scholarly journals Posterior reversible encephalopathy syndrome as the first manifestation of mixed connective tissue disorder: a case report

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Phani Krishna Machiraju ◽  
Neetu Mariam Alex ◽  
Safinaaz ◽  
Sriram Sankaran

Abstract Background Posterior reversible encephalopathy syndrome (PRES) is a neurological syndrome characterised by a range of neurological symptoms and signs, and distinctive neuroimaging findings reflecting vasogenic oedema. Posterior reversible encephalopathy syndrome has been described in association with many autoimmune diseases, but its association with mixed connective tissue disorder (MCTD) is very rare. After an extensive review of the literature, we found only three cases of posterior reversible encephalopathy syndrome in association with mixed connective tissue disorder. But unlike other cases, in our patient, PRES is the presenting manifestation of mixed connective tissue disorder which is first of its kind. Case presentation We present a 30-year-old female from Southern India who had initially reported with complaints of fever, multiple episodes of vomiting and cough with expectoration. She had accelerated hypertension and moderate thrombocytopenia. Two days later, she developed sudden onset of visual disturbances and had a drop in sensorium. Neuroimaging done was suggestive of atypical posterior reversible encephalopathy syndrome, and autoimmune workup was positive for mixed connective tissue disorder. With prompt blood pressure control and anti-seizure medications, she recovered completely. Conclusion Early diagnosis and prompt control of blood pressure, along with anti-seizure measures, play a crucial role in management. Awareness about this rare association is essential for early diagnosis and treatment, and therefore reducing the risk of permanent neurologic deficits. This case is being reported because of its rarity.

Author(s):  
Malyaban Das ◽  
Prajit Mazumdar ◽  
Divakar Kumar ◽  
Vaibhav Mathur

Posterior reversible encephalopathy syndrome (PRES) is a condition which is characterized by symmetric involvement of posterior white matter on brain imaging and neurological impairments such as seizures, altered mental status, headache, and visual disturbances. This entity has been classically described with hypertension, renal failure and eclampsia but it can also been seen in cases with normal blood pressure especially in patients receiving immunosuppressive therapy, chemotherapy and in patients with underlying autoimmune disease. Although PRES has been reported with several autoimmune disorders, association of Posterior reversible encephalopathy syndrome (PRES) with mixed connective tissue disease (MCTD) is very rare, hence we report a case of Posterior reversible encephalopathy syndrome in a patient with underlying mixed connective tissue disease (MCTD).


2020 ◽  
pp. 1-3
Author(s):  
Sunny Aggarwal ◽  
Priyanka dev ◽  
Sagar Mazumder

Posterior reversible encephalopathy syndrome (PRES) usually presents with headache, confusion, visual disturbances or blindness and seizures. MRI is gold standard with parieto-occipital white matter changes due to vasogenic oedema in most of the cases. We report a 20-year-old unbooked primigravida with eclampsia prior to delivery which got complicated by PRES after delivery on day 1 postpartum. General anesthesia was given for caesarean section and post operatively patient was managed in ICU with strict blood pressure and seizure control. Clinical improvement with complete resolution without any complications was observed on the day 10 postpartum.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Jayawardane Pathiranage Roneesha Lakmali ◽  
Kanapathipillei Thirumavalavan ◽  
Danapala Dissanayake

Abstract Background Leptospirosis is a zoonotic spirochetal disease caused by Leptospira interrogans. The clinical presentation ranges from an asymptomatic state to a fatal multiorgan dysfunction. Neurological manifestations including aseptic meningitis, spinal cord and peripheral nerve involvement, cranial neuropathies and cerebellar syndrome are well recognized with varying frequencies among patients with this disease. Posterior reversible encephalopathy syndrome is a very rare occurrence in leptospirosis and only two cases are reported in the medical literature up to now. We report a case of posterior reversible encephalopathy syndrome in a patient with leptospirosis with rhabdomyolysis and acute kidney injury. Case presentation A 21 year-old male presented with fever and oliguric acute kidney injury with rhabdomyolysis. A diagnosis of leptospirosis was made and he was being managed according to the standard practice together with regular hemodialysis. The clinical condition was improving gradually. On day 8 of the illness, he developed headache and sudden painless complete bilateral vision loss followed by several brief generalized tonic clonic seizure attacks. Examination was significant for a Glasgow Coma Scale of 14/15, blood pressure of 150/90 mmHg and complete bilateral blindness. The findings of magnetic resonance imaging of the brain were compatible with posterior reversible encephalopathy syndrome. He was managed with blood pressure control and antiepileptics with supportive measures and standard treatment for leptospirosis and made a complete recovery. Conclusion Posterior reversible encephalopathy syndrome, though very rare with leptospirosis, should be considered as a differential diagnosis in a patient with new onset visual symptoms and seizures, especially during the immune phase. Optimal supportive care together with careful blood pressure control and seizure management would yield a favourable outcome in this reversible entity.


