Search Results (278)

Background and Clinical Significance: Guillain–Barré syndrome (GBS) can rarely progress to fulminant paralysis with loss of brainstem reflexes, mimicking coma or brain death despite preserved cortical function. Case Presentation: A 38-year-old man developed rapidly progressive weakness following a diarrheal illness, culminating in quadriplegia, areflexia, respiratory failure, and complete loss of brainstem reflexes within 72 h. Neuroimaging was unrevealing. EEG demonstrated preserved cerebral activity with an alpha coma pattern. Despite initial intravenous immunoglobulin therapy, neurological deterioration continued, prompting escalation to plasma exchange. Gradual recovery of brainstem reflexes and motor function ensued, followed by substantial functional improvement over nine months. This case highlights the diagnostic and prognostic challenges of fulminant GBS at the interface of peripheral and brainstem dysfunction. Neurophysiologic assessment and disciplined exclusion of central etiologies are essential. Timely immunotherapy and supportive care can lead to meaningful recovery even in extreme presentations. Conclusions: Fulminant GBS should be recognized as a potentially reversible cause of apparent coma, underscoring the importance of early diagnosis and aggressive treatment. Full article

Rapidly progressive infectious keratitis may involve the anterior uveal tract and lead to anterior segment inflammation, resulting in severe structural damage of the cornea and potentially causing corneal perforation or endophthalmitis if not promptly treated. We report the case of a 63-year-old male admitted to the Emergency Ophthalmology Department of the University Clinical Center in Katowice, Poland, with a rapidly progressive corneal ulcer of the left eye that had not responded to two weeks of outpatient topical antibiotic therapy. The condition developed after ocular trauma sustained while chopping wood. At presentation, visual acuity was limited to light perception with preserved projection. Multimodal imaging, including slit-lamp examination, anterior segment optical coherence tomography (AS-OCT), and in vivo confocal microscopy, revealed extensive corneal ulceration with severe stromal destruction, progressive corneal melting, and marked anterior segment inflammation, with an imminent risk of perforation. Microbiological cultures identified Pseudomonas aeruginosa. Despite intensive empiric topical antimicrobial therapy targeting both bacterial infection and a possible fungal component related to trauma with organic material, rapid clinical deterioration necessitated emergency therapeutic penetrating keratoplasty (PK). The procedure resulted in rapid resolution of inflammation and improvement in visual acuity, with best-corrected visual acuity (BCVA) reaching 0.3 logMAR during follow-up. At the three-month follow-up, the corneal graft remained clear with stable visual acuity and no recurrence of infection. The patient remains under regular long-term follow-up, with ongoing monitoring of graft clarity, intraocular pressure (IOP), and visual function. This case differs from routine presentations of infectious keratitis by demonstrating exceptionally rapid stromal melting despite promptly initiated empiric topical therapy. Multimodal imaging, particularly AS-OCT provided clinically meaningful information by revealing structural instability and an imminent risk of perforation not fully appreciable on slit-lamp examination, thereby supporting timely urgent keratoplasty. These findings highlight the practical diagnostic value of imaging-based assessment in advanced infectious keratitis and underscore its role in guiding surgical decision-making in eyes at high risk of corneal perforation. Full article

Background: Arrhythmogenic cardiomyopathy (ACM) is an inherited disorder characterized by fibrofatty replacement of cardiomyocytes. The inflammatory episodes of ACM, known as the “hot phase”, can mimic acute myocarditis. It was seldom observed in a DES-associated ACM as a “hot-phase” presentation. Case Presentation: The proband, a 13-year-old female, initially presented with a series of clinical manifestations of fulminant myocarditis. Although recommendation-guided anti-immunotherapy had been provided, this patient still developed into an aggressive cardiomyopathy with biventricular dilation and severe systolic heart failure. Additionally, cardiac magnetic resonance demonstrated circumferential late gadolinium enhancement in left ventricular myocardium with diffuse fibrosis. Whole-exon sequencing identified a de novo missense variant, as c.335T>A (p.L112Q) of the DES gene, resulting in protein dysfunction. And a diagnosis of ACM due to a DES variant had been identified. Finally, this patient received heart transplantation, and biventricular fibrofatty infiltration was confirmed by pathological analysis. Conclusions: This case presented a de novo genetic variant that can induce severe and aggressive heart failure. This finding emphasizes the importance of comprehensive genetic analysis in patients suspected of having fulminant myocarditis, which would greatly benefit the precise clinical management and outcomes. Full article

