关键词： Craniofacial   Fibro-osseous malformation   Fibrous dysplasia   Hemorrhage   Optic nerve compression  

摘要： BACKGROUND: Fibrous dysplasia is a rare, benign fibro-osseous malforma-tion whose occurrence in the craniofacial area can result in optic nerve compression, a cerebral mass effect, and cosmetic deformity. Most lesions will progress slowly, and the risk of malignant progression is rare. CASE DESCRIPTION: We present the case of a 21-year-old woman who had presented with acute worsening visual loss secondary to hemorrhagic fibrous dysplasia with ensuing optic nerve compression. Emergent surgical decompression resulted in rapid improvement of her visual dysfunction. The pathological features demonstrated a mixed pattern of woven bone in a fibrous background and secondary aneurysmal bone cyst-like changes. CONCLUSIONS: Hemorrhagic transformation of craniofacial FD remains rare but can present with acute neurologic deterioration. Rapid diagnosis and treatment can allow reversal of patient morbidity. We have also included Supplementary Video 1 to illustrate the surgical principles, and we review the reported data of similar cases.

关键词： Postoperative stroke   Postoperative cognitive decline   Thoracic aortic surgery   Selective antegrade cerebral perfusion  

摘要： Purpose Neurologic complications are seen often after the surgery of the thoracic aorta that uses selective antegrade cerebral perfusion. The objective of this study was to evaluate the impact of atherosclerotic risk factors on neurologic complications in patients who underwent surgery to the thoracic aorta using SCP. Methods Data were collected retrospectively on 94 patients who underwent elective ascending aorta/aortic arch replacement. Concomitant procedures were performed as needed. All patients had magnetic resonance imaging (MRI), angiography (MRA) and carotid ultrasound before surgery. Individual cognitive status was measured using four neuropsychological tests before surgery and 7 days after extubation. We compared perioperative factors for risk factors associated with postoperative stroke and postoperative cognitive decline (POCD). Results 11 patients had strokes after surgery. Operation and extracorporeal circulation times were significantly longer in patients with stroke than those without stroke. Coronary artery disease and SCP time > 150 min were independently associated with postoperative stroke. Of the 83 patients without postoperative stroke, 20 suffered POCD. POCD patients had a significantly higher rate of heterogeneous carotid plaque, and operation time was significant longer in patients with POCD than those without POCD. Independent predictors of POCD were concomitant CABG, heterogeneous carotid plaque, history of cerebrovascular disease and operation time > 450 min. Conclusions We found that prolonged SCP time and coronary artery disease increased the risk of postoperative stroke. Heterogeneous carotid plaque, history of cerebrovascular disease, concomitant CABG and prolonged operation time were further significant predictors of POCD.

关键词： COVID-19   Ageusia   Myalgia   Anticoagulants   Methylprednisolone  

摘要： Emerging Viral diseases are incredibly infectious and proficient in inducing pandemics. Unlike the previous emerging coronaviruses (ECoVs) which neurological complexities were uncommon, with neurological features exhibition at 14-25 days post-onset, yet with critical outcomes exhibiting >50% mortality in central nervous (CNS) presenting pathologies. The COVID 19 neurological consequences occur more frequently even in mild cases, presenting with CNS involvement in up to 25%, musculoskeletal and peripheral manifestation (PNM). Through preceding ECoVs case reports, the PNM not linked to fatal outcomes, however, required, repeated neuro-imaging as notable CT and MRI changes appeared as late as 21 days while the likelihood of Cerebrospinal fluid to test positive for ECoV was 25%, only in the CNS presenting cases. Owing to 44-60% myalgia presentation, risk of the high inflammatory state, and coagulation cascade abnormalities reported in ECoVs, testing for C-reactive protein, serum creatine kinase, and D-dimer level is mandatory. Presently, there is no antiviral medication or vaccination for the ECoVs, early induction of antiviral drugs remains the backbone of management. Neurologically, the therapeutic dosages of anticoagulants are linked to the high incidence of thrombotic complexities, while methylprednisolone is associated with myopathy. Future studies expected to apply more neuro-imaging techniques for CNS exploration and further explore the pathogenesis of the COVID 19 myalgia, anosmia/ageusia reported in the majority of the initial cases. (C) 2020 Elsevier Ltd. All rights reserved.

