关键词： 神经病学   临床   教学   沟通   探索  

摘要： 近年来,医患矛盾已逐渐成为一个社会问题,我国医患矛盾形势越来越严峻,与社会价值观的扭曲、医疗体制机制不完善有密切的联系,而很大一部分则与人文关怀的缺失、医患沟通不到位有关。良好的医患沟通和交流不仅能提升患者满意度和依从性,还能有效避免医患纠纷事件发生。提高临床医师与患者及家属的沟通能力对于构建和谐的医患关系及减少不必要的医疗纠纷具有重要的意义。即将踏上工作岗位的临床医师,在大学理论课期间,只学过医患沟通相关理论知识,缺乏基本的医患沟通能力及技巧。本文结合神经疾病科临床实际,从如何培养一个合格的具备良好医患沟通能力的医师出发,提出更合理化的医学教学方法,希望探索出一套对提高神经疾病科临床医师医患沟通能力有帮助的临床教学方法,将医患沟通纳入常规教学,并推广应用。让学生在掌握理论技能的同时,掌握医患沟通的方法,增强实践性,为临床工作的顺利进行提供保障。

关键词： Leadership   Organized neurosurgery   YNC   Young Neurosurgeons Committee  

摘要： The Young Neurosurgeons Committee, founded in 1991 by Dr. Roberto C. Heros, has been a vital component of the American Association of Neurological Surgeons. It has grown from an opportunity for young neurosurgeons to network with senior neurosurgeons to becoming the major voice of young neurosurgeons and a training ground for the future leaders of organized neurosurgery. The present report has reviewed the leadership involvement of 168 previous Young Neurosurgeons Committee members and briefly highlighted the careers of 4 of these members who have attained leadership positions in neurosurgery.

关键词： attitudes   caring   clinical guidelines   communication   experiences   focus groups   knowledge   nurse   older patients   qualitative approaches  

摘要： Aims and objectives To investigate nurses' experiences of caring for older (65+ years) patients afflicted by delirium in a neurological department. Background Delirium is a frequent, acute and potentially fatal condition. Patients experience delirium as painful and stressful. The literature shows that nursing care is crucial in the prevention and treatment of delirium. The nurses' approach to the patient with delirium is essential for the quality of care provided to the patient. Design A qualitative, exploratory design was used to study the nurses' experiences of caring for patients afflicted by delirium. The study complied with the Consolidated Criteria for Reporting Qualitative Research (COREQ). Methods A total of fourteen nurses participated in three focus groups. The nurses' narratives were used to elucidate their experiences, perceptions, attitudes and views towards the patient with delirium. The texts were analysed using thematic analysis. Results The analysis revealed three themes: The relationship with the patient is challenged, expertise is requested, and barriers to cooperation exist. Conclusions Nurses experienced a relationship with patients with delirium that was influenced by a lack of knowledge about communication with such patients. This resulted in a lack of person-centred care and knowledge about the patient's habitual condition and life story;hence, nurses did not discover the patient's initial delirium. Inadequate and unstructured documentation did the nursing effort initiated late so the patient's delirium was developing. Uncertainty caused lack of dialogue between nurses, which afflicted the partnership and relation between the patient and the nurse. A fast pace workload affected relationships, knowledge, documentation and collaboration. Thus, the person-centred approach to the patient was challenged, which made the provision of professional nursing care difficult. Relevance to clinical practice A continuous focus on patients with delirium, as

关键词： Gaucher   GBA   glucocerebrosidase   neuronopathic   Parkinson  

摘要： The association between Gaucher disease (GD) and Parkinson disease (PD) has been described for almost two decades. In the biallelic state (homozygous or compound heterozygous) mutations in the glucocerebrosidase gene (GBA) may cause GD, in which glucosylceramide, the sphingolipid substrate of the glucocerebrosidase enzyme (GCase), accumulates in visceral organs leading to a number of clinical phenotypes. In the biallelic or heterozygous state, GBA mutations increase the risk for PD. Mutations of the GBA allele are the most significant genetic risk factor for idiopathic PD, found in 5%-20% of idiopathic PD cases depending on ethnicity. The neurological consequences of GBA mutations are reviewed and the proposition that GBA mutations result in a disparate but connected range of clinically and pathologically related neurological features is discussed. The literature relating to the clinical, biochemical and genetic basis of GBA PD, type 1 GD and neuronopathic GD is considered highlighting commonalities and distinctions between them. The evidence for a unifying disease mechanism is considered.