Background: Giant cell myocarditis (GCM) is a rare condition with an incompletely understood immune pathogenesis, characterized by inflammatory damage to the myocardium and the presence of multinucleated giant cells on histopathological examination. The frequently fulminant and severe course requires rapid intervention for a correct diagnosis and the initiation of immunosuppressive therapy, which is often life-saving. Materials and methods: This article contains information from observational studies and case reports, systematically collected from prestigious publications such as JACC, NEJN, ESC, JCC, Heliyon, and Cureus found in the PubMed and ClinicalTrials.gov databases. Thus, 25 patients diagnosed with giant cell myocarditis between March 2019 and May 2025 were analyzed, with a focus not only on the initial clinical evolution, mortality incidence, and the need for heart transplantation but also on the incidence of major complications such as cardiogenic shock and malignant rhythm and conduction disorders refractory to drug treatment. These parameters were studied according to certain intrinsic factors that cannot be influenced, such as age at onset, gender, and associated pathology of the patient, as well as extrinsic factors that can be influenced, such as the time of diagnosis and the start of immunosuppressive therapy. The results obtained were compared with those in the literature from previous years, considering the limitations of the current study. Results: The selected patients were 13 women (52%) and 12 men (48%), mostly from the US and Japan, aged between 22 and 76 years, with an average age of 44.92 years. An associated autoimmune pathology was found in 40% of patients in this group, and previous cardiovascular pathology in 28%. Only 8% had a history of GCM. The clinical onset of new-onset heart failure, refractory to usual therapy, with progressive dyspnea as the cardinal symptom was found in 12 patients, representing 48% of cases; palpitations as an expression of rhythm or conduction disorders were found in five patients, representing 20%; precordial discomfort to precordial pain accompanied or not by ST-T segment changes was present in four patients, representing 16%; and general signs and symptoms or those of other organs were present in three (12%) cases. The diagnosis was made by histological examination of the biopsy fragment obtained by endomyocardial biopsy or from the myocardial fragment obtained during the implantation of mechanical cardiovascular support devices and, less frequently, on the explanted heart and at autopsy. In terms of progression, of the 25 patients, four (16%) died, four (16%) required heart transplantation, and 16 (64%) had a severe progression with cardiogenic shock, which required mechanical circulatory support in 11 (44%) cases. The outcome was mainly influenced by the early diagnosis and administration of immunosuppressive medication, but also by the age of the patients and associated chronic diseases. Conclusions: Giant cell myocarditis is a serious condition that, in the absence of rapid diagnosis and appropriate immunosuppressive therapy, has a fulminant, often fatal course. Clinical suspicion of giant cell myocarditis remains important in the initial diagnosis. Raising this suspicion, together with modern and improved paraclinical investigations compared to previous years, has led to faster diagnosis and administration of immunosuppressive therapy in this pathology. Histological examination remains the gold standard for final diagnosis, but it should be noted that it may be non-diagnostic. In the face of a strong suspicion of giant cell myocarditis, the best approach is to start immunosuppressive therapy and monitor the patient’s progress. Immunosuppressive treatment remains decisive in influencing the evolution of this condition, both through prompt administration and through the adaptation of therapeutic regimens to the evolution of patients. A more detailed understanding of the immune-mediated pathogenesis of GCM and the identification of clinical risk factors for unfavorable short- and long-term outcomes may enable earlier risk stratification and the development of more targeted, individualized therapeutic strategies. Full article