2021 ◽  
Vol 7 (2) ◽  
pp. 143-145
Author(s):  
Maythem Abdulhassan Al-Kaisy

Objective: During the late 2019, a group of patients had unexplained chest infections in Wuhan which turned out to be the new pandemic coronavirus disease 2019 (COVID-19). New neurological symptoms have been reported in COVID-19 patients. Posterior reversible encephalopathy syndrome (PRES) is a new neurological finding and is associated or caused by COVID-19. Case Presentation: A 32-year-old lady, with no medical background had COVID-19 infection and needed mechanical ventilation. After surviving the intensive care, she started to have multiple seizures that required general anesthesia to be aborted. The patient turned out to have PRES. Conclusion: PRES is a neurological syndrome causing seizures, headaches, and blurred vision. It is usually associated with high blood pressure, renal failure, and other risk factors. The patient in this case had nearly normal blood pressure, but still had a diagnosis of PRES. The new reported neurological symptoms associated with COVID-19 infection need further research and attention from the academic society to predict and prevent the morbidity and mortality of COVID-19 patients.


2020 ◽  
Vol 7 (10) ◽  
pp. 1537
Author(s):  
Sriramchandra Rahul Pulavarty ◽  
Nagabhushana Midathala

Background: Posterior reversible encephalopathy syndrome is a neurological disorder which can present with sudden onset headache, visual disturbances, seizures and altered sensorium which is potentially reversible with early detection and treatment of the precipitating factor. The range of presentations is being constantly widened and this endeavour is a step towards understanding the wide array of presentation and primary etiology.Methods: This is a prospective observational study of 25 patients presenting to a tertiary care hospital with symptoms and imaging features suggestive of PRES. Thorough clinical examination and MRI brain were performed in all patients.Results: Out of the 25 patients, 18 (72%) were females and 7 (28%) were males. Most common symptom was headache (84%) followed by seizures (56%), nausea (40%), visual blurring (36%) and altered sensorium (20%). In patients presenting with seizure, 28.57% had recurrent seizures.The most common precipitating cause was postpartum state without hypertension (40%) followed by accelerated hypertension (28%), eclampsia (16%), chronic kidney disease (12%) and one patient of chronic severe anaemia had PRES following blood transfusion (4%). Most of the patients improved with no residual neurological deficit.Conclusions: Good neurological outcomes can be achieved by early diagnosis and appropriate imaging in patients with PRES. In pregnant and postpartum patients, PRES should be always considered even with normal blood pressure. Rapid correction of chronic severe anaemia is a rare but preventable cause of PRES.


Stroke ◽  
2020 ◽  
Vol 51 (Suppl_1) ◽  
Author(s):  
Molly Bates ◽  
Kyle Darpel ◽  
Nneka Amadife ◽  
Adam Dugan ◽  
Jessica D Lee

Introduction: Several theories exist regarding the pathogenesis of posterior reversible encephalopathy syndrome (PRES). One theory suggests that PRES occurs when systemic blood pressure exceeds the upper limit of cerebral autoregulation. Endothelial dysfunction has been proposed as an alternative pathogenesis to account for PRES outside the setting of acute hypertension. This mechanism has been implicated in other conditions associated with PRES including autoimmune diseases, cytotoxic medications, sepsis, and eclampsia. The purpose of this study was to determine if COPD, a disease known to cause endothelial dysfunction, has a causative association with the development of PRES. Methods: A single center retrospective, age-matched, case-control study was performed from January 2013 to June 2019 comparing patients discharged with a primary diagnosis of PRES to a control group with acute ischemic stroke. Demographics, medical comorbidities, initial blood pressure, and clinical outcomes were compared between the two groups. For categorical variables, p-values were calculated using χ2 and Fisher’s exact tests. For continuous variables, p-values were calculated using two-sample t-tests. The effect of COPD and acute hypoxic respiratory failure on PRES status was investigated using multivariate logistic regression. Results: A total of 94 PRES subjects and 109 control subjects were included for analysis. Mean age did not differ between the two groups; however, the PRES group was more likely to be female (78.7% vs. 49.5%, p<0.001). COPD was present in 26.6% (n=25) of cases and 11% (n= 12) of controls (odds ratio 4.12, p=0.003). Occurrence of hypertension did not differ significantly between the two groups (78.0% vs 86.2%). Among patients with PRES in the setting of COPD (n=25), 60% (n=16) did not meet criteria for hypertensive emergency. Controlling for hypertensive emergency status in a multivariate logistic regression analysis, patients with COPD were 3.21 times more likely to develop PRES (p= 0.004). Conclusions: To our knowledge, very few reports of PRES in the setting of COPD have been described in the literature and no association of PRES and COPD has been defined to date. Our data support the role of COPD as a risk factor in the development of PRES.


2012 ◽  
Vol 2012 (sep14 1) ◽  
pp. bcr2012006852-bcr2012006852 ◽  
Author(s):  
R. Teotonio ◽  
D. Marmoto ◽  
C. Januario ◽  
C. Bento

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