scholarly journals A broad spectrum of posterior reversible encephalopathy syndrome - a case series with clinical and paraclinical characterisation, and histopathological findings

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Fatme Seval Ismail ◽  
Johannes van de Nes ◽  
Ilka Kleffner

Abstract 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 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 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 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.

1970 ◽  
Vol 29 (4) ◽  
Author(s):  
Zewde Yared ◽  
Yifru Yared

BACKGROUND: Posterior Reversible Encephalopathy Syndrome (PRES) is a potentially reversible neurological disorder of acute to subacute onset characterized by headache, nausea and vomiting, visual disturbance, seizure and altered mental status.Neuroimaging findings are characteristic, which allow early diagnosis in the appropriate clinical setting and enable to institute appropriate therapy timely.CASE PRESENTATION: We report 3 adult patients with a history of hypertension presented with recurrent episode of seizure and altered mentation.While all the 3 patients were preliminarily diagnosed with Ischemic stroke, they were subsequently diagnosed with posterior reversible encephalopathy syndrome after neuroimaging revealed the typical features of the syndrome. They were started on antihypertensive and anticonvulsant drugs. On follow-up examination after 3-4 weeks, the patients showed marked clinical and neuro-imaging improvements.CONCLUSION: Posterior reversible encephalopathy syndrome is a rare condition. The presenting clinical symptoms are non-specific and may mimic other neurological disorders. Therefore, early recognition of classic radiographic features is vital to the diagnosis. Timely diagnosis and treatment of this syndrome is important as the treatment outcome is mostly favorable. 


2018 ◽  
Vol 6 (5) ◽  
pp. 851-854
Author(s):  
Samra Kadić-Vukas ◽  
Mirsada Hodžić ◽  
Lejla Tandir-Lihić ◽  
Lejla Hrvat ◽  
Azra Kožo-Kajmaković ◽  
...  

BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is a clinical-radiological syndrome with seizures, altered consciousness, visual disturbances and headache among other symptoms. Hinchey et al. first described Pres in 1996, with two other case series published shortly after.CASE REPORT: A 23-year-old women patient was emergency sent from General Hospital Tešanj due to a crisis of consciousness and repeated epileptic seizures. The patient had a second birth before 10 days (postpartum cesarean) in general endotracheal anaesthesia (two cesarean-born babies). On magnetic resonance imaging (MRI) of cranium described both sides of the symmetrically frontal, parietal (and pre-ventricular gyri) and occipitally visible T2W/FLAIR hyperintensity focuses on the cortex and the thin layer of white mass subcortically. In the projection of the lesions parts, discrete DWI hyperintensity is seen without a reliable ADC correlate. The patient improved after management with intravenous fluids, antibiotics, antiepileptics and monitoring of blood pressure. According to latest experiences delayed diagnosis and treatment may lead to mortality or irreversible neurological deficit. Aggravating circumstances are differential diagnoses that include cerebral infarction (ischemic, haemorrhage), venous thrombosis, vasculitis, pontine or extrapontine myelinolysis.CONCLUSION: MRI of the brain is key to make this distinction with crucial recognition and an open mind from radiology and neurology specialist.


2021 ◽  
Vol 14 (7) ◽  
pp. e242231
Author(s):  
Catarina Bernardes ◽  
Cristiana Silva ◽  
Gustavo Santo ◽  
Inês Correia

A 71-year-old woman presented to the emergency room with dysphonia, diplopia, dysphagia and generalised weakness since that day. Neurological examination revealed eye adduction limitation, ptosis, hypoactive reflexes and gait ataxia. Blood and cerebrospinal fluid analysis and brain CT were normal. Electromyography revealed a sensory axonal polyneuropathy. She was diagnosed with Miller-Fisher syndrome (MFS) and started on intravenous immunoglobulin. Two days after intravenous immunoglobulin treatment was completed, she developed a sustained hypertensive profile and presented a generalised tonic-clonic seizure. Brain MRI was suggestive of posterior reversible encephalopathy syndrome (PRES) and supportive treatment was implemented with progressive improvement. PRES may be a possible complication of MFS not only due to autonomic and inflammatory dysfunctions, but also as a consequence of its treatment. Patients with MFS should be maintained under close surveillance, especially in the first days and preferably in intermediate care units.


