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The distinct clinical and radiological characteristics of right temporal variant FTD have only been recently recognized. METHODS: Eight patients with right temporal variant FTD were prospectively recruited and underwent a standardised neuropsychological assessment, clinical MRI, and quantitative neuroimaging. RESULTS: Our voxelwise grey analyses captured bilateral anterior and mesial temporal grey matter atrophy with a clear right-sided predominance. Bilateral hippocampal involvement was also observed, as well as disease burden in the right insular and opercula regions. White matter integrity alterations were also bilateral in anterior temporal and sub-insular regions with a clear right-hemispheric predominance. Extra-temporal white matter alterations have also been observed in orbitofrontal and parietal regions. Significant bilateral but right-predominant thalamus, putamen, hippocampus, and amygdala atrophy was identified based on subcortical segmentation. The clinical profile of our patients was dominated by progressive indifference, decline in motivation, loss of interest in previously cherished activities, incremental social withdrawal, difficulty recognising people, progressive language deficits, increasingly rigid routines, and repetitive behaviours. CONCLUSIONS: Right temporal variant FTD has an insidious onset and may be mistaken for depression at symptom onset. It manifests in a combination of apathy, language, and behavioural features. Quantitative MR imaging captures a characteristic bilateral but right-predominant temporal imaging signature with extra-temporal frontal and parietal involvement.
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Introduction and importance: Macrophage activation syndrome (MAS) is a severe form of hemophagocytic lymphohistiocytosis that is frequently associated with either a flare-up of rheumatologic diseases, or infection and is characterized by intermittent fever, organomegaly, and multisystem dysfunction. Early diagnosis and treatment are crucial for outcome improvement. Case presentation: The authors present a 9-year-old male with systemic onset juvenile idiopathic arthritis who presented with fever, vomiting, and nose bleeding, as well as being jaundiced, and having hepatomegaly and ascites. Pancytopenia, hepatic dysfunction, and elevated ferritin levels were discovered, along with negative virological and immunological tests. He was given broad-spectrum antibiotics, and a high-dose steroid showed a good response, and he was discharged about a week later. Clinical discussion: It is hypothesized that decreased natural killer cells' function could lead to the inability to clear the infection, and subsequent lymphocytes-induced macrophages activation. Despite being beneficial in this case, steroids led to no improvement in other similar cases. Conclusion: MAS is a real life-threatening complication for patients with systemic Juvenile idiopathic arthritis (sJIA), and early diagnosis and prompt initial treatment can both offer a favourable result against such syndrome.
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BACKGROUND: Electrical storm (ES) is a life-threatening condition, associated with substantial early and subacute mortality. Catheter ablation (CA) is a well-established therapy for ES. However, data regarding the impact of CA on the short-term and midterm survival of patients admitted for ES remain unclear. OBJECTIVES: This multicenter study aimed to investigate the impact of CA of ES on survival outcomes, while accounting for key patient characteristics associated with treatment selection. METHODS: A propensity score-matching (PSM) analysis was performed on 780 consecutive patients admitted for ES in 4 tertiary centers. PSM (1:1) based on the main characteristics associated with the use of CA or medical therapy alone was performed, resulting in 2 groups of 288 patients. RESULTS: After PSM, patients who underwent CA (n = 288) and those treated with medical therapy alone (n = 288) did not present any significant differences in the main demographic characteristics, ES presentation, and management. Compared with medical therapy alone, CA was associated with a significantly lower rate of ES recurrence at 1 year (5% vs 26%; P < 0.001). Similarly, CA was associated with a higher 1-year (91% vs 81%; P < 0.001) and 3-year (78% vs 71%; P = 0.017) survival after discharge. In subgroup analyses, effect of ablation therapy remained consistent in patients older than 70 years of age (HR: 0.39; 95% CI: 0.24-0.66), with substantial efficacy in patients with a LVEF <35% (HR: 0.39; 95% CI: 0.27-0.59). CONCLUSIONS: In propensity-matched analyses, this large study shows that CA-based management of patients admitted for ES is associated with a reduction in mortality compared with medical treatment, particularly in patients with a low ejection fraction.
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Future construction projects will need the implementation of industry 4.0 and Internet-of-Things (IoT) technologies. The construction sector has, however, falling behind other industries in the application of these technologies and is currently facing considerable challenges. One of the industries that lag behind in the use of new innovative technological tools is the construction industry. This study reviews the research work in industry 4.0 and the Internet of Things as they relate to construction and examines key Ghana-based construction professionals and firms to ascertain their level of understanding of these emerging innovative technologies, including the challenges and benefits associated with their implementation. An extensive review of pertinent literature was done to help identify the important paradigms and variables which were cautiously tested. Adopting a quantitative research approach, the attained variables were used to design into a close-ended questionnaire. The sample frame was a survey of people from 154 construction experts and researchers with good standing by using the purposive sampling. Relative importance index (RII) analysis was used to analyzed the data. It was discovered from the findings that smart construction was the most popular industry 4.0 technology in the Ghanaian construction industry. The most important benefit of these technologies is that they will add sustainable policy requirements to tendering, with the most pressing technology being the lack of talent and skills in using industry 4.0 and IoT technologies. The scope of this research is based on the questionnaire survey, proving a sustainable pathway to the construction industry community, which creates its own significance by including key stakeholders and those affected by these technologies.
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Construction Industry , Internet of Things , Humans , Ghana , Internet , TechnologyABSTRACT
Neurobrucellosis (NB) is a rare complication of the most common zoonotic infection worldwide. Meningitis and encephalitis are the most common manifestations. While it is endemic in many countries, it is frequently misdiagnosed due to its nonspecific presentations, and it requires a high index of suspicion and special care to be cured. Case presentation: The first case is from a rural area and presented with a prolonged fever associated with profuse sweating, which was complicated later by headache and sudden left-sided weakness associated with urine incontinence without signs of meningeal irritation. Laboratory and radiological tests confirmed the diagnosis of NB after other cerebral infections were ruled out, and the patient received the full brucella regimen and recovered well. The second patient presented with a gradual onset of fever that did not respond to conventional treatment. Days later, his condition was complicated by a convulsion that was not preceded by an aura and was not accompanied by symptoms of weakness, increased intracranial pressure, or sphincteric disturbances. He has a history of drinking raw milk, and tests for brucella were positive, ruling out other intracranial infections and masses. He received full brucella treatment and showed good recovery. Conclusion: A patient from an endemic area who has a prolonged fever and neurological symptoms should be considered positive for NB until it is ruled out.