Successful Treatment with Mycophenolate Mofetil in a Patieint with Anti-MDA 5 Antibody-positive Interstitial Lung Disease and Posterior Reversible Encephalopathy syndrome: A Case Report.

A 59-year-old woman presented with a rash on the top part of her hands and pain in the wrist joint and was diagnosed with dermatomyositis complicated by interstitial pneumonia positive for anti-melanoma differentiation-associated gene 5 (MDA-5) antibody. However, the patient reported a severe headache following treatment with oral prednisolone and tacrolimus. Posterior reversible encephalopathy syndrome (PRES) was diagnosed based on the brain magnetic resonance imaging findings. Tacrolimus was discontinued, and mycophenolate mofetil was instead administered with a favorable outcome. Mycophenolate mofetil should therefore be considered as an alternative treatment for anti-MDA-5-positive interstitial lung disease in cases where calcineurin inhibitors cannot be used.

Electrifying Consequences: Posterior Reversible Encephalopathy Syndrome After an Electric Shock Injury.

Reversible posterior leukoencephalopathy syndrome, often referred to as posterior reversible encephalopathy syndrome (PRES), is a disorder characterized by acute cerebral dysfunction and is seen in conjunction with vasogenic edema on brain imaging. Headaches, visual issues, seizures, abnormal mentation, disturbances in awareness, and focal neurological symptoms are its defining features. In this case report, we present a 40-year-old male patient who developed PRES after experiencing a high-voltage electric shock.

Posterior reversible encephalopathy syndrome post stem cell transplantation in sickle cell disease: case series and literature review.

Introduction: Posterior reversible encephalopathy syndrome (PRES) is a serious neurological syndrome that may develop following immunosuppressive therapy for stem cell transplantation (SCT). We report 8 patients with sickle cell disease (SCD) who developed PRES, which is likely to be related to immunosuppression. Methods: This is retrospective cohort analysis of the SCD registry at the King Faisal Specialist Hospital and Research Center (KFSHRC) in Riyadh, Saudi Arabia. Inclusion criteria included all adults SCD patients who underwent SCT from 2011 until 2022. We explored all cases of PRES in patients with SCT. PRES was diagnosed with MRI imaging showing reversible vasogenic cerebral edema associated with neurological symptoms including severe headache, seizures, encephalopathy, delirium, and visual disturbances. Results: During ten years follow-up (2011-2022) we found 8 patients with PRES (age range between 14 to 37 years at diagnosis) PRES occurred 8 to 124 days following SCT in 7 cases and one patient developed PRES 8 months prior to SCT. All patients were on immunosuppressive medications, including tacrolimus, cyclosporine, sirolimus and or mycophenolate mofetil. Headache, seizures, visual hallucinations, confusion, and drowsiness were the most common presenting symptoms. MRI showed abnormalities in the occipital, parietal and frontal lobes in most cases. Recovery was complete in all patients and no recurrences were noted. Two patients had graft versus host disease (GVHD). We compared risk factors for PRES among the 8 cases and 136 SCT in SCD patients who did not develop PRES. There was a significant association between PRES and imaging abnormalities, including previous bi-hemispheric infarctions (p = 0.001), and cerebral microbleeds (CBMs). PRES was strongly associated with presence (p = 0.006), size (p = 0.016) and number (p = 0.005) of CMBs. Conclusion: PRES can develop days to weeks following SCT in patients with SCD, and is associated with immunosuppressive therapy, previous bi-hemispheric infarctions and CMB. Prompt recognition and intervention leads to good recovery.

Posterior reversible encephalopathy syndrome in immunocompromised children - A single-center study from South India.

