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Key Clinical Message: Posterior Reversible Encephalopathy Syndrome, typically characterized by parieto‐occipital vasogenic edema, can present atypically, as a bilateral symmetrical vasogenic edema in the basal ganglia, featuring the called "lentiform fork sign." Prompt recognition of such variations is crucial for accurate diagnosis and tailored management, highlighting the complexity of this syndrome's manifestations. Posterior Reversible Encephalopathy Syndrome (PRES) manifests as transient neurological symptoms and cerebral edema, commonly associated with immunosuppressive drugs (ISDs) in transplant recipients. ISDs can lead to endothelial dysfunction and compromise the blood–brain barrier. Typically, PRES exhibits identifiable MRI patterns, often demonstrating vasogenic edema in the bilateral parieto‐occipital white matter. Identifying unique presentations, such as the recently observed "lentiform fork sign," commonly seen in uremic encephalopathy, emphasizes this syndrome's broad spectrum manifestations. A 19‐year‐old male, who underwent bilateral lung and liver transplantation, experienced a bilateral tonic–clonic seizure of unknown onset 47 days post‐surgery. MRI findings revealed an unconventional PRES pattern, featuring the "lentiform fork sign" as bilateral symmetrical vasogenic edema in the basal ganglia, surrounded by a hyperintense rim outlining the lentiform nucleus bilaterally. Subsequent management, including ISD modification and magnesium supplementation, resulted in clinical and neuroimaging resolution. An almost complete clinical and radiological resolution was achieved after 14 days. The occurrence of PRES in transplant recipients highlights the intricate interplay among ISDs, physiological factors, and cerebrovascular dynamics, potentially involving direct neurovascular endothelial toxicity and disruption of the blood–brain barrier. Neuroimaging plays a pivotal role in diagnosis. The distinctive "lentiform fork sign" was observed in this patient despite the absence of typical metabolic disturbances. Management strategies usually involve reducing hypertension, discontinuing ISDs, correcting electrolyte imbalances, and initiating antiseizure drugs if necessary. Identifying the presence of the "lentiform fork sign" alongside typical PRES edema in a patient lacking renal failure emphasizes that this manifestation is not solely indicative of uremic encephalopathy. Instead, it might represent the final common pathway resulting from alterations in the blood–brain barrier integrity within the deep white matter. Understanding such atypical imaging manifestations could significantly aid earlier and more precise diagnosis, influencing appropriate management decisions. [ABSTRACT FROM AUTHOR]

Background: Rapidly progressive dementia (RPD) is a syndrome caused by different disease entities. Accurate diagnosis is crucial as numerous proportions of these diseases are highly treatable. There are few RPD series published in South America. Knowing regional data may be useful to establish specific diagnostic guidelines based on local information. The main study objectives are to describe the etiological diagnosis of patients with RPD in Argentina and to compare the clinical characteristics and data obtained from complementary studies among patients with Autoimmune Encephalitis (AE), Prion Diseases (PD), Neurodegenerative Diseases (ND) and Other Causes (OC). Method: A multicenter observational cross‐sectional study was performed with the information obtained from patients referred to the neurology department of the participant centers. Patients with RPD criteria (developing dementia in less than 24 months) were retrospectively included between Dec‐2001 and Jun‐2019. Based on International diagnostic criteria, patients were divided into: AE, PD, ND and OC. We compared baseline clinical data, complementary exams, MRI and serologic characteristics between groups. Parametric or non‐parametric statistic and multivariate analysis was used according to the sample distribution of the available data. Result: On a sample of 328 patients with a diagnosis of RPD, 54.87% were men (n=180) with a mean age of 60.78±5.57 years at admission. The diagnosis time for dementia was 4.06 (0.83–23.6) months. The three most frequent causes were AE 23.17% (n=76), ND 22.86% (n=75) and PD 15.85% (n = 52). In 15.85% of the patients the diagnosis remained unknown. When comparing AE, ND, PD and OC, statistically significant differences were observed in age (younger age for AE, p=0.0001), higher mortality for PD (p=0.0001) and larger time of evolution in ND (p=0.0001). In the PD group more behavioral symptoms (p = 0.002) and pathological electroencephalogram (p=0.001) were found. Conclusion: In accordance with similar series, autoimmune etiology was the more frequent cause. This is the first South American RPD multicentric study. These results might supply valuable data for the healthcare system and future epidemiological investigations more focused diagnostic guidelines. [ABSTRACT FROM AUTHOR]

Tricuspid regurgitation (TR) is the most frequent valvular complication after heart transplantation with different clinical sequelae. In its most severe form, it can cause right heart failure with a poor long-term prognosis. Its management is complex, both medical, surgical, and percutaneous. The TricValve system, a bicaval system with two self-expanding valves (superior vena cava and inferior vena cava), dedicated to treating symptomatic IT refractory to medical therapy, is safe and effective in improving quality of life. We present the first heart transplant patient with severe symptomatic TR who underwent successful bicaval valve (TricValve) implantation.

Introduction Posterior reversible leukoencephalopathy syndrome (PRES) is a clinical-radiological condition characterized by reversible subcortical vasogenic cerebral edema of acute or subacute onset in circumstances that disrupt capillary permeability, unfrequently accompanied by cytotoxic and/or hemorrhagic lesions. We describe a case series of PRES with hemorrhagic features. Subjects and methods Electronic medical records of hospitalized patients diagnosed with PRES from January 2009 to December 2021 were collected. Demographic data, medical history, clinical presentation, and outcome were recorded. Variables were compared between patients with and without hemorrhagic features using the Wilcoxon-Mann-Whitney test with a statistical significance level of p<0.05. Results Over a 12-year period, 33 patients were diagnosed with PRES, of whom 10 had hemorrhagic features: seven cortical microbleeds, two intraparenchymal hematomas, and one subarachnoid hemorrhage. Half of the patients were women, with a median age of 45.8 years (interquartile range (IQR) 21.8), and were admitted for non-neurological reasons. The sample included nine transplant recipients (six solid organa, three bone marrowa), with four patients in the immediate post-transplant period. PRES occurred in the context of infections and blood pressure fluctuations under cytotoxic drugs, such as immunosuppressants. Seventy percent showed improvement/resolution on neuroimaging at a median of 70 days (IQR 62.9). The three major hemorrhages occurred in the context of thrombocytopenia. The recorded in-hospital mortality was 10%. When compared to PRES without hemorrhagic features, patients with hemorrhagic features had a lower use of corticosteroids (50% vs. 78.8%; p=0.02) and a higher presence of restrictive lesions on neuroimaging (60% vs. 17%; p=0.04), with no differences in the other analyzed variables. Conclusion Patients with PRES and hemorrhagic features had a lower use of corticosteroids and a higher presence of restrictive lesions on neuroimaging. Further studies are needed to better understand the clinical implications and management of PRES with hemorrhagic manifestations., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Appiani et al.)