVF graft, but investigation showed no signs of infection surrounding the surgical site. A lumbar puncture was performed showing pleocytosis, elevated protein, and no microorganisms. The patient became increasingly febrile and lethargic leading to her being intubated, requiring cooling blankets, and stepped up to the intensive care unit. The patient was treated with antibiotics and steroids out of concern for both infectious and autoimmune causes. A full infectious workup was negative. Autoimmune workup showed a positive ANA and elevated C4 complement, but the remaining workup was negative or inconclusive. A magnetic resonance image of the brain showed T2 hyperintensities throughout the periventricular white matter, external capsule, and corpus callosum. She briefly underwent hemodialysis for uremia with subsequent improvement in her mental status. The patient showed improving mental and physical function following extubation and returned to her mental baseline after a few days. Discussion: We had a broad differential that included lupus cerebritis, infection, and adverse drug effect. Infection, lupus cerebritis, and anti-NMDA encephalitis were ruled out due to a negative laboratory work up. Our top differential is medication- induced encephalopathy due to imaging and lab findings. While other calcineurin inhibitors such as cyclosporine and tacrolimus have been associated with toxic leukoencephalopathy, there is no documented literature showing an association between voclosporin and toxic leukoencephalopathy. Voclosporin has been shown in studies to have neurotoxic effects, specifically with the development of headaches, migraines, seizures, and posterior reversible encephalopathic syndrome in higher doses of either 23.7mg or 39.5mg twice daily. Voclosporin is a next generation analog of cyclosporine that was FDA approved in 2021 for the treatment of lupus nephritis. While there are no current studies suggesting a link between toxic encephalopathy and voclosporin, it should still be considered when working up toxic leukoencephalopathy.

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Apr 24th, 9:00 AM Apr 24th, 3:00 PM

Toxic Encephalopathy and Associations with Calcineurin Inhibitors

Virtual

Introduction: Immunosuppressive encephalopathy is a syndrome of acute altered mentation often associated with fevers, focal deficits, and aphasia. Common causes include anti-neoplastic agents and calcineurin inhibitors. Laboratory findings include pleocytosis and elevated protein on lumbar puncture with a negative infectious workup. Pertinent imaging includes T2/FLAIR hyperintensities in white matter particularly in the frontal and parietal lobes and corpus callosum. Case: This is a 39-year-old female with systemic lupus erythematosus, bilateral blindness due to lupus, and chronic kidney disease who presented with altered mentation and fevers following an arteriovenous fistula (AVF) procedure. Pertinent medications include voclosporin, azathioprine, hydroxychloroquine, and steroids. Concern was given for sepsis related infection from AVF graft, but investigation showed no signs of infection surrounding the surgical site. A lumbar puncture was performed showing pleocytosis, elevated protein, and no microorganisms. The patient became increasingly febrile and lethargic leading to her being intubated, requiring cooling blankets, and stepped up to the intensive care unit. The patient was treated with antibiotics and steroids out of concern for both infectious and autoimmune causes. A full infectious workup was negative. Autoimmune workup showed a positive ANA and elevated C4 complement, but the remaining workup was negative or inconclusive. A magnetic resonance image of the brain showed T2 hyperintensities throughout the periventricular white matter, external capsule, and corpus callosum. She briefly underwent hemodialysis for uremia with subsequent improvement in her mental status. The patient showed improving mental and physical function following extubation and returned to her mental baseline after a few days. Discussion: We had a broad differential that included lupus cerebritis, infection, and adverse drug effect. Infection, lupus cerebritis, and anti-NMDA encephalitis were ruled out due to a negative laboratory work up. Our top differential is medication- induced encephalopathy due to imaging and lab findings. While other calcineurin inhibitors such as cyclosporine and tacrolimus have been associated with toxic leukoencephalopathy, there is no documented literature showing an association between voclosporin and toxic leukoencephalopathy. Voclosporin has been shown in studies to have neurotoxic effects, specifically with the development of headaches, migraines, seizures, and posterior reversible encephalopathic syndrome in higher doses of either 23.7mg or 39.5mg twice daily. Voclosporin is a next generation analog of cyclosporine that was FDA approved in 2021 for the treatment of lupus nephritis. While there are no current studies suggesting a link between toxic encephalopathy and voclosporin, it should still be considered when working up toxic leukoencephalopathy.