This study describes the profile of children diagnosed with posterior reversible encephalopathy syndrome (PRES) in the pediatric hematology oncology unit and highlights the clinical features of PRES in immunosuppressed children. This retrospective study included 10 children diagnosed with PRES with a mean age of 6.8 years. Acute lymphoblastic leukemia was the most common primary diagnosis followed by post-hematopoietic stem cell transplant patients. Most cases of PRES occurred within one month of treatment initiation. Hypertension was noted in all at the time of diagnosis. Neuroimaging revealed bilateral lesions with parietal and occipital lobe involvement being the most common. All patients received corticosteroids as part of treatment for primary diagnosis. Controlling blood pressure was critical in managing PRES. Consideration of PRES as a clinical possibility in pediatric hematology oncology unit in children presenting with symptoms such as headache, seizures, and visual disturbances will aid in early diagnosis after ruling out other causes of these symptoms.

Hemolysis, Elevated Liver Enzymes and Low Platelet Count Syndrome Complicated by Disseminated Intravascular Coagulation and Posterior Reversible Encephalopathy Syndrome: A Case Report.

A 22-year-old pregnant woman was transferred from an external medical facility after experiencing an eclamptic seizure linked to hemolysis, elevated liver enzymes and low platelet count syndrome (HELLP) syndrome, and posterior reversible encephalopathy syndrome (PRES). Her situation was further complicated by intrauterine fetal demise and disseminated intravascular coagulation, necessitating a comprehensive multidisciplinary approach. This report details the diagnostic process and challenges in managing this complex patient with diverse medical requirements. Emphasis is placed on the observed hemostatic abnormalities, and we delineate the nuances in our approach compared to managing a similar condition in a nonpregnant patient. Heightened awareness among healthcare professionals is imperative for prompt diagnosis and effective intervention in such uncommon neurological complications during pregnancy.

PRESenting a Challenge: Posterior Reversible Encephalopathy Syndrome in Pediatric Patients With Guillain-Barré Syndrome: A Case Series and Review of Literature.

BACKGROUND: Guillain-Barré syndrome (GBS) is an autoimmune disorder characterized by demyelination of peripheral nerves. GBS-associated posterior reversible encephalopathy syndrome (PRES) is a rare and potentially life-threatening complication in the pediatric population. We aimed to report and analyze the clinical features, management, and outcomes of three cases of GBS-associated PRES in our setting in the light of the existing literature. METHODS: Medical records of 75 pediatric patients with GBS were reviewed for autonomic changes and GBS-associated PRES. Thirty-one developed dysautonomia while three were identified to have PRES. Clinical, radiological, laboratory, and treatment data were collected and analyzed. RESULTS: All three patients were male and presented with symptoms of acute flaccid paralysis and respiratory distress requiring mechanical ventilation. All three patients experienced various complications, including hypertension, seizures, and hyponatremia, and were subsequently diagnosed with PRES. Multimodal intensive care resulted in patient improvement and discharge in an ambulatory state after an average of 104 days of care. CONCLUSIONS: GBS-associated PRES is a rare and potentially life-threatening complication that can occur in pediatric patients with GBS. Our findings suggest that early recognition, prompt intervention, and multimodal intensive care can improve patient outcomes. Further studies are needed to determine optimal treatment strategies for GBS-associated PRES.

Clinical and Radiological Features of Unilateral Posterior Reversible Encephalopathy Syndrome (PRES): A Case Report.

Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiological entity characterized by reversible vasogenic edema predominantly affecting the posterior regions of the cerebral hemispheres. However, unilateral presentation of PRES is an exceptionally rare manifestation. We describe the case of a 34-year-old woman who presented with left-sided hemiparesis, hemisensory loss, headache, and focal motor seizures. Brain CT revealed right anterior and posterior hypodensities in the right frontal and parietal subcortical locations, brain MRI showed vasogenic edema in the subcortical right cerebral hemisphere, and cerebral angiogram revealed diffuse narrowing of the left internal carotid artery just distal to the carotid bifurcation with no flow of contrast beyond the ophthalmic segment. The patient's symptoms resolved during her admission, MRI findings improved on repeated imaging, and she was ultimately diagnosed with unilateral PRES. Unilateral PRES is a complex and challenging diagnosis, and this case sheds light on the atypical radiological features of unilateral PRES possibly intricately linked with contralateral steno-occlusive disease of the carotid artery. It is essential to maintain the atypical variants of PRES as part of the differential diagnosis when encountering acute neurological symptoms and vasogenic edema on imaging in the context of contralateral steno-occlusive disease of the carotid artery.