摘要： Over the past twenty years there have been numerous advances in our understanding of Huntington's disease (HD) and other neurodegenerative proteopathies such as Alzheimer's disease and Parkinson's disease. In each case, disease-specific proteins are expressed and accumulate;what has been less clear is precisely what problems are caused by the accumulation. Recently we have begun to appreciate that increased protein levels or changes in the ratios of different isoforms affect the movement of molecules along the axon, thereby disrupting neuronal function. Huntingtin, the protein involved in HD, plays a special role in axonal transport, and very recent studies have found that its activity - and the movement of its cargoes - is altered not only in HD but in other neurological diseases. Here, we contextualize these studies and consider how modulating huntingtin activity could provide new avenues to therapy.

关键词： Burnout   Compassion fatigue   Narrative medicine  

摘要： Objective Aim of the research was to define the quality of life of Italian neurologists and nurses' professional caring for multiple sclerosis, to understand their living the clinical practice and identify possible signals of compassion fatigue. Material and methods One hundred five neurologists and nurses from 30 Italian multiple sclerosis centres were involved in an online quali-quantitative survey on the organization of care, combined with the Satisfaction and Compassion Fatigue Test and a collection of narratives. Descriptive statistics of the quantitative data were integrated with the results obtained by the narrative medicine methods of analysis. Results Most of the practitioners were neurologists, 46 average years old, 69% women, 43% part time dedicated to multiple sclerosis. An increased number of patients in the last 3 years were referred in 29 centres. Differences were found between neurologists and nurses. Physicians showed higher risks of burnout, reporting intensive working paces, lack of medical personnel, and anxiety caused by the precarious employment conditions. Nurses appeared more satisfied, although the reference to the lack of spaces, and the cross professional roles risk of compassion fatigue. Both positive and negative relationships of care were depicted as influencing the professional quality of life. Conclusion The interviewed neurological teams need to limit the risk of compassion fatigue, which appeared from the first years of the career. The prevalence of the risk among neurologists suggests more awareness among scientific societies and health care managers on the risk for this category, as first step to prevent it.

关键词： Abnormal signs   anatomical localization   hypothesis testing   MRCP   neurological examination   normal signs   quantification of function  

摘要： Clinical examination allows the neurologist to test hypotheses generated by their interpretation of the patient's story. By eliciting abnormal clinical signs, the examining doctor works out a differential diagnosis for the part of the nervous system affected and, using information from the clinical history, a differential diagnosis of the pathology. Clinical examination also allows the clinician to observe and quantify function, hear more story and provide reassurance. The focus of the examination should be dictated by the hypothesis being tested, the patient's clinical state and the situation. Examination of the different parts of the nervous system remains very important in all clinical situations as the best available index of function of the nervous system as a whole. © 2020

关键词： Clinical historian   diagnosis   history   MRCP   neurology   sources   story  

摘要： If clinicians are considered the historians, and patients the story-tellers, the approach to the task of taking ‘the’ history can usefully be informed by the work of academic historians who make explicit the importance of the historian's own views and expertise in their writing of history, and of the ways in which they consult and scrutinize sources. For any bedside diagnosticians – depicted here as clinical historians – their possible sources include the patient, relatives and witnesses, and other healthcare professionals. The information provided needs to be filtered and distilled, like any historical account of a series of events, but is done – typically – in 20–30 minutes in a hospital environment, not over months and years on location or in a university library. This platform of thinking may help to align and enrich the standardized approach to compiling the clinical history, and informs the role – one of many – of the physician as historian. © 2020