BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) in children has a propensity towards atypical features on magnetic resonance (MR) imaging, with limited literature on perfusion changes and clinicoradiological correlation.
OBJECTIVE: We aimed to comprehensively study MR imaging patterns of pediatric PRES, including cerebral blood flow variations on arterial spin labeling, and looked for any MR biomarkers of poor clinical outcome.
METHODS: In this retrospective observational study conducted in a tertiary hospital setting, MR records over a 4-year period (May 2019 to May 2023) were systematically searched along with their clinical details. Patients with an age less than 18 years and a clinicoradiological constellation consistent with PRES were included. MR scans were analyzed by two neuroradiologists with 8 years\' and 10 years\' experience. Association was sought with poor clinical outcome (defined as modified Rankin Scale score at discharge of > 2).
RESULTS: A total of 45 patients (29 boys) were included in the study, with a mean age (± standard deviation) of 11.19 (± 4.53) years. On MR imaging, 95.6% of patients (n = 43) showed atypical features and/or atypical areas of involvement. The superior frontal sulcus (n = 18) was the most predominant MR pattern, and cerebellar involvement was not uncommon (n = 15). Unilateral involvement (n = 3), isolated central pattern (n = 1), and spinal cord involvement (PRES-SCI: n = 1) were also encountered. Brainstem involvement (n = 4) showed a characteristic \"V-sign\" of anterior medullary hyperintensity. Patchy restricted diffusion (46.6%), punctate hemorrhages (37.7%), and leptomeningeal contrast enhancement (36%) were not uncommon. Arterial spin labeling sequence (available in 24 patients) showed increased cerebral blood flow in the involved areas in 79.2% of patients. Univariate analysis showed a significant association of the presence of hemorrhage (P = 0.003), involvement of brainstem (P = 0.007), deep white matter (P = 0.008), and thalamus (P = 0.026) with poor clinical outcome. Multivariate regression analysis found that hemorrhage on MRI (P = 0.011, odds ratio 8) was an independent factor associated with poor clinical outcome.
CONCLUSIONS: The conventionally described atypical features in PRES are common in children and therefore may no longer be considered exceptions. Raised perfusion on arterial spin labeling sequence was seen in the majority of cases. Hemorrhage on MRI was an independent predictor of poor clinical outcome in pediatric PRES.

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.

Posterior reversible encephalopathy syndrome (PRES) is characterized by nonspecific symptoms, including not only pronounced non-focal and various focal neurological signs but also specific neuroimaging features, including vasogenic edema affecting predominantly the posterior area. PRES usually develops in the setting of acute arterial hypertension. However, it is not uncommon for PRES to develop in non-hypertensive patients, including people with autoimmune disorders (multiple sclerosis, neuromyelitis optica spectrum disorder, etc). PRES could also be due to the toxic effects of drugs or other substances. The pathophysiological mechanisms of PRES include impaired autoregulation of cerebral blood flow due to acute arterial hypertension and toxic endotheliotropic effects of endogenous and exogenous factors.
Синдром задней обратимой энцефалопатии (PRES) характеризуется неспецифической симптоматикой в виде выраженных общемозговых и разнообразных очаговых неврологических симптомов, сопровождается характерными нейровизуализационными изменениями (вазогенный отек, поражающий преимущественно теменно-затылочную область). PRES как правило развивается на фоне острой артериальной гипертензии, однако нередки случаи его развития у пациентов с нормальным артериальным давлением, в том числе и при аутоиммунных расстройствах, включая рассеянный склероз, заболевания спектра оптиконейромиелита, а также вследствие токсического воздействия ряда лекарственных препаратов или иных веществ. Патофизиологические механизмы PRES включают нарушение ауторегуляции мозгового кровотока на фоне острой артериальной гипертензии и токсическое эндотелиотропное воздействие эндогенных и экзогенных факторов.

暂无摘要。

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.