Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Letter to the Editor
  • Published:

Posterior reversible encephalopathy syndrome due to sirolimus

Bone Marrow Transplantation volume 39, pages 653–654 (2007)Cite this article

Posterior reversible encephalopathy syndrome (PRES) was first described in 15 patients by Hinchey et al. in 1996.1 The most common clinical symptoms and signs included headache, altered alertness and confusion, vomiting, seizures, and visual disturbance ranging from blurred vision to cortical blindness. Neuroimaging typically showed white matter edema in the posterior portions of the cerebral hemispheres, particularly in the parieto-occipital regions. The presumed etiologies for the 15 patients included hypertensive encephalopathy (n=4), preeclampsia (n=3), cyclosporine (n=4), tacrolimus (n=3), and interferon-α (n=1). With control of blood pressure and withdrawal or reduction of the immunosuppressive agent, all of the patients had resolution of symptoms within 2 weeks and neuroimaging normalized. Since the initial description of PRES, there have been a number of reports of its incidence following allogeneic bone marrow transplant in patients receiving cyclosporine or tacrolimus for graft-versus-host disease (GVHD) prophylaxis.2, 3, 4 Toxic levels of the immunosuppressive medications do not appear to be required for the development of PRES.5 In a previous report of a lung transplant recipient patient on sirolimus that developed a clinical syndrome similar to PRES, the neurotoxicity was attributed to dialysis disequilibrium syndrome.6 The patient improved with adjustment in the dialysis regimen and continued on sirolimus without recurrent neurological complaints. An association between treatment with sirolimus and PRES has otherwise never been reported. Here we present a case of PRES occurring in the setting of treatment with sirolimus for GVHD prophylaxis.

Although PRES is usually reversible with prompt withdrawal or reduction of the immunosuppressive agent and blood pressure control, it can be associated with permanent neurologic deficits and death.2 Early recognition and treatment is therefore warranted in patients presenting with the signs and symptoms associated with PRES. The differential diagnosis for this patients presenting symptoms of cortical blindness and confusion include stroke, cerebral sinus thrombosis, encephalitis, and demyelinating disorders. PRES can usually be distinguished from these other possible diagnoses by its characteristic MRI findings.7

This is a preview of subscription content, access via your institution

Access options

Buy this article

  • Purchase on Springer Link
  • Instant access to full article PDF
Buy now

Prices may be subject to local taxes which are calculated during checkout

Figure 1

References

  1. Hinchey J, Chaves C, Appignani B, Breen J, Pao L, Wang A et al. A reversible posterior leukoencephalopathy syndrome. N Engl J Med 1996; 334: 494–500.

    Article  CAS  Google Scholar 

  2. Wong R, Beguelin GZ, de Lima M, Giralt SA, Hosing C, Ippoliti C et al. Tacrolimus-associated posterior reversible encephalopathy syndrome after allogeneic haematopoietic stem cell transplantation. Br J Haematol 2003; 122: 128–134.

    Article  CAS  Google Scholar 

  3. Teive HA, Brandi IV, Camargo CH, Bittencourt MA, Bonfim CM, Friedrich ML et al. Reversible posterior leucoencephalopathy syndrome associated with bone marrow transplantation. Arq Neuropsiquiatr 2001; 59: 784–789.

    Article  CAS  Google Scholar 

  4. Uoshima N, Karasuno T, Yagi T, Kawamoto S, Hasegawa T, Yasumi M et al. Late onset cyclosporine-induced cerebral blindness with abnormal SPECT imagings in a patient undergoing unrelated bone marrow transplantation. Bone Marrow Transplant 2000; 26: 105–108.

    Article  CAS  Google Scholar 

  5. Covarrubias DJ, Luetmer PH, Campeau NG . Posterior reversible encephalopathy syndrome: prognostic utility of quantitative diffusion-weighted MR images. AJNR Am J Neuroradiol 2002; 23: 1038–1048.

    PubMed  Google Scholar 

  6. Sheth KN, Wu GF, Messe SR, Wolf RL, Kasner SE . Dialysis disequilibrium: another reversible posterior leukoencephalopathy syndrome? Clin Neurol Neurosurg 2003; 105: 249–252.

    Article  Google Scholar 

  7. Garg RK . Posterior leukoencephalopathy syndrome. Postgrad Med J 2001; 77: 24–28.

    Article  CAS  Google Scholar 

  8. Serkova N, Christians U, Flogel U, Pfeuffer J, Leibfritz D . Assessment of the mechanism of astrocyte swelling induced by the macrolide immunosuppressant sirolimus using multinuclear nuclear magnetic resonance spectroscopy. Chem Res Toxicol 1997; 10: 1359–1363.

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Medicine, Allogeneic Bone Marrow Transplantation Service, Memorial Sloan-Kettering Cancer Center, New York, NY, USA

    A Moskowitz, H Castro-Malaspina & M-A Perales

  2. Department of Neurology, Memorial Sloan-Kettering Cancer Center, New York, NY, USA

    C Nolan

  3. Department of Radiology, Memorial Sloan-Kettering Cancer Center, New York, NY, USA

    E Lis

Authors
  1. A Moskowitz
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. C Nolan
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. E Lis
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. H Castro-Malaspina
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. M-A Perales
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to M-A Perales.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Moskowitz, A., Nolan, C., Lis, E. et al. Posterior reversible encephalopathy syndrome due to sirolimus. Bone Marrow Transplant 39, 653–654 (2007). https://doi.org/10.1038/sj.bmt.1705659

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1038/sj.bmt.1705659

This article is cited by

Search

Advanced search

Quick links