背景:多系统萎缩(MSA)是一种进行性,无法治愈,以猝死风险为独特特征的危及生命的神经退行性疾病,这使得神经科医生的诊断交付具有挑战性。关于打破MSA诊断的实证研究很少,目前没有制定指导方针。本研究旨在调查神经学家在提供MSA诊断方面的当前实践和经验。
方法:我们进行了一项多中心在线调查,并采用了混合方法(定量和定性)研究设计,其中使用关键事件技术对开放式问题的回答进行定性分析。
结果:在接受调查的194位神经科医生中,166人开始了调查(回复率=85.6%),其中日本各地区的144名受访者完成了调查。因此,92.3%和82.8%的参与神经科医师认为提供MSA的诊断和解释猝死的风险是困难的。分别。与诊断交付困难独立相关的因素包括解释家庭决策过程在延长生命治疗中的重要性,在提供有关猝死风险的信息方面感知到的困难,以及MSA鉴别诊断中的困难。
结论:我们的研究结果表明,大多数神经科医生认为要诊断MSA并解释猝死的风险是困难的,这可能与打破MSA诊断的困难有关。在MSA中传达坏消息的困难是由各种因素引起的,例如由MSA的进行性和无法治愈的性质引起的神经科医师的移情负担,需要解释复杂而重要的细节,包括家庭决策过程在延长生命治疗中的重要性,MSA诊断困难,以及患者或其家庭成员的精神状态和认知障碍造成的沟通障碍。神经学家在解释猝死风险时考虑各种因素(例如,病人的个性,精神状态,以及接受和理解的程度)并调整他们的沟通方式,例如限制他们在此类问题上的交流,或避免在疾病早期使用“猝死”一词。虽然神经学家努力达到良好实践的基本标准,有多方面的改进空间。
BACKGROUND: Multiple system atrophy (MSA) is a progressive, incurable, life-threatening neurodegenerative disease uniquely characterized by the risk of sudden death, which makes diagnosis delivery challenging for neurologists. Empirical studies on breaking a diagnosis of MSA are scarce, with no guidelines currently established. This study aimed to investigate neurologists\' current practices and experiences in delivering the diagnosis of MSA.
METHODS: We conducted a multicenter online survey and employed a mixed-methods (quantitative and qualitative) study design in which responses to open-ended questions were analyzed qualitatively using critical incident technique.
RESULTS: Among the 194 neurologists surveyed, 166 opened the survey (response rate = 85.6%), of whom 144 respondents across various Japanese regions completed the survey. Accordingly, 92.3% and 82.8% of the participating neurologists perceived delivering the diagnosis of MSA and explaining the risk of sudden death as difficult, respectively. Factors independently associated with difficulties in diagnosis delivery included explaining the importance of the family decision making process in life-prolonging treatment, perceived difficulties in delivering information regarding the risk of sudden death, and perceived difficulties in differential diagnosis of MSA.
CONCLUSIONS: Our findings showed that the majority of neurologists perceived delivering the diagnosis of MSA and explaining the risk of sudden death as difficult, which could have been associated with the difficulty of breaking the diagnosis of MSA. Difficulty in conveying bad news in MSA are caused by various factors, such as empathic burden on neurologists caused by the progressive and incurable nature of MSA, the need to explain complex and important details, including the importance of the family decision-making process in life-prolonging treatment, difficulty of MSA diagnosis, and communication barriers posed by mental status and cognitive impairment in patients or their family members. Neurologists consider various factors in explaining the risk of sudden death (e.g., patient\'s personality, mental state, and degree of acceptance and understanding) and adjust their manner of communication, such as limiting their communication on such matters or avoiding the use of the term \"sudden death\" in the early stages of the disease. Although neurologists endeavor to meet the basic standards of good practice, there is room for the multiple aspects for improvement.