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Lights Out on Postoperative Day 21

Clinical Vignette

A 47-year-old woman with end-stage renal disease secondary to hypertensive nephrosclerosis underwent a deceased-donor kidney transplant 21 days ago from a CMV-seropositive donor (CMV D+/R−). Her immunosuppressive regimen consists of tacrolimus, mycophenolate mofetil, and prednisone. Her post-transplant course was initially unremarkable and she was discharged on postoperative day 5 with stable graft function and a tacrolimus trough of 11.4 ng/mL.

She presents to the emergency department after a witnessed generalized tonic-clonic seizure lasting approximately 90 seconds, with postictal confusion. Her husband reports that over the preceding 48 hours she had developed a severe bifrontal headache and episodes of blurry vision that progressed to near-total loss of vision in both eyes. She had no fever, neck stiffness, or focal weakness. She had no prior history of seizures.

On examination she is afebrile, blood pressure 182/114 mmHg, heart rate 98 bpm, and oxygen saturation 97% on room air. She is oriented to person only and slow to respond. Pupillary reflexes are intact bilaterally. Visual acuity is severely reduced in both eyes. There is no nuchal rigidity, papilledema, or focal motor deficit. The transplant incision is clean and healing appropriately.

Tacrolimus trough level returns at 15.8 ng/mL. Serum creatinine is 1.6 mg/dL, up from a nadir of 1.2 mg/dL at discharge. Complete blood count and metabolic panel are otherwise unremarkable. Serum CMV NAT is pending. Serum cryptococcal antigen is negative. Serum Toxoplasma IgG is negative. MRI of the brain with gadolinium reveals bilateral symmetric T2-FLAIR hyperintensities predominantly involving the posterior parietal and occipital white matter with no restricted diffusion on diffusion-weighted imaging and no gadolinium enhancement. Lumbar puncture shows an opening pressure of 19 cm H₂O, WBC 3 cells/μL, protein 41 mg/dL, and glucose 74 mg/dL with a serum glucose of 138 mg/dL. CSF cryptococcal antigen is negative. No organisms are seen on Gram stain. CSF viral PCR studies including HSV-1, HSV-2, CMV, and HHV-6 are pending.

Question 1

Which of the following is the most likely diagnosis?

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Question 2

PRES is diagnosed. Which of the following is the most appropriate initial management?

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Answer the question above to reveal the rationale.
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References

Fugate JE, Rabinstein AA. Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. Lancet Neurology. 2015;14(9):914-925.

DOI: 10.1016/S1474-4422(15)00111-8

Bartynski WS. Posterior reversible encephalopathy syndrome, part 1: fundamental imaging and clinical features. American Journal of Neuroradiology. 2008;29(6):1036-1042.

DOI: 10.3174/ajnr.A0928

Eidelman BH, Abu-Elmagd K, Wilson J, et al. Neurologic complications of FK 506. Transplantation Proceedings. 1991;23(6):3175-3178.

PMID: 1721392

Legriel S, Schraub O, Azoulay E, et al. Determinants of recovery from severe posterior reversible encephalopathy syndrome. PLOS ONE. 2012;7(9):e44534.

DOI: 10.1371/journal.pone.0044534