关键词： Cardiac arrest   Prognosis   Risk score   CAHP   OHCA   Cardiopulmonary resuscitation  

摘要： Aim: Several scores are available to predict mortality and neurological outcome in cardiac arrest patients admitted to the intensive care unit (ICU). The aim of the study was to externally validate the prognostic value of four previously published risk scores. Methods: For this observational, single-center study, we prospectively included 349 consecutive adult cardiac arrest patients upon ICU admission. We calculated two cardiac arrest specific risk scores (OHCA and CAHP) and two general severity of illness scores (APACHE II and SAPS II). The primary endpoint was in-hospital mortality. Secondary endpoints were neurological outcome at hospital discharge and 30-day mortality. Results: 170 patients (49%) died until hospital discharge. All scores were independently associated with outcomes in logistic regression analysis and showed acceptable discrimination for in-hospital mortality with highest AUCs of the cardiac arrest specific risk scores (OHCA: 0.80 (95% CI 0.75-0.85) and CAHP: 0.84 (95% CI 0.79-0.88) compared to the severity of illness scores (APACHE II: 0.78 (95% CI 0.73-0.83) and SAPS II: 0.77 (95% CI 0.720.82). Results were robust in subgroup analysis except for worse performance in elderly patients (>75 years) and patients with respiratory cause of cardiac arrest. Results were similar for 30-days mortality and slightly higher for neurological outcome. Conclusions: This study confirms the good prognostic performance of cardiac arrest specific scores to predict mortality and neurological outcomes in cardiac arrest patients. Routine use of OHCA or CAHP score helps to objectively risk stratify these vulnerable patients and thereby may improve therapeutic decisions.

关键词： CX3CL1   Neuroprotective   Neurotoxic   Neuroinflammatory   Neurological disease  

摘要： Fractalkine (CX3C chemokine ligand 1, CX3CL1) is an essential chemokine, for regulating adhesion and chemotaxis through binding to CX3CR1, which plays a critical role in the crosstalk between glial cells and neurons by direct or indirect ways in the central nervous system (CNS). Fractalkine/CX3CR1 axis regulates microglial activation and function, neuronal survival and synaptic function by controlling the release of inflammatory cytokines and synaptic plasticity in the course of the neurological disease. The multiple functions of fractalkine/CX3CR1 make it exert neuroprotective or neurotoxic effects, which determines the pathogenesis. However, the role of fractalkine/CX3CR1 in the CNS remains controversial. Whether it can be used as a therapeutic target for neurological diseases needs to be further investigated. In this review, we summarize the studies highlighting fractalkine/CX3CR1-mediated effects and discuss the potential neurotoxic and neuroprotective actions of fractalkine/CX3CR1 in brain injury for providing useful insights into the potential applications of fractalkine/CX3CR1 in neurological diseases.

关键词： Extracranial-intracranial bypass   Leg plegia   Perfusion  

摘要： While intracranial ischemic insults often result in neuronal death and permanent neurological deficits, some patients may develop potentially reversible neurological dysfunction from persistent hypoperfusion, as surviving neurons remain in an idling state. We report a unique series of three patients with long-standing neurological deficits who underwent EC-IC bypass for repeated TIAs and demonstrated unexpected, rapid resolution of long-standing pre-existing neurological deficits. We suggest that these individuals harbored regions of underperfused, idling neurons that responded rapidly to restored cerebral reperfusion.