2021 ◽  
Author(s):  
Meng-Ko Tsai ◽  
Chao-Hung Lai ◽  
Tsung-Ju Chuang

Abstract Background Posterior reversible encephalopathy syndrome (PRES) following the development of diabetic ketoacidosis (DKA) is rare and usually occurs in children. This is the first case of DKA following PRES that we know of that occurred in an adult.Case report We encountered a middle-aged woman with a one-day history of nausea and vomiting who presented with DKA and seizure, along with hallucinations. On presentation, we performed physical examinations and blood biochemistry tests to ascertain the cause of these symptoms. We also performed magnetic resonance imaging (MRI) of her brain, which showed typical brain edema in the bilateral occipital and parietal regions, which indicated PRES. We treated the patient’s symptoms by administering adequate hydration and administering an infusion of insulin of 30 U after breakfast and 15 U after dinner to bring her blood sugar levels under control.The brain MRI we performed showed hyperintensity of the bilateral occipital and parietal cortexes on a fluid-attenuated inversion recovery T2 weighted image, after which the patient was diagnosed with PRES. The patient was discharged thirteen days after admission with stable blood sugar and blood pressure levels. Conclusions Physicians should keep this condition in mind as a possible complication of DKA and treat it quickly and efficiently in order to attain a good patient outcome.This is the first report of DKA-induced PRES in an adult, and physicians should keep this condition in mind as a possible complication of DKA, which is treatable and may have a good prognosis.


2011 ◽  
Vol 7 (3) ◽  
pp. 235-237 ◽  
Author(s):  
Melanie G. Hayden Gephart ◽  
Bonnie P. Taft ◽  
Anne-Katrin Giese ◽  
Raphael Guzman ◽  
Michael S. B. Edwards

Posterior reversible encephalopathy syndrome (PRES) has been described in pediatric neurooncology patients, although it has not been documented perioperatively in pediatric neurosurgery patients not actively receiving chemotherapy. Recently at the authors' facility, 2 cases of PRES were diagnosed perioperatively in children with brainstem ependymoma. Both patients had presented with hypertension, altered mental status, and seizures and demonstrated MR imaging features consistent with PRES. The patients were treated with antiseizure and antihypertension medications, leading to improvement in both clinical symptoms and neuroimaging findings. These cases are the first to document PRES in perioperative pediatric neurosurgery patients not actively receiving chemotherapy. Both patients had ependymoma involving the brainstem, which may have led to intra- and perioperative hemodynamic instability (including hypertension) and predisposed them to this syndrome. An awareness of PRES in similar scenarios will aid in the prevention, diagnosis, and treatment of pediatric neurosurgery patients with this syndrome.


2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Tariq Hammad ◽  
Alison DeDent ◽  
Rami Algahtani ◽  
Yaseen Alastal ◽  
Lawrence Elmer ◽  
...  

Posterior Reversible Encephalopathy Syndrome (PRES) is a clinical neuroradiological condition characterized by insidious onset of neurological symptoms associated with radiological findings indicating posterior leukoencephalopathy. PRES secondary to cerebrospinal fluid (CSF) leak leading to intracranial hypotension is not well recognized etiology of this condition. Herein, we report a case of PRES that occurred in the setting of CSF leak due to inadvertent dural puncture. Patient underwent suturing of the dural defect. Subsequently, his symptoms resolved and a repeated brain MRI showed resolution of brain lesions. The pathophysiology and mechanistic model for developing PRES in the setting of intracranial hypotension were discussed. We further highlighted the importance of tight blood pressure control in patients with CSF leak and suspected intracranial hypotension because they are more vulnerable to develop PRES with normal or slightly elevated bleed pressure values.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Kefeng Jia ◽  
Weili Yin ◽  
Fang Wang ◽  
Zhongsong Gao ◽  
Cheng Sun ◽  
...  

Abstract Background Posterior reversible encephalopathy syndrome (PRES) is a very rare complication secondary to transcatheter arterial chemoembolization (TACE). Only two patients with liver metastasis have been reported. We report for the first time two cases of hepatocellular carcinoma (HCC) patients occurred PRES secondary toTACE. Case presentation The two patients with HCC developed headache, epilepsy, expressive aphasia, visual impairment and loss of consciousness, 11 and 3 h after conventional TACE (c-TACE) surgery. One patient experienced raised blood pressure during and after TACE, accompanied by a significant elevated creatinine. The magnetic resonance imaging (MRI) of the two patients showed multiple abnormal signals in the brain, mainly located in the white matter region. Combined with the clinical symptoms and MRI findings, PRES was diagnosed. Their symptoms and MRI changes improved significantly in the next two weeks. Conclusion The PRES in this report is chemoembolization-associated syndrome, which might be related to the use of chemotherapy agents during TACE. And if neurological symptoms occur after TACE, patients should be closely monitored to exclude PRES.


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