Posterior Reversible Encephalopathy Syndrome Secondary to CSF Leak and Intracranial Hypotension: A Case Report and Literature Review

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.

Download Full-text

Posterior reversible encephalopathy syndrome following a thoracic discectomy–induced dural leak: case report

Journal of Neurosurgery Spine ◽

10.3171/2016.4.spine1623 ◽

2016 ◽

Vol 25 (5) ◽

pp. 586-590 ◽

Cited By ~ 9

Author(s):

Lisa B. E. Shields ◽

John R. Johnson ◽

Christopher B. Shields

Keyword(s):

Posterior Reversible Encephalopathy Syndrome ◽

Vasogenic Edema ◽

Brain Mri ◽

Csf Leak ◽

Posterior Reversible Encephalopathy ◽

First Case ◽

Thoracic Spinal ◽

Blood Patch ◽

Dural Leak ◽

Reversible Encephalopathy

Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiological syndrome characterized by headaches, altered mental status, seizures, and visual disturbances. Classic MRI findings include white matter changes of the parieto-occipital regions. This syndrome has been encountered in myriad medical illnesses, including hypertension, preeclampsia/eclampsia, and immunosuppressive conditions. While the pathogenesis of the disorder is unclear, vasoconstriction and hypoperfusion leading to brain ischemia and vasogenic edema have been implicated as potential mechanisms. The authors present, to the best of their knowledge, the first case of PRES following a thoracic spinal surgery–induced dural leak noted on resection of the fifth rib during a thoracotomy for a T4–5 discectomy. Brain MRI revealed large areas of increased FLAIR and T2 hyperintensity in the superior posterior frontal lobes, superior and medial parietal lobes, and bilateral occipital lobes. Following repair of the CSF leak, the patient's symptoms resolved. Spinal surgeons should be alert to the potentially life-threatening condition of PRES, especially in a hypertensive patient who experiences surgery-induced dural leakage. The development of a severe positional headache with neurological signs is a red flag that suggests the presence of PRES. Prompt attention to the diagnosis and treatment of this condition by repairing the dural leak via surgery or expeditious blood patch increases the likelihood of a favorable outcome.

Download Full-text

A rare case of posterior reversible encephalopathy syndrome in a patient with severe leptospirosis complicated with rhabdomyolysis and acute kidney injury; a case report

BMC Infectious Diseases ◽

10.1186/s12879-021-06240-2 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Jayawardane Pathiranage Roneesha Lakmali ◽

Kanapathipillei Thirumavalavan ◽

Danapala Dissanayake

Keyword(s):

Blood Pressure ◽

Acute Kidney Injury ◽

Blood Pressure Control ◽

Posterior Reversible Encephalopathy Syndrome ◽

Vision Loss ◽

Kidney Injury ◽

Pressure Control ◽

Posterior Reversible Encephalopathy ◽

Peripheral Nerve Involvement ◽

Reversible Encephalopathy

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.

Download Full-text

Posterior reversible encephalopathy syndrome following Miller-Fisher syndrome

BMJ Case Reports ◽

10.1136/bcr-2021-242231 ◽

2021 ◽

Vol 14 (7) ◽

pp. e242231

Author(s):

Catarina Bernardes ◽

Cristiana Silva ◽

Gustavo Santo ◽

Inês Correia

Keyword(s):

Intravenous Immunoglobulin ◽

Posterior Reversible Encephalopathy Syndrome ◽

Brain Mri ◽

Clonic Seizure ◽

Miller Fisher Syndrome ◽

Fisher Syndrome ◽

Posterior Reversible Encephalopathy ◽

Fluid Analysis ◽

Immunoglobulin Treatment ◽

Reversible Encephalopathy

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.