Background/Objectives: Primary amoebic meningoencephalitis (PAM) is a rare, fulminant, and often fatal central nervous system infection caused by the opportunistic free-living amoeba Naegleria fowleri. Although Naegleria species are widely present in freshwater and soil worldwide, human disease is associated specifically with pathogenic N. fowleri rather than the many nonpathogenic environmental species, and virulence may vary across N. fowleri isolates. This systematic review aimed to synthesize contemporary global data from 2000 to 2024 to identify recent trends in epidemiology, clinical presentation, diagnosis, treatment, and outcomes. Methods: A systematic literature search was conducted across PubMed, Scopus, and the Cochrane Library, identifying 58 eligible publications encompassing 66 individual cases. Results: Most reports originated from the United States, India, and China. The median patient age was 14 years, with 78% of cases occurring in males. Annual case reports increased from one per year (2000–2005) to over four per year (2020–2024), reflecting either a true rise in incidence or improved detection. Common presenting symptoms included fever, headache, and altered mental status. Diagnosis was confirmed via polymerase chain reaction (PCR) testing or post-mortem biopsy in nearly one-third of cases. Treatment regimens varied, with amphotericin B and miltefosine being the most frequently used agents. Overall mortality was 83%, with survival strongly associated with early initiation of combination therapy. Pediatric patients had a higher survival rate (22%) compared to adults (7.1%). Conclusions: The findings highlight the need for heightened clinical awareness, especially in the context of climate-driven ecological changes that may expand N. fowleri’s geographic range. This review underscores critical gaps in surveillance and diagnostics and emphasizes the importance of a One Health approach to addressing emerging threats like PAM. Further research into novel therapeutics, rapid diagnostics, and global case reporting systems is urgently needed. Full article

Myocarditis is a complex inflammatory myocardial disease. Although traditionally regarded as exclusively immune-mediated, recent evidence highlights the significant role of underlying genetics on susceptibility, phenotypic variability, and long-term prognosis. This narrative review examines the evolving genetic architecture of myocarditis and its relationship to inherited cardiomyopathies, integrating mechanistic insights from molecular, imaging, and clinical studies. Variants in desmosomal genes such as desmoplakin (DSP) and plakophilin-2 (PKP2) are increasingly linked to recurrent myocarditis that may evolve into arrhythmogenic cardiomyopathy, supporting the concept of a genetically predisposed myocardium in which inflammatory stressors can act as triggers. Truncating variants in titin (TTN) and Filamin C (FLNC) are associated with fulminant or dilated phenotypes. Conversely, mutations in Lamin A/C (LMNA), Desmin (DES), and BCL2-Associated Athanogene 3 (BAG3) contribute to inflammatory myocardial remodeling and other forms of inherited cardiomyopathies. These findings collectively have the potential to redefine myocarditis as an inflammatory disorder influenced by genetic factors. Furthermore, advancements in genetic testing and multi-omics approaches show promise in enhancing diagnostic accuracy and informing management strategies. Full article

Background: Sinonasal mucosal melanoma is a rare and aggressive malignancy arising from the nasal cavity and paranasal sinuses, characterized by high local recurrence rates and poor survival. Skull-base and orbital progression can occur rapidly, particularly when preoperative imaging underestimates local extension. This paper reports a case of sinonasal mucosal epithelioid melanoma with fulminant postoperative skull-base breach and orbital invasion, highlighting its clinical course, management challenges, and histopathological features. Methods: A 60-year-old woman with progressive unilateral nasal obstruction, recurrent epistaxis, and headache underwent clinical evaluation, contrast-enhanced head MRI, CT, and PET-CT staging. Preoperative imaging demonstrated no intracranial or orbital invasion. Biopsy confirmed mucosal epithelioid melanoma with high proliferative activity (Ki-67 ~80–85%). The patient underwent extensive image-guided endoscopic resection with intraoperative cerebrospinal fluid leak repair. Results: Definitive histopathology confirmed pigmented epithelioid melanoma with extensive necrosis, bone invasion, and non-assessable resection margins due to specimen fragmentation (pT4a, Rx). Within two weeks postoperatively, CT and MRI demonstrated extensive local recurrence with cribriform plate destruction, anterior skull-base dural infiltration, and rapid orbital progression with optic nerve compression and loss of vision. Despite hemorrhage control and hypofractionated palliative radiotherapy (VMAT, 33 Gy in 11 fractions), the patient experienced progressive neurological decline, refractory pain, and recurrent tumour bleeding, and died approximately 4.5 months after initial presentation. Conclusions: In patients with sinonasal mucosal epithelioid melanoma, fulminant local progression with skull-base and orbital involvement may occur despite apparently limited preoperative imaging. When rapid vision loss, dural infiltration, and refractory nasal bleeding develop, structured palliation, hemorrhage control, and aggressive multimodal analgesia should be prioritized early alongside ongoing multidisciplinary decision-making. Full article