Exploring the Impact of Neurophysiotherapy in Managing Leukoencephalopathy Challenges: A Case Report.

Leukoencephalopathy (LE), characterized by structural changes affecting cerebral white matter, presents a complex clinical picture with diverse etiologies. This case report details the presentation, clinical findings, and physiotherapy management of a 32-year-old female with colony-stimulating factor 1 receptor (CSF1R)-related leukoencephalopathy and a history of diabetes and hypertension. She suddenly stopped her medications, which led to the worsening of her condition. She presented with symptoms of headache, slurred speech, visual disturbances, cognitive impairment, and impaired balance and coordination, due to which her activities of daily living were affected. The symptoms highlighted the challenges and multidisciplinary approach required for its management. The patient exhibited neurological deficits, cognitive decline, and abnormal reflexes, with magnetic resonance imaging (MRI) revealing white matter abnormalities. Outcome measures demonstrated significant improvements in cognitive and functional abilities, emphasizing the effectiveness of tailored rehabilitation in managing the complexities of colony-stimulating factor 1 receptor-related leukoencephalopathy. A six-week physiotherapy rehabilitation program addressed various domains, including strength training, task-specific exercises, errorless learning, facial muscle retraining, balance exercises, visual restoration therapy, and mobility training. All these interventions effectively improved her functional capacity and made the patient independent in performing activities of daily living.

Posterior Reversible Encephalopathy Syndrome Presenting as Delirium With Psychosis and Agitation in the Postpartum Period.

Posterior reversible encephalopathy syndrome (PRES), which was first described in 1996, is a neurologic condition characterized by a combination of clinical and neuroimaging findings. PRES may arise in the context of preeclampsia, eclampsia, renal failure, and sepsis, among other conditions. Neuropsychiatric symptoms of PRES include altered mental status, agitation, and in some cases psychosis. PRES occurring in the postpartum period is understudied, especially with regard to its psychiatric manifestations. We aim to add to the literature a case of PRES associated with psychosis and agitation in a postpartum woman, highlighting clinical implications and offering suggestions for practice. A female in her late 20s, with no significant psychiatric or medical history, presented to the hospital at 29 weeks and one day of gestation following a witnessed seizure. She was found to be hypertensive and hyponatremic, was diagnosed with eclampsia, and underwent an emergent cesarean section due to fetal malpresentation. The next day, the patient developed paranoia with acute agitation, and the psychiatry team diagnosed her with delirium with psychosis/agitation secondary to her underlying medical condition. She required intramuscular medications for agitation, was placed in restraints, and was transferred to the ICU for sedation. Subsequently, CT and MRI scans of her head both indicated that she had developed PRES. The patient's delirium and psychotic behavior resolved after appropriate treatment of her eclampsia. To our knowledge, this case report is the second documented case in the literature, of a patient who presented with PRES characterized by agitation and psychotic features in the postpartum period. Due to the significant overlap in symptoms between delirium and postpartum psychosis, this case highlights the crucial importance of interdisciplinary collaboration for accurate diagnosis and prompt treatment of PRES in the postpartum period. The case also speaks to the importance of differentiating postpartum psychosis associated with a primary psychiatric disorder from delirium arising in postpartum patients with or without a previous psychiatric history.

Unveiling the Hidden Culprit of Posterior Reversible Encephalopathy Syndrome Superimposing Postpartum Eclampsia: A Rare Case.

