Posterior reversible encephalopathy syndrome following Miller-Fisher syndrome

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.

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.


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.


2013 ◽  
Vol 12 (3) ◽  
pp. 341-343
Author(s):  
Giordano Rafael Tronco Alves ◽  
Isadora Cristina Olesiak Cordenonsi ◽  
Régis Vinícius de Andrade Silva ◽  
Carlos Jesus Pereira Haygert

Posterior reversible encephalopathy syndrome (PRES) comprises a unique pattern of brain vasogenic edema that is seen in the setting of a neurotoxic status. Besides many etiologies have been already associated with PRES development, such as chronic renal disease, use of chemotherapy agents and inflammatory conditions, the imaging features are very suggestive and helpful for an appropriate diagnosis. We report here a case of PRES secondary to post-streptococcal glomerulonephritis (PSGN), which evolved successfully after clinical management. An 11-year-old boy was admitted with a typical history and findings of PSGN, associated with sensory alterations, headache and recent tonic-clonic seizure. Computed tomography (CT) scan of the head has revealed bilateral and symmetric hypodense areas, remarkably located at posterior cerebral regions, indicating PRES. Patient received support therapy with diuretics, and antibiotics prescription after discharge. At ambulatory follow-ups, the patient remains asymptomatic, with complete clinical and radiological improvement.Bangladesh Journal of Medical Science Vol. 12 No. 03 July ’13 Page 341-343 DOI: http://dx.doi.org/10.3329/bjms.v12i3.13323


2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
Hiroto Hirashima ◽  
Masahiro Iwamoto ◽  
Tadashi Ozawa ◽  
Shigeyoshi Kijima ◽  
Shigeki Matsubara ◽  
...  

Posterior reversible encephalopathy syndrome with stroke is very rare in puerperal women. A 36-year-old nulliparous woman with both rheumatoid arthritis and recurrent pregnancy loss, probably due to a high titer of anti-phospholipid IgM antibody, was referred at 10 weeks of gestation. Low-dose aspirin at 100 mg/day and heparin calcium subcutaneous injection at 10,000 units/day were started before pregnancy and stopped at 35+6 and 40+2 weeks, respectively. She transabdominally delivered a male infant weighing 3,344 g at 40+5 weeks. A tonic-clonic seizure abruptly occurred without either hypertension or proteinuria 5 days after delivery. Intracerebral hemorrhage involving an area of 2 cm in diameter in the right frontal lobe and subarachnoid hemorrhage with PRES were confirmed. Seizure recurred 2 days after the initial episode. She showed severe headache and mild disturbance of consciousness but no neurological findings. We suggested that a high titer of anti-phospholipid IgM antibody might be associated with stroke.


2019 ◽  
Vol 6 ◽  
pp. 2329048X1985677 ◽  
Author(s):  
Omar Abdel-Mannan ◽  
Luigi D’Argenzio ◽  
Matthew Pitt ◽  
Felice D’Arco ◽  
Sanjay Bhate ◽  
...  

We describe 2 pediatric cases presenting with posterior reversible encephalopathy syndrome secondary to autonomic dysfunction preceding the onset of motor symptoms in Guillain-Barré syndrome variants. Patient 1 presented acutely with encephalopathy, cerebellar signs, hypertension, lower limb weakness, and respiratory decompensation. Magnetic resonance imaging (MRI) brain showed occipital lesions consistent with posterior reversible encephalopathy syndrome. Nerve conduction studies were consistent with Miller-Fisher syndrome. After intravenous immunoglobulin and plasmapheresis, he improved clinically with radiological resolution. Patient 2 presented with headache, leg pain, seizures, and significant hypertension. Brain MRI was normal but spine MRI revealed enhancement of the cauda equina ventral nerve roots. She was areflexic with lower limb weakness a few days after intensive care unit admission and made a significant improvement after treatment with intravenous immunoglobulin. In children presenting with posterior reversible encephalopathy syndrome in the absent of other causes of primary hypertension, Guillain-Barré syndrome variants are an important differential etiology, presenting with autonomic dysfunction, even before signs of motor weakness become evident.


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.


2017 ◽  
Vol 9 (2) ◽  
pp. 173-178 ◽  
Author(s):  
Stephan Grimaldi ◽  
Emilie Doche ◽  
Caroline Rey ◽  
Nadia Laksiri ◽  
Salah Boussen ◽  
...  

Introduction: An association of posterior reversible encephalopathy syndrome (PRES) and takotsubo is rare. We present the first case of a male patient. Case Report: A 69-year-old man presented to the hospital in a persistent comatose state following a generalized tonic-clonic seizure with high blood pressure. The electrocardiogram revealed transient left bundle branch block. Troponin and BNP were elevated. Cardiac ultrasound showed large apical akinesia with altered left ventricular ejection fraction, and the left ventriculogram showed characteristic regional wall motion abnormalities involving the mid and apical segments. Brain MRI showed bilateral, cortical, and subcortical vasogenic edema predominant in the posterior right hemisphere. The lumbar puncture and cerebral angiography were normal. Paraclinical abnormalities were reversible within 2 weeks with a clinical recovery in 3 months, confirming the takotsubo and the PRES diagnoses. Discussion: Several theories hypothesize the underlying pathophysiology of takotsubo or PRES. Circulating catecholamines are up to 3 times higher in patients with takotsubo causing impaired microcirculation and apical hypokinesia. An association of both takotsubo and asthma crisis and PRES and asthma crisis underlines the role of catecholamines in the occurrence of these disorders. Conclusion: Early recognition of this rare association, in which heart and neurological damage may require rapid intensive care support, is needed.


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