Background Expanded dengue syndrome represents a severe and atypical spectrum of dengue virus infection characterized by multisystem involvement beyond classic manifestations. While mild gastrointestinal symptoms are common in classic dengue, expanded dengue syndrome may present with clinically significant digestive organ involvement, including hepatitis, fulminant hepatic failure, pancreatitis, and acute acalculous cholecystitis. These manifestations often resemble primary gastrointestinal diseases, leading to diagnostic delays and inappropriate management. Despite increasing recognition, the true frequency of digestive system involvement remains poorly defined due to heterogeneous reporting and limited quantitative evidence. Methodology/Principal Findings A systematic review and meta-analysis was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines and registered in the International Prospective Register of Systematic Reviews (PROSPERO; CRD420251270772). MEDLINE, Scopus, Web of Science, Embase, CENTRAL, Scielo, and BIREME were searched from inception to December 10, 2025. Primary studies reporting laboratory-confirmed dengue infection with atypical digestive system involvement and sufficient quantitative data were included. Seven studies comprising 1774 participants met eligibility criteria. Random-effects meta-analyses were performed to estimate pooled frequencies of gastrointestinal manifestations. The pooled frequency of hepatic involvement was 7% (95% confidence interval: 0–21), including fulminant hepatic failure (3%) and hepatitis (33%), with substantial heterogeneity. Acute pancreatitis occurred in 3% (95% confidence interval: 0–11) of cases. Acute acalculous cholecystitis was the most frequent manifestation, with a pooled frequency of 21% (95% confidence interval: 3–48). All included studies were classified as low risk of bias. Full article

Background: Neonatal sepsis is a bloodstream infection and constitutes a major cause of morbidity and mortality among newborns. The diagnosis is primarily based on a positive blood culture result. An additional diagnostic and therapeutic challenge may be congenital infection, particularly in preterm infants. This is associated with the lack of routine screening of the maternal genital tract, except for testing for group B streptococci during earlier stages of an uncomplicated pregnancy. Case presentation: This paper presents a case of a neonate with early-onset sepsis (EONS) caused by Escherichia coli. Methods: We carried out analysis of documents combined with the case report method. Results: Preterm birth associated with PPROM constitutes a significant risk factor for early-onset neonatal sepsis (EONS). Conclusions: The case illustrates the dynamic course of congenital infection and the resulting need for immediate, life-saving interventions. It also demonstrates abnormalities identified in ancillary investigations involving both the visual system and the central nervous system (CNS), confirming the intrauterine onset of the infectious process. Full article