Download Full-text

Síndrome da Encefalopatia Posterior Reversível (PRES) no ciclo gravídico puerperal: revisão bibliográfica

Revista Eletrônica Acervo Saúde ◽

10.25248/reas.e4724.2020 ◽

2020 ◽

Vol 12 (11) ◽

pp. e4724

Author(s):

Márcia Cristina Taveira Pucci Green ◽

Felipe Pimenta Rodrigues ◽

Karina Martins Milaré

Keyword(s):

Posterior Reversible Encephalopathy Syndrome ◽

Posterior Reversible Encephalopathy ◽

Posterior Leukoencephalopathy ◽

Reversible Encephalopathy

Objetivo: Elencar as principais características fisiopatológicas, clínicas, diagnósticas e manejo terapêutico da Síndrome da Encefalopatia Posterior Reversível (PRES) em gestantes e puérperas. Métodos: Esta revisão da literatura usou a base de dados de pesquisa CAPES, com os descritores: “PRES”, “Posterior Reversible Encephalopathy Syndrome”, “Posterior Leukoencephalopathy Syndrome” e “Pregnancy”, entre os anos de 2003 a 2019, resultando em 50 artigos que preencheram os critérios de inclusão. Resultados: Verificou-se que, os mecanismos fisiopatológicos que buscam explicar a ocorrência geral dessa síndrome, ainda não são claros. Os principais sinais clínicos identificados foram convulsões associadas a cefaleia e alterações visuais. O exame padrão-ouro é a ressonância magnética, preferencialmente em T1, T2, ADC e DWI, estando a restrição de difusão relacionada a um pior prognóstico. Feito o diagnóstico, é necessário o manejo terapêutico com estabilização da paciente, controles pressórico, controle de convulsões e afastar a causa base que desencadeou o processo da PRES. Considerações finais: A PRES é uma síndrome de início agudo, sendo importante o reconhecimento das suas características clínicas e diagnósticas. Por isso quando pensar em PRES? Sugerimos um recurso mnemônico para lembrar como diagnóstico diferencial. P: Pré-eclâmpsia e eclâmpsia; R: Restrição de difusão na Ressonância Magnética; E: Edema cerebral; S: Sintomas visuais.

Download Full-text

Atypical Posterior Reversible Encephalopathy Syndrome in a Postpartum Woman With Moyamoya Disease: A Case Report and Literature Review

Frontiers in Neurology ◽

10.3389/fneur.2021.696056 ◽

2021 ◽

Vol 12 ◽

Author(s):

Ning Zou ◽

Guixiang Guo ◽

Fangchao Wan ◽

Xin Li

Keyword(s):

Glasgow Coma Scale ◽

Moyamoya Disease ◽

Posterior Reversible Encephalopathy Syndrome ◽

Reversible Posterior Leukoencephalopathy Syndrome ◽

Postpartum Woman ◽

Posterior Reversible Encephalopathy ◽

Coma Scale ◽

Posterior Leukoencephalopathy ◽

Collateral Vessels ◽

Reversible Encephalopathy

Background: Moyamoya disease is a rare cerebrovascular occlusive disease, which is characterized by stenosis and gradual occlusion of the internal carotid arteries, causing the progression of characteristic collateral vessels. To date, most studies investigating moyamoya disease have focused on medical implications, and the potential implications for neurocognitive and/or neuropsychiatric functioning were inconclusive.Case Presentation: we present a case of a 26-year-old Chinese postpartum woman who presented to the emergency department with a 19-h history of cognitive decline, vomiting, and convulsions. Blood pressure, heart rate, and respiration rate were 200/120 mmHg, 115 beats/minute, and 30 breaths/minute, respectively, on arrival. The Glasgow Coma Scale, modified RANKIN scale (mRS), and National Institute of Health stroke scale (NIHSS) scores were 3, 5, and 18, respectively. Moyamoya disease was diagnosed using cerebral angiography and digital subtraction angiography. The cognitive functions of orientation, use of language, ability to calculate, and memory significantly improved after 11 days of treatment (Glasgow Coma Scale: 15; mRS: 0; NIHSS: 0).Conclusions:This patient was diagnosed with reversible posterior leukoencephalopathy syndrome related to moyamoya disease. This case highlights that atypical posterior reversible encephalopathy syndrome can occur in patients with moyamoya disease, and should be considered for the differential diagnosis of cerebral infarcts and hemorrhage in a postpartum female.

