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Posterior reversible encephalopathy syndrome as a consequence of high dose steroid administration after autologous PBSCT

Bone Marrow Transplantation volume 45, pages 779–780 (2010)Cite this article

Posterior reversible encephalopathy syndrome (PRES) or reversible posterior leukoencephalopathy syndrome is a syndrome characterized clinically by a wide array of neurological symptoms including headache, confusion, drowsiness, vomiting, seizures and even visual loss in some patients.1 PRES has been reported in association with adult and pediatric Allo-SCT, as well as adult cord blood transplantation.2 Typically, PRES occurs in the setting of hypertensive encephalopathy resulting from the use of CY3 or tacrolimus4 to prevent the development of GVHD. Herein, we report a case of PRES that occurred in the absence of any exposure to calcineurin inhibitors and was attributable to high-dose steroid administration in a patient undergoing autologous peripheral SCT for multiple myeloma.

Posterior reversible encephalopathy syndrome has been reported to occur in association with numerous co-morbidities, including hypertensive encephalopathy,6 pre-eclampsia, eclampsia,7 infections,8 electrolyte imbalance,8 immunosuppressive drugs,1, 2 allogeneic BMT,1 solid organ transplantation,9 autoimmune diseases,1 and high-dose cancer chemotherapy.10 In a large series of patients reported from the Mayo Clinic (Rochester, MN, USA), the most common presenting features were clinical seizures (87%), encephalopathy (92%), visual symptoms (39%) and headache (53%).2 Radiographically, PRES is characterized by sub-cortical vasogenic edema, patchy symmetrical bilateral involvement with preferential involvement of the posterior brain regions, and in most cases eventual resolution with clinical intervention and reversal of primary insult.6 By neuro-imaging criteria, fluid-attenuated inversion recovery or T2-weighted signal is bright, the diffusion magnetic resonance image is normal and the apparent diffusion coefficient map shows increased activity. The most widely accepted hypothesis regarding the pathogenesis of PRES proposes a capillary leak secondary to drugs that affect the endothelium or increase blood pressure and/or blood vol.1 It has been suggested that the vertebrobasilar territory, owing to its relatively sparse sympathetic innervation, may experience preferential disruption of autoregulatory mechanisms, leading to increased perfusion and edema.11 Typically, resolution of symptoms and neuro-imaging abnormalities occur in the range of several days to weeks after removal of the primary inciting agent.

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Authors and Affiliations

  1. Department of Internal Medicine, Medical College of Wisconsin, Milwaukee, WI, USA

    S Sukumaran

  2. Bone Marrow Transplant Program, Medical College of Wisconsin, Milwaukee, WI, USA

    B George, H P Nair & W R Drobyski

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  1. S Sukumaran
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  2. B George
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  3. H P Nair
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  4. W R Drobyski
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Correspondence to W R Drobyski.

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Sukumaran, S., George, B., Nair, H. et al. Posterior reversible encephalopathy syndrome as a consequence of high dose steroid administration after autologous PBSCT. Bone Marrow Transplant 45, 779–780 (2010). https://doi.org/10.1038/bmt.2009.211

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