A broad spectrum of posterior reversible encephalopathy syndrome - a case series with clinical and paraclinical characterisation, and histopathological findings.
Histopathological findings
Posterior reversible encephalopathy syndrome (PRES)
Reversibility
Journal
BMC neurology
ISSN: 1471-2377
Titre abrégé: BMC Neurol
Pays: England
ID NLM: 100968555
Informations de publication
Date de publication:
06 Oct 2021
06 Oct 2021
Historique:
received:
10
06
2021
accepted:
14
09
2021
entrez:
7
10
2021
pubmed:
8
10
2021
medline:
16
10
2021
Statut:
epublish
Résumé
Posterior reversible encephalopathy syndrome (PRES) is clinical-neuroradiologically defined and potentially reversible, so there are limited data about histopathological findings. We aimed to describe the clinical and paraclinical features of patients with PRES with regard to its reversibility. This retrospective case series encompasses 15 PRES cases out of 1300 evaluated patients from a single German center between January 1, 2010, and June 15, 2020. PRES was established according to the diagnostic criteria as proposed by the Berlin PRES Study 2012. One of the cases studied was subject to brain autopsy. From the 15 patients studied (median age 53 years, range 17-73; 11 female), 67 % presented with epileptic seizures, 40 % suffered from encephalopathy with reduced consciousness and 53 % developed delirium, while 47 % had headache and visual disturbances. Subcortical brain MRI abnormalities related to PRES were observed in all patients. One patient developed spinal ischemia and another Guillain-Barré syndrome in addition to PRES. Hypertensive blood pressure was the main underlying/trigger condition in all patients. Clinical symptoms and MRI changes were not reversible in 42 %, even progressive in 3 out of these 5 patients. Median time from symptom onset to diagnosis in these non-reversible cases was 7 days (range 0-13), while the median delay in diagnosis in the reversible group was 1 day (range 0-3). Cerebellar/brain stem involvement and status epilepticus were more frequently in patients with non-reversible disease course. Mortality due to PRES occurred in 13 % of these patients. Neuropathological examination of the brain of a 57-year-old female patient revealed major leukencephalopathic changes, fibrinoid necrosis of endothelial cells and fresh petechial hemorrhages in accordance with PRES. Our case series demonstrates that PRES was not reversible in 42 % of the studied patients. Delay in diagnosis seems to contribute to limited reversibility and poor outcome.
Sections du résumé
BACKGROUND
BACKGROUND
Posterior reversible encephalopathy syndrome (PRES) is clinical-neuroradiologically defined and potentially reversible, so there are limited data about histopathological findings. We aimed to describe the clinical and paraclinical features of patients with PRES with regard to its reversibility.
METHODS
METHODS
This retrospective case series encompasses 15 PRES cases out of 1300 evaluated patients from a single German center between January 1, 2010, and June 15, 2020. PRES was established according to the diagnostic criteria as proposed by the Berlin PRES Study 2012. One of the cases studied was subject to brain autopsy.
RESULTS
RESULTS
From the 15 patients studied (median age 53 years, range 17-73; 11 female), 67 % presented with epileptic seizures, 40 % suffered from encephalopathy with reduced consciousness and 53 % developed delirium, while 47 % had headache and visual disturbances. Subcortical brain MRI abnormalities related to PRES were observed in all patients. One patient developed spinal ischemia and another Guillain-Barré syndrome in addition to PRES. Hypertensive blood pressure was the main underlying/trigger condition in all patients. Clinical symptoms and MRI changes were not reversible in 42 %, even progressive in 3 out of these 5 patients. Median time from symptom onset to diagnosis in these non-reversible cases was 7 days (range 0-13), while the median delay in diagnosis in the reversible group was 1 day (range 0-3). Cerebellar/brain stem involvement and status epilepticus were more frequently in patients with non-reversible disease course. Mortality due to PRES occurred in 13 % of these patients. Neuropathological examination of the brain of a 57-year-old female patient revealed major leukencephalopathic changes, fibrinoid necrosis of endothelial cells and fresh petechial hemorrhages in accordance with PRES.
CONCLUSIONS
CONCLUSIONS
Our case series demonstrates that PRES was not reversible in 42 % of the studied patients. Delay in diagnosis seems to contribute to limited reversibility and poor outcome.
Identifiants
pubmed: 34615476
doi: 10.1186/s12883-021-02408-0
pii: 10.1186/s12883-021-02408-0
pmc: PMC8492815
doi:
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
386Informations de copyright
© 2021. The Author(s).
Références
J Neurol Sci. 2014 Dec 15;347(1-2):59-65
pubmed: 25271189
Lancet Neurol. 2015 Sep;14(9):914-925
pubmed: 26184985
J Neuropathol Exp Neurol. 2018 Feb 1;77(2):100-118
pubmed: 29325172
AJNR Am J Neuroradiol. 2007 Aug;28(7):1320-7
pubmed: 17698535
J Neurol Neurosurg Psychiatry. 2018 Jan;89(1):14-20
pubmed: 28794149
AJNR Am J Neuroradiol. 2002 Jun-Jul;23(6):1038-48
pubmed: 12063238
J Neurol Sci. 2017 Apr 15;375:382-387
pubmed: 28320172
Arch Neurol. 2008 Feb;65(2):205-10
pubmed: 18268188
J Comput Assist Tomogr. 2007 Jan-Feb;31(1):148-56
pubmed: 17259848
J Neurol. 2012 Jan;259(1):155-64
pubmed: 21717193
Pediatr Dev Pathol. 2010 Sep-Oct;13(5):397-403
pubmed: 20158377
Front Neurol. 2020 Feb 14;11:34
pubmed: 32117007
J Stroke Cerebrovasc Dis. 2015 Aug;24(8):e191-5
pubmed: 26082344
Curr Pain Headache Rep. 2021 Feb 25;25(3):19
pubmed: 33630183
J Neurol Sci. 2020 Sep 15;416:117019
pubmed: 32679347
J Neurol. 2016 Jan;263(1):30-4
pubmed: 26477022
J Clin Neurosci. 2021 Jun;88:108-112
pubmed: 33992168
Neurohospitalist. 2019 Apr;9(2):58-64
pubmed: 30915182