关键词： Stroke   Pediatrics   Schimke immuno-osseous dysplasia   Transient ischemic attack   Reversible cerebral vasoconstriction syndrome   Treatment  

摘要： Background: Schimke immuno-osseous dysplasia is a rare autosomal recessive disease resulting from biallelic SMARCAL1 mutations. It presents in early childhood and is characterized by short stature, nephropathy, and immunodeficiency. Approximately 50% of those affected have neurological complications including migraines, transient ischemic attacks, and strokes. Methods: We present a six-year-old boy with Schimke immuno-osseous dysplasia without evidence of atherosclerosis with recurrent episodes of severe headache, fluctuating hemiparesis, and aphasia. Results: Magnetic resonance imaging and angiography were normal during the initial episode;multiple areas of reversible restricted diffusion with decreased perfusion and arterial stenosis were seen with subsequent attacks. Conclusions: This constellation of symptoms and imaging findings is suggestive of reversible cerebral vasoconstriction syndrome, which we propose as a mechanism for the transient ischemic attacks and infarcts seen in some patients with Schimke immuno-osseous dysplasia, as opposed to accelerated atherosclerosis alone. This new insight may provide a basis for novel preventative therapy in this rare disorder. (C) 2018 Elsevier Inc. All rights reserved.

关键词： 25-hydroxyvitamin D   early neurological deterioration   ischemic stroke  

摘要： Background and Aims Vitamin D deficiency has been linked to a higher risk of ischemic stroke. We therefore explored the relationship between serum 25-hydroxyvitamin D [25(OH)D] levels and early neurological deterioration (END) after acute ischemic stroke in a hospital-based prospective study. Methods From June 2016 to June 2018, patients with ischemic stroke within 48 hr from symptom onset were consecutively recruited. Serum 25(OH)D levels were measured at admission. END was defined as an increase of >= 1 point in motor power or >= 2 points in the total National Institute of Health Stroke Scale score within 7 days after admission. Multiple logistic regression models were performed to calculate the odds ratio (OR) and confidence intervals (CI) of 25(OH)D levels in predicting END. Results A total of 478 subjects were enrolled, of which 136 (28.5%) patients developed END. The mean 25(OH)D levels were 49.5 +/- 15.8 nmol/L. Univariate logistic regression analysis showed that advanced age, white matter lesions, high level of body mass index, diastolic blood pressure, fasting blood glucose and homocysteine, and low 25(OH)D levels were associated with END. Furthermore, multivariate regression analysis demonstrated that the first quartile of 25(OH)D concentrations [OR, 2.628;95% CI,1.223-5.644;p = 0.013] was independently risk factor for END. Conclusions This study illustrated that lower 25(OH)D levels might be associated with an increasing risk of END in acute ischemic stroke patients.

关键词： postnatal repair   prenatal diagnosis   spina bifida  

摘要： Objective Parents faced with the choice between postnatal management and prenatal surgery for spina bifida need to have up-to-date information on the expected outcomes. The aim of this study was to report the long-term physical and neurological outcomes of infants with prenatally diagnosed isolated spina bifida that underwent postnatal surgical repair and were managed by a multidisciplinary team from a large tertiary center. Methods This was a retrospective cohort study of all cases of fetal spina bifida managed in a tertiary unit between October 1999 and January 2018. All cases of fetal spina bifida from the local health region were routinely referred to the tertiary unit for further perinatal management. Details on surgical procedures and neonatal neurological outcomes were obtained from institutional case records. Ambulatory status, bladder and bowel continence and neurodevelopment were assessed at a minimum of 3 years. Results During the study period, 241 pregnancies with isolated spina bifida were seen in the unit. Of these, 84 (34.9%) women opted to continue with the pregnancy after multidisciplinary counseling by clinicians. Sixty-seven infants underwent postnatal repair of spina bifida aperta and were included in the analysis. After birth, hindbrain herniation was observed in 91.5% of infants with only seven requiring surgical decompression. Ventriculoperitoneal shunt placement was needed in 64.2% of infants, while normal cognitive development or mild impairment was demonstrated in 85.4% of cases with data for this outcome available, at a mean age of 8 years. Cumulatively, 40% of infants were walking independently or using minor support, and normal or mild impairment of bladder and bowel function was reported in 45.5% and 44.4% of infants, respectively. Conclusions Neurodevelopmental and neurological outcomes between prenatal and postnatal repair are similar. As with fetal surgery, conventional postnatal surgery is associated with the reversal of hindbrain h