Download Full-text

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

BMC Neurology ◽

10.1186/s12883-021-02408-0 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Fatme Seval Ismail ◽

Johannes van de Nes ◽

Ilka Kleffner

Keyword(s):

Posterior Reversible Encephalopathy Syndrome ◽

Clinical Symptoms ◽

Brain Mri ◽

Case Series ◽

Epileptic Seizures ◽

Retrospective Case ◽

Posterior Reversible Encephalopathy ◽

Histopathological Findings ◽

Delay In Diagnosis ◽

Reversible Encephalopathy

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.

Download Full-text

Posterior Reversible Encephalopathy Syndrome Following Diabetic Ketoacidosis: A Case Report

10.21203/rs.3.rs-495918/v1 ◽

2021 ◽

Author(s):

Meng-Ko Tsai ◽

Chao-Hung Lai ◽

Tsung-Ju Chuang

Keyword(s):

Case Report ◽

Diabetic Ketoacidosis ◽

Blood Sugar ◽

Posterior Reversible Encephalopathy Syndrome ◽

Brain Mri ◽

Posterior Reversible Encephalopathy ◽

First Case ◽

Aged Woman ◽

Adequate Hydration ◽

Reversible Encephalopathy

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.

Download Full-text

A case of PRES following intracranial hypotension, with spontaneous resolution with epidural blood patch

International Journal of Reproduction Contraception Obstetrics and Gynecology ◽

10.18203/2320-1770.ijrcog20184992 ◽

2018 ◽

Vol 7 (12) ◽

pp. 5190

Author(s):

Sarada Murali Mamilla

Keyword(s):

Posterior Reversible Encephalopathy Syndrome ◽

Intracranial Hypotension ◽

Sinus Thrombosis ◽

Cerebral Haemorrhage ◽

Clinical Syndrome ◽

Acute Hypertension ◽

Posterior Reversible Encephalopathy ◽

Blood Patch ◽

Clonic Convulsions ◽

Reversible Encephalopathy

Posterior reversible encephalopathy syndrome (PRES (also known as reversible posterior leukoencephalopathy syndrome)) presents with rapid onset of symptoms including headache, seizures, altered consciousness, and visual disturbance. It is often but by no means always associated with acute hypertension. Diffusion-weighted MRI is the most sensitive exam to confirm the diagnosis of PRES. If promptly recognized and treated, the clinical syndrome usually resolves within a week, and the changes seen in magnetic resonance imaging (MRI) resolve over days to weeks. Posterior reversible encephalopathy syndrome is an increasingly recognized disorder, with a wide clinical spectrum of both symptoms and triggers, and yet it remains poorly understood. Differential diagnosis includes venous sinus thrombosis, cerebral haemorrhage, encephalitis, vasculititis and metabolic encephalopathys. No clinical trials have evaluated the management of PRES, but rapid withdrawal of the trigger appears to hasten recovery and to avoid complications: for example, aggressive blood pressure management, withdrawal of the offending drug, or delivery in eclampsia. We report a case of women presenting with severe headache, tonic clonic convulsions 7 days after spinal anaesthesia for caesarean delivery, MRI showed evidence of intracranial hypotension and posterior reversible encephalopathy syndrome. She was treated with Blood patch which lead to complete resolution of symptoms and radiological findings. The possible pathogenetic relationship between intracranial hypotension, secondary to the inadvertent dural puncture, and PRES is discussed. Prompt diagnosis and proper treatment results in complete cure without permanent neurological sequelae.

Download Full-text

Association of Posterior Reversible Encephalopathy Syndrome and Transient Apical Ballooning Syndrome (Takotsubo): First Case Report of a Man and Review of the Literature

Case Reports in Neurology ◽

10.1159/000474933 ◽

2017 ◽

Vol 9 (2) ◽

pp. 173-178 ◽

Cited By ~ 2

Author(s):

Stephan Grimaldi ◽

Emilie Doche ◽

Caroline Rey ◽

Nadia Laksiri ◽

Salah Boussen ◽

...

Keyword(s):

Case Report ◽

Posterior Reversible Encephalopathy Syndrome ◽

Brain Mri ◽

Left Ventricular ◽

Left Ventricular Ejection ◽

Apical Ballooning Syndrome ◽

Ventricular Ejection Fraction ◽

Posterior Reversible Encephalopathy ◽

First Case ◽

Reversible Encephalopathy

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.

Download Full-text