UNASSIGNED: A patient with Sjögren\'s syndrome (SS), immune-mediated thrombotic thrombocytopenic purpura (ITTP), and posterior reversible encephalopathy syndrome (PRES) was reported, and all published cases with thrombotic thrombocytopenic purpura (TTP), PRES, and SS were retrieved and analysed. The patient\'s clinical data and treatment procedure have been discussed.
UNASSIGNED: A 45-year-old Chinese female was hospitalized with headache and low platelet count. She had previously presented to a local hospital with a 7-month history of epigastric discomfort and anorexia, and was diagnosed with SS and ITTP. Laboratory investigations after admission showed platelet (PLT) of 13*10^9/L, red blood cell (RBC) fragments of 6 %, ADAMTS13 Activity＜0.2 %, anti-ADAMTS13 IgG of 88.3U/mL. Brain magnetic resonance imaging (MRI) showed gyriform restricted diffusion along with increased T2-FLAIR signal in the left frontal cortex and bilateral parietal temporal cortex. She was diagnosed with SS, ITTP and PRES, and received the treatment of methylprednisolone, cyclosporine, plasma exchange, IVIG, and rituximab. This patient did not experience the recurrence during the 8-month follow-up period.
UNASSIGNED: ITTP and PRES are rare manifestations of SS. After a suspected or confirmed diagnosis of ITTP, plasma exchange and immunosuppressive therapy should be immediately administered. We suggest that rituximab could have additional therapeutic value for SS combined with ITTP and PRES.

Atypical hemolytic uremic syndrome commonly presents as rapidly progressive renal failure and is histologically characterized by thrombotic microangiopathy (TMA). TMA presenting with acute renal failure requires aggressive medical management. Here, we present a case of a 30-year-old man who presented with a history of accelerated hypertension and a strong family history of end-stage renal disease, in September 2023. Upon evaluation, he was found to have a creatinine level of 2 mg/dl, bland urine and normal-sized kidneys; a renal biopsy revealed chronic interstitial nephritis. Genetic analysis for autosomal dominant tubulointerstitial kidney disease and nephronophthisis yielded negative results. The patient was managed with antihypertensive medications. In January 2024, he was admitted with a history of confusion, headache, and alcohol binge. He had a blood pressure of 200/100 mmHg and had grade 3 hypertensive retinopathy. Laboratory tests revealed anemia with thrombocytopenia, bland urine, normal coagulation parameters, indirect hyperbilirubinemia, normal-sized kidneys on ultrasound, and elevated lactate dehydrogenase levels. MRI of the brain revealed symmetrical hyperintensities in bilateral cerebellum and the dorsal brainstem. Complement levels revealed low C3 levels and genetic analysis revealed a homozygous deletion in the complement factor H-related 3 (CFHR3) gene. The autoantibody for complement factor H was negative. The patient was managed conservatively with adequate blood pressure control. This case highlights the effects of complement dysregulation on the renal tubulointerstitium.

Early screening and management of postpartum posterior reversible encephalopathy syndrome (PRES) can reduce hospital stay and complications. Obstetricians, gynecologists, ophthalmologists, and even general physicians should be aware of PRES since its presentation is variable.

Posterior reversible encephalopathy syndrome (PRES) is a neurological disease characterized by a variety of neurological findings, in accordance with radiological characteristics. PRES is commonly secondary to elevated BP and/or conditions such as autoimmune patients receiving immunosuppressive drugs. Our case involves a 36-year-old female with a history of autoimmune hepatitis (AIH), who presented with sudden onset headaches from 3 weeks prior, and a single episode of seizure attack the morning before admission. In the initial examination she had highly elevated blood pressure (BP) (190/116). Her neurological examination revealed decline in force of limbs in addition to mild paresthesia. After primary stabilization, she underwent brain magnetic resonance imaging. Due to the clinical and radiological findings, the patient was diagnosed with PRES. In the following work-up of BP elevation, abdominopelvic sonography and subsequent computed tomography scan, multiple lesions were observed in spleen and both kidneys consistent with infarction. In further evaluation, Lupus-like anticoagulants were found to be elevated, which, in conjunction with the confirmed antiphospholipid syndrome (APS), suggested a possible role for APS-nephropathy as the missing link between PRES and APS. However, despite the role of an autoimmune disease in increasing the risk of developing other autoimmune conditions, APS and AIH have been rarely observed together. Our study indicates that developing APS in the context of AIH is a rare occurrence. However, APS could serve as a critical intermediary, potentially facilitating the onset of PRES despite lower BP.