Background: Fulminant type 1 diabetes (FT1D) is a rare but life-threatening subtype of type 1 diabetes. The concurrence of FT1D with myocarditis is uncommon and attracts further clinical attention. Case Presentation: A 33-year-old female was transferred by a local hospital to West China Hospital because of altered consciousness, abrupt onset of hyperglycemia with ketoacidosis, significantly increased cardiac biomarkers, and ST segment elevations. Her random blood glucose at the local hospital was 50.19 mmol/L. Insulin infusion and fluid resuscitation were started immediately before referral. On admission, her random blood glucose was 14.17 mmol/L. HbA1C and glycosylated albumin (GA) were 6.3% and 21.45%, respectively. Her fasting C-peptide level was 0.022 nmol/L. Anti-Glutamic Acid Decarboxylase (anti-GAD) antibody was 25.06 IU/mL. FT1D was diagnosed based on the 2012 New Diagnosis Criteria of FT1D. Electrocardiogram showed significant ST segment elevation in leads II, III, aVF, and V3-V6. Echocardiography revealed a mildly reduced left ventricular ejection fraction (LVEF) of 46%. Coronary angiography displayed no abnormality. Cardiac magnetic resonance imaging revealed areas of increased signal intensity in the interventricular septum, basal and mid inferolateral walls, and apical inferior wall and subepicardial late gadolinium enhancement (LGE), particularly in the lateral aspects of the left ventricle on T2-weighted imaging (T2WI). Acute myocarditis was diagnosed based on the European Society of Cardiology 2013 Task Force Criteria. She was treated with insulin, fluid resuscitation, and supportive care, leading to rapid recovery of ketoacidosis and cardiac function. At the four-month follow-up, she remained on insulin therapy with good glycemic control but persistent low C-peptide levels. Conclusion: This case report raises awareness about FT1D, determines the differential diagnosis of acute cardiac presentations in an FT1D patient, and highlights clinical reasoning so that clinicians can recognize and manage similar presentations on time. Full article

Hepatitis D virus (HDV) is a very peculiar virus that shares many characteristics with plant viroids. One of its unique characteristics is the requirement for the presence of a helper virus for its replication, and in particular enveloping its virion, a role often played by the hepatitis B virus (HBV). Infection with HDV is frequently associated with more severe disease, which may present with fulminant hepatitis or a more rapid progression to cirrhosis and hepatocellular carcinoma (HCC), when compared to HBV mono-infection. HDV exhibits many peculiarities and enigmas, which have led to it being considered a neglected virus. This review aims to identify the most important gaps in knowledge and peculiarities in the study of this enigmatic virus, from virology to clinical implications. Full article

(1) Background: The clinical course of acute myocarditis in adolescents is heterogeneous, and reliable predictors of early functional changes remain limited, particularly in patients without severe systolic dysfunction. Routine hematologic parameters may reflect the early inflammatory response, but their prognostic relevance in pediatric non-fulminant myocarditis is poorly defined. This exploratory study aimed to assess whether admission inflammatory blood indices are associated with short-term changes in left ventricular systolic function in adolescents with acute myocarditis. (2) Methods: We retrospectively analyzed 44 adolescents (median age 16 years, 84% male) hospitalized with suspected acute non-fulminant myocarditis between 2020 and 2023. All patients had preserved or mildly reduced left ventricular ejection fraction (LVEF) at presentation. Clinical, laboratory, electrocardiographic, and echocardiographic data obtained at admission were analyzed. Changes in LVEF between the acute and post-acute phases during hospitalization were assessed using transthoracic echocardiography. Cardiac magnetic resonance imaging was performed in a subset of patients to support diagnosis but was not uniformly available for quantitative analysis. (3) Results: No in-hospital deaths occurred. A modest positive correlation was observed between neutrophil count at admission and improvement in LVEF during hospitalization (r = 0.348, p = 0.028). No significant associations were found between LVEF change and white blood cell count, lymphocyte count, monocyte count, neutrophil-to-lymphocyte ratio (NLR), troponin I, or NT-proBNP. (4) Conclusions: In adolescents with non-fulminant acute myocarditis and preserved or mildly reduced systolic function, admission neutrophil count was associated with short-term improvement in left ventricular ejection fraction. Given the retrospective design, limited sample size, and absence of mechanistic data, these findings should be interpreted as hypothesis-generating. Further prospective studies incorporating standardized cardiac magnetic resonance imaging and immunologic profiling are needed to clarify the clinical significance of this association. Full article