The rare yet potentially fatal neurological complication known as posterior reversible encephalopathy syndrome (PRES) can manifest during pregnancy. Alongside symptoms such as headaches, nausea, visual disturbances, and altered mental status, patients often experience seizures or loss of consciousness. Imaging typically reveals vascular edema affecting the parietal and occipital lobes within the subcortical region. We present the case of a 24-year-old patient who developed postpartum eclampsia followed by PRES. MRI findings demonstrated hyperintensities in the posterior parietal, frontal, and occipital lobes bilaterally, confirming the diagnosis. Prompt administration of levetiracetam and labetalol led to the resolution of the patient's symptoms. Subsequently, we thoroughly searched online databases for peer-reviewed articles examining the etiology, clinical presentation, and treatment options for PRES. Our evaluation of the case findings alongside existing literature underscored the rarity of PRES occurring concurrently with postpartum eclampsia, highlighting the importance of timely identification and intervention in managing this condition. Further research is warranted to enhance our understanding of PRES in the context of pregnancy-related complications.

Hemorrhagic Posterior Reversible Encephalopathy Syndrome in a Pediatric Patient With Acute Lymphoblastic Leukemia: A Case Report.

Posterior reversible encephalopathy syndrome (PRES) is an uncommon yet severe neurological disorder characterized by a combination of clinical and radiological features. Common clinical presentations of PRES include headaches, seizures, altered mental status ranging from lethargy to coma, visual disturbances, and behavior changes.  This case report outlines the occurrence of hemorrhagic PRES in an 11-year-old girl with B-cell acute lymphoblastic leukemia (ALL) relapse. Hospitalized for ALL relapse, the patient underwent reinduction chemotherapy. On the ninth day of admission, she had a generalized tonic-clonic seizure with a blood pressure peak of 170/120 mmHg. Magnetic resonance imaging (MRI) and a seizure episode suggested PRES. Initially, after the first tonic-clonic seizure, the neurological examination was normal, but after the second seizure, the meningeal symptoms were negative, and gaze palsy and right-sided homonymous hemianopsia were observed; muscle strength was symmetrically reduced in the upper and lower extremities and reflexes were symmetrical and diminished. A bilateral Babinski reflex was observed at the time of examination; the patient had mild motor aphasia, and she opened her eyes only in response to tactile stimulation. A follow-up MRI four days after the second seizure episode showed extensive PRES damage with hemorrhagic changes. Over two weeks, the patient's neurological status and blood pressure gradually improved, with persistent changes in the visual field. Subsequent MRI revealed a significant reduction in PRES lesions, but residual hemorrhage measuring 6x4 cm remained evident.

Posterior reversible encephalopathy syndrome: a rare cause of seizures following non-transplant cardiac surgery.

Posterior reversible encephalopathy syndrome (PRES) is rarely reported as a cause for seizures following cardiac surgery. PRES in non-transplant cardiac surgery may reflect under-diagnosis and under-reporting. While the condition is reversible, a delay in diagnosis can lead to irreversible brain injury. We describe a case of PRES that occurred after aortic valve replacement with concomitant coronary artery bypass grafting.

Posterior reversible encephalopathy syndrome associated with antibiotic therapy: a case report and systematic review.

Posterior reversible encephalopathy syndrome (PRES) is an acute neurological condition associated with different etiologies, including antibiotic therapy. To date, most data regarding antibiotic-related PRES are limited to case reports and small case series. Here, we report a novel case description and provide a systematic review of the clinico-radiological characteristics and prognosis of available cases of PRES associated with antibiotic therapy. We performed a systematic literature search in PubMed and Scopus from inception to 10 January 2024, following PRISMA guidelines and a predefined protocol. The database search yielded 12 subjects (including our case). We described the case of a 55-year-old female patient with PRES occurring one day after administration of metronidazole and showing elevated serum neurofilament light chain protein levels and favorable outcome. In our systematic review, antibiotic-associated PRES was more frequent in female patients (83.3%). Metronidazole and fluoroquinolones were the most reported antibiotics (33.3% each). Clinical and radiological features were comparable to those of PRES due to other causes. Regarding the prognosis, about one third of the cases were admitted to the intensive care unit, but almost all subjects (90.0%) had a complete or almost complete clinical and radiological recovery after prompt cessation of the causative drug. Antibiotic-associated PRES appears to share most of the characteristics of classic PRES. Given the overall good prognosis of the disease, it is important to promptly diagnose antibiotic-associated PRES and discontinue the causative drug.

