Abstract
The purpose of this study is to characterize the spectrum of cerebrospinal fluid (CSF) findings in patients with posterior reversible encephalopathy syndrome (PRES) and determine its associations with severity of edema. In this retrospective cross-sectional study electronic medical reports were screened for patients with PRES. Clinical and laboratory data closest to onset of clinical symptoms were collected. Neuroimaging reports from all patients were categorized according to the presence of vasogenic edema, cytotoxic edema and contrast enhancement. From Jan 1999 to Feb 2015, 87 patients with PRES and CSF findings were included. Mean total protein levels were 793 ± 929 mg/l. Median cell counts were 2/µl (IQR 1–4). Eight patients had cell counts >6/µl with a maximum of 41/µl. In 31 patients with extended CSF analysis, mean CSF/serum albumin quotient (QAlb) was 10.1 (IQR 6–17.3). An elevated QAlb (age-adjusted) was found in 74 % of these 31 patients. PRES patients with severe edema had higher mean total protein levels in CSF (mean difference = 407 mg/l; 95 % CI 187–628). Significant correlations were found for edema severity with levels of CSF protein (r = 0.48, p < 0.001) as well with QAlb (r = 0.44, p = 0.013). This study shows that total protein in CSF and QAlb are elevated and correlate with severity of edema in PRES patients, whereas pleocytosis was rare in our cohort. This supports the theory that PRES is caused by a dysfunctional blood brain barrier.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Fugate JE, Rabinstein AA (2015) Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. Lancet Neurol. doi:10.1016/S1474-4422(15)00111-8
Liman TG, Bohner G, Endres M, Siebert E (2014) Discharge status and in-hospital mortality in posterior reversible encephalopathy syndrome. Acta Neurol Scand 130:34–39
Alhilali LM, Reynolds AR, Fakhran S (2014) A multi-disciplinary model of risk factors for fatal outcome in posterior reversible encephalopathy syndrome. J Neurol Sci 347:59–65
Legriel S, Schraub O, Azoulay E et al (2012) Determinants of recovery from severe posterior reversible encephalopathy syndrome. PLoS One 7:e44534
Bartynski WS (2008) Posterior reversible encephalopathy syndrome, part 2: controversies surrounding pathophysiology of vasogenic edema. AJNR Am J Neuroradiol 29:1043–1049
Marra A, Vargas M, Striano P et al (2014) Posterior reversible encephalopathy syndrome: the endothelial hypotheses. Med Hypotheses 82:619–622
Deisenhammer F, Bartos A, Egg R et al (2006) Guidelines on routine cerebrospinal fluid analysis. Report from an EFNS task force. Eur J Neurol 13:913–922
Lee VH, Wijdicks EFM, Manno EM, Rabinstein AA (2008) Clinical spectrum of reversible posterior leukoencephalopathy syndrome. Arch Neurol 65:205–210
Hamilton BE, Nesbit GM (2008) Delayed CSF enhancement in posterior reversible encephalopathy syndrome. AJNR Am J Neuroradiol 29:456–457
Liman TG, Bohner G, Heuschmann PU et al (2012) Clinical and radiological differences in posterior reversible encephalopathy syndrome between patients with preeclampsia-eclampsia and other predisposing diseases. Eur J Neurol 19:935–943
Liman TG, Bohner G, Heuschmann PU et al (2012) The clinical and radiological spectrum of posterior reversible encephalopathy syndrome: the retrospective Berlin PRES study. J Neurol 259:155–164
Reiber H, Peter JB (2001) Cerebrospinal fluid analysis: disease-related data patterns and evaluation programs. J Neurol Sci 184:101–122
Mehdi A, Hajj-Ali RA (2014) Reversible cerebral vasoconstriction syndrome: a comprehensive update. Curr Pain Headache Rep 18:443
Terranova S, Kumar JD, Libman RB (2012) Posterior reversible encephalopathy syndrome mimicking a left middle cerebral artery stroke. Open Neuroimag J 6:10–12
Pires PW, Dams Ramos CM, Matin N, Dorrance AM (2013) The effects of hypertension on the cerebral circulation. Am J Physiol Heart Circ Physiol 304:H1598–H1614
Granata G, Greco A, Iannella G et al (2015) Posterior reversible encephalopathy syndrome-Insight into pathogenesis, clinical variants and treatment approaches. Autoimmun Rev 14:830–836
Deisenhammer F, Bartos A, Egg R et al (2006) Guidelines on routine cerebrospinal fluid analysis. Report from an EFNS task force. Eur J Neurol 13:913–922
Acknowledgments
The research leading to these results has also received funding from the Federal Ministry of Education and Research (BMBF) through the Grant Center for Stroke Research Berlin (01 EO 0801), from the German Science Foundation (DFG; Neurocure) and from the Volkswagen Foundation (Lichtenberg program to Matthias Endres).
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflicts of interest
None declared.
Ethical standards
The local ethics committee (Charité Universitätsmedizin Berlin) has approved the study. All study procedures have been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments. Due to the retrospective review of depersonalized medical records for this study, the ethics committee granted a waiver of informed consent.
Electronic supplementary material
Below is the link to the electronic supplementary material.
Rights and permissions
About this article
Cite this article
Neeb, L., Hoekstra, J., Endres, M. et al. Spectrum of cerebral spinal fluid findings in patients with posterior reversible encephalopathy syndrome. J Neurol 263, 30–34 (2016). https://doi.org/10.1007/s00415-015-7928-8
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00415-015-7928-8