(1) Background: Malignant arrhythmia complicating fulminant myocarditis is associated with high in-hospital mortality, but evidence regarding its long-term prognosis and specific risk factors is limited. (2) Methods: This single-center retrospective cohort study (2016–2025) analyzed 241 consecutive fulminant myocarditis patients, stratified by malignant arrhythmia status (n = 58 vs. 183). The malignant arrhythmia group was further subclassified into malignant tachyarrhythmia (n = 22) and bradyarrhythmia (n = 36). Endpoints included major adverse cardiovascular events (MACE), cardiac dysfunction, and structural abnormalities. (3) Results: At 3-month follow-up, malignant arrhythmia patients had a significantly higher incidence of MACE compared to non-malignant arrhythmia patients (15.5% vs. 4.9%, p = 0.008), but no significant differences were found in cardiac dysfunction or structural abnormalities. Multivariate analysis identified low triglyceride level as an independent risk factor for in-hospital malignant tachyarrhythmia. For in-hospital malignant bradyarrhythmia, independent risk factors were delayed, such as intrinsicoid deflection, low diastolic blood pressure, bradycardia, and an elevated E/Em ratio, with the predictive model showing high discriminatory power. (4) Conclusions: Malignant arrhythmia is an independent predictor of adverse short-term, but not long-term, prognosis in fulminant myocarditis patients, with distinct risk factor profiles identified for malignant tachyarrhythmia and malignant bradyarrhythmia subtypes. Full article

Congenital toxoplasmosis presents with a wide clinical spectrum, ranging from asymptomatic infection to severe disease with multi-organ failure. We report a rare fatal case of disseminated congenital toxoplasmosis in a human neonate. The infant initially had thrombocytopenia and mild hepatitis, which rapidly progressed to fulminant liver failure. Despite initiation of standard therapy with pyrimethamine, sulfadiazine, and folinic acid on postnatal day 25, the infant died two days later. Autopsy revealed widespread involvement of the liver, spleen, brain, heart, lungs, urinary bladder, and skeletal muscle. To further characterize the infection, genomic sequencing of the isolate (TgHsUS2) was performed, which placed it within clade C (Haplogroup 9) and closely related to reference strains P89 and TgCatBr3. Variant analysis showed type III-like alleles in ROP18, ROP16, and GRA15. These alleles are known to modulate host immunity and may have influenced disease severity in this case. This report highlights the need for rapid recognition and targeted therapy as well as how strain genomics can inform disease mechanisms. Prevention through prenatal screening and maternal treatment during pregnancy may reduce infant mortality. Full article

Background/Objectives: Guidelines recommend combination therapy with oral vancomycin and intravenous (IV) metronidazole for fulminant Clostridioides difficile infection (CDI). Although patients with severe CDI are often managed with combination therapy, evidence supporting this practice remains limited. This study was performed to compare the clinical outcomes of vancomycin monotherapy versus combination therapy in patients with severe CDI. Methods: We conducted a multicenter, retrospective, observational cohort study including adult patients with severe CDI who received oral vancomycin between January 2017 and May 2021. Patients were classified as receiving combination therapy if IV metronidazole was administered for at least 72 h within 48 h of initiating oral vancomycin; otherwise, they were classified as receiving vancomycin monotherapy. The primary outcome was a composite of inpatient all-cause death or colectomy within 60 days after CDI diagnosis. The secondary outcomes were the clinical cure rate, CDI recurrence rate, time to discharge after CDI diagnosis, and duration of ICU admission. Results: In total, 215 patients were included, with 100 (46.5%) receiving combination therapy. There were no significant differences in in-hospital mortality or colectomy between the monotherapy and combination therapy groups (25.2% vs. 26.0%, p = 1.00). Recurrence rates (19.1% vs. 16.8%, p = 0.81), total length of stay (31.0 vs. 23.0 days, p = 0.16), and ICU stay duration (35.0 vs. 32.0 days, p = 0.89) were also similar. However, the clinical cure rate was significantly higher in the monotherapy group than in the combination therapy group (79.1% vs. 65.0%, p = 0.03). Conclusions: Combination therapy with oral vancomycin and IV metronidazole was not associated with improved clinical outcomes in patients with severe CDI. Prospective randomized studies are needed to clarify optimal management strategies for severe CDI. Full article