A Novel Adult Case of Recurrent Acute Pancreatitis Caused by Hypercalcemia With Concurrent Manifestation of Posterior Reversible Encephalopathy Syndrome Revealing Undiagnosed Primary Hyperparathyroidism due to an Underlying Parathyroid Adenoma.

Background: Hypercalcemia-induced posterior reversible encephalopathy syndrome (PRES) is a rare entity primarily associated with iatrogenic vitamin D/calcium overdose, malignancy, or, infrequently, primary hyperparathyroidism. Case Report: We present a novel case of an adult male from rural India who experienced recurrent acute pancreatitis caused by hypercalcemia with concurrent manifestation of PRES. Diagnostic evaluation revealed markedly elevated serum calcium levels and parathyroid hormone concentrations, consistent with primary hyperparathyroidism. Imaging studies identified a parathyroid adenoma near the right thyroid lobe, subsequently surgically excised. Discussion: This case underscores the importance of considering primary hyperparathyroidism as an underlying cause of PRES, especially in the absence of acute arterial hypertension or autonomic dysfunction. Early recognition and intervention are essential in mitigating the morbidity and mortality of PRES.

Preeclampsia With Posterior Reversible Encephalopathy Syndrome at 19 Weeks Gestation Resulting in Intrauterine Fetal Demise.

Posterior reversible encephalopathy syndrome (PRES) can be defined as a clinical syndrome of headache, seizures, visual disturbance, altered mental status, and characteristic magnetic resonance imaging (MRI) findings of vasogenic edema in the posterior subcortical parietal-occipital white matter. There are numerous potential inciting factors, including immunosuppression, renal disease, malignancy, cytotoxic medications, hypertension, preeclampsia, and eclampsia. In this paper, we present the case of a 21-year-old female at 19 weeks gestation presenting with symptoms consistent with preeclampsia with severe features and PRES. She was transferred to our facility after initial stabilization. She had an atypical course of preeclampsia prior to 20 weeks gestation, PRES lacking seizure activity, and ultimately her case resulted in intrauterine fetal demise (IUFD) at 20 weeks and six days gestation. As indicated by its name, PRES is considered a fully reversible syndrome, and the patient recovered after stabilization of her hypertensive disorder and delivery of the fetus. This case illustrates the importance of prompt recognition and treatment of hypertensive disorders in pregnant patients and the possibility of complications that can result in significant morbidity and mortality for both the mother and fetus.

A Rare Case of Postpartum Paraplegia: A Case Report.

In this case report, a 27-year-old woman who had pre-eclampsia in the past and had a cesarean section as a result of the condition presents with an uncommon and difficult form of postpartum paraplegia. She experienced bilateral lower limb paralysis and urine incontinence soon after the surgery, which quickly led to unconsciousness and required mechanical ventilator support and intensive care treatment. Comprehensive diagnostic testing, which included magnetic resonance imaging scans of the brain and spinal cord, identified signs typical of "Posterior Reversible Encephalopathy Syndrome (PRES)" and spinal cord infarction affecting segments C3 to D2. "Antiphospholipid Antibody Syndrome (APLA)" was identified by laboratory testing, highlighting the significance of taking a thorough approach to comprehending this uncommon clinical condition. Treatment included anticoagulant therapy, high-dose steroid therapy, and antihypertensive drugs, emphasizing the crucial importance of inter-disciplinary care in handling such complex situations. Even if the patient's symptoms have partially improved, their condition is still being closely monitored in the intensive care unit. In the context of postpartum neurological problems and the complex interplay between pre-eclampsia, spinal cord infarction, and related clinical symptoms, this case emphasizes the need for increased awareness and prompt management.

Posterior Reversible Encephalopathy Syndrome With Hemorrhagic Transformation in the Postoperative Period of a Kidney Transplant.