Acute intermittent porphyria is a rare autosomal dominant metabolic disorder. It can affect the autonomic, peripheral, and central nervous system. The present study reports on the case of 28-year-old Chinese female patient with posterior reversible encephalopathy syndrome, reversible cerebral vasoconstriction syndrome and myocardial ischemia which have been very rarely reported in patients with acute intermittent porphyria.

UNASSIGNED: Posterior reversible encephalopathy syndrome (PRES) is a medical condition characterized by symptoms such as headache, altered consciousness, visual abnormalities, seizures, and associated radiological vasogenic edema. The condition, as its name suggests, is generally reversible. PRES typically affects individuals around the age of 45 and is rarely seen in the elderly population.
UNASSIGNED: In this report, the authors present the case of an 83-year-old woman who presented to the emergency department with a history of seizures and visual disturbances. Upon examination, her Glasgow Coma Scale (GCS) was 14, and she had bilateral round,reactive, regular pupils. A plain computed tomography (CT) head was performed, which revealed multiple hemorrhagic areas in the occipital region associated with vasogenic edema. magnetic resonance imaging was done which was suggestive of PRES. The individual underwent conservative management in the authors\' institute and gradually recovered.
UNASSIGNED: PRES typically presents in middle-aged individuals, with infrequent occurrences in the elderly population. Early radiological and clinical assessments are crucial for the prompt diagnosis of this condition. Additionally, it is essential to identify predisposing factors such as hypertension, eclampsia, electrolyte imbalances, renal failure, autoimmune diseases, and the use of cytotoxic drugs for optimum management of the patient.
UNASSIGNED: PRES is generally reversible, as its name implies. Maintaining a high level of suspicion based on radiological and clinical correlations, coupled with the effective management of underlying conditions, can contribute to its potential reversibility.

UNASSIGNED: Posterior reversible encephalopathy syndrome (PRES) primarily shows neurological symptoms and is more frequent in males, often occurring in oncological patients. It can also be associated with renal conditions like post-streptococcal glomerulonephritis, a common cause of pediatric hypertension. Management involves blood pressure and seizure treatment. In some cases, it may lead to irreversible and severe complications. Early treatment is essential for prevention.
UNASSIGNED: In the past six months, we have documented the cases of two patients, aged 15 and 10, both of whom presented with PRES and renal disease. These patients were admitted because of general malaise, headaches, nausea, vomiting, visual disturbances, and elevated blood pressure. Subsequently, both patients experienced epileptic episodes. Only the first patient required transfer to the Pediatric Intensive Care Unit (PICU). Cerebral magnetic resonance imaging (MRI) scans revealed distinct PRES lesions in both cases. Following comprehensive investigations, both cases were diagnosed with PRES in the context of acute post-streptococcal glomerulonephritis.
UNASSIGNED: The patients showed improvement following the administration of antihypertensive and anticonvulsant medications, along with treatment for the underlying renal condition.

Posterior reversible encephalopathy syndrome (PRES) is an increasingly recognized clinical entity associated with a variety of medical conditions. It is commonly considered in the presentation of uncontrolled, severe hypertension. However, more recently, it has been described in the setting of blood transfusion, particularly in those with chronic anemia, even in the absence of severe hypertension. We describe a patient who presented to the emergency department 12 days after large blood transfusion for severe, chronic anemia with headache, vision loss, expressive aphasia and a change in mental status, with only mild blood pressure elevation, who was ultimately diagnosed with PRES and refractory non-convulsive status epilepticus. Emergency physicians are often the first to initiate blood transfusion for those with a low hemoglobin. Therefore, it is prudent to proceed with caution in transfusing those with chronic anemia. It is also important for the emergency physician to keep PRES on the differential for those presenting with a neurologic complaint after correction of their chronic anemia, even in the absence of severe hypertension.

Posterior reversible encephalopathy syndrome (PRES) is a rare neurological disease that may be associated with hypertension, autoregulatory failure, and the use of calcineurin inhibitors following heart transplantation (HT). In this article, we present a case series of PRES, discussing its potential causes and management strategies. Among the 126 HT recipients at our hospital, four were diagnosed with PRES. Three of these patients developed PRES within 7 days after HT. Prior to the onset of PRES, all patients experienced sustained hypertension, and strict blood pressure (BP) control was maintained. Three of the four patients recovered without PRES recurrence, while one patient died of sepsis after an episode of altered consciousness. Hypertension was observed in all patients prior to the onset of PRES, and the majority experienced symptom improvement with BP control. While most cases of PRES were reversible with conservative treatment, including the administration of antiepileptics, one irreversible case resulted in in-hospital mortality. Thus, PRES can have serious outcomes and is not invariably benign.

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.