Patients with end-stage renal disease (ESRD) who undergo kidney transplantation are at an increased risk of developing surgical and/or medical complications. Posterior reversible encephalopathy syndrome (PRES) is a rare complication that occurs in 0.34% of kidney transplant patients. It is characterized by a combination of neurological manifestations, risk factors, and characteristic radiological findings in neuroimaging studies. The development of PRES has been associated with various medical conditions and factors, including hypertension, the use of cytotoxic and immunosuppressive drugs, acute or chronic kidney disease, pre-eclampsia/eclampsia, autoimmune diseases, and solid organ and bone marrow transplantation. This report presents the case of a 19-year-old woman diagnosed with ESRD on hemodialysis due to lupus nephritis who experienced an episode of PRES with intraparenchymal hemorrhage during the postoperative period of kidney transplantation. The case emphasizes the importance of closely monitoring these patients during this period to enable early diagnosis and timely treatment of complications, ensuring a favorable prognosis.

Perioperative Cerebrovascular Accidents in Spine Surgery: A Retrospective Descriptive Study and A Systematic Review with Meta-Analysis.

Introduction: Perioperative cerebrovascular accidents (CVAs) related to spine surgery, although rare, can lead to significant disabilities. More studies on spine surgeries are required to identify those at risk of perioperative CVAs. The characteristics and outcomes of patients that experienced CVAs during spine surgery were assessed through a retrospective descriptive study and meta-analysis. Methods: Patients aged ≥18 years who underwent spine surgery under general anesthesia at a hospital between April 2011 and March 2023 were examined. Of the 2,391 initially identified patients, 2,346 were included after excluding 45 who underwent debridement for surgical site infections. Subsequently, a meta-analysis including the present retrospective descriptive study was conducted. Databases such as PubMed and Google Scholar were searched for original peer-reviewed articles written in English. Results: Of the 2,346 patients, 4 (0.17%) (three men, one woman) exhibited perioperative CVAs associated with spine surgery. The CVAs were diverse in nature: one case of cerebral hemorrhage resulting from dural injury during posterior occipitocervical fusion, two cases of cerebral infarctions after lumbar laminectomy and anterior thoracic fusion due to anticoagulant discontinuation, and one case of posterior reversible encephalopathy syndrome following microscopic lumbar discectomy due to gestational hypertension. The subsequent meta-analysis included three studies (n=186,860). It showed several risk factors for perioperative CVAs, including cervical level (pooled odds ratio [OR]=1.33), hypertension (pooled OR=2.27), atrial fibrillation (pooled OR=8.78), history of heart disease (pooled OR=2.47), and diabetes (pooled OR=2.13). Conclusions: It was speculated that the potential risk factors for the four perioperative CVA cases of spine surgery in this retrospective descriptive study were intraoperative dural injury, preoperative anticoagulant discontinuation, and gestational hypertension history. The meta-analysis revealed that cervical spine surgery, hypertension, atrial fibrillation, heart disease, and diabetes increased the CVA risk. This highlights the need for risk assessment, preoperative optimization, and postoperative care to reduce spine surgery-associated perioperative CVAs.

Posterior Reversible Encephalopathy Syndrome in a Late Postpartum Patient With a Rare Complication of Subarachnoid Hemorrhage.

Posterior reversible encephalopathy syndrome (PRES) is considered a neuroclinical syndrome of headache, confusion, visual changes, and seizures associated with neuroimaging findings of posterior cerebral white matter edema. Although the incidence of the syndrome is largely unknown, this condition is becoming increasingly recognized. The prognosis is generally good with most symptoms resolving within one week and lesions on imaging resolving in two weeks. Death and significant neurological disability have been reported but are relatively rare. In this report, we present a 10-day postpartum patient with an atypical history of headache and seizure-like activity. Neuroimaging revealed findings consistent with PRES as well as a rare complication of subarachnoid hemorrhage. This case highlights the importance of clinicians considering preeclampsia/eclampsia-induced PRES when encountering a postpartum patient with headache and hypertension to further reduce morbidity and mortality in this patient population.