OBJECTIVE In this population-based study, we aimed to determine whether neuropsychiatric history, medication or family history of neuropsychiatric disorders predicted cognitive and/or behavioural impairment in motor neuron disease (MND). METHODS People with MND (pwMND) on the Scottish Clinical, Audit, Research and Evaluation of MND (CARE-MND) register, diagnosed from January 2015 to January 2018, with cognitive and/or behavioural data measured using the Edinburgh Cognitive and Behavioural ALS Screen were included. Data were extracted on patient neuropsychiatric, medication and family history of neuropsychiatric disorders. We identified patients with cognitive impairment (motor neuron disease with cognitive impairment (MNDci)), behavioural impairment (motor neuron disease with behavioural impairment (MNDbi), both (motor neuron disease with cognitive and behavioural impairment (MNDcbi)) or motor neuron disease-frontotemporal dementia (MND-FTD). RESULTS Data were available for 305 pwMND (mean age at diagnosis=62.26 years, SD=11.40), of which 60 (19.7%) had a neuropsychiatric disorder. A family history of neuropsychiatric disorders was present in 36/231 (15.58%) of patients. Patient premorbid mood disorders were associated with increased apathy (OR=2.78, 95% CI 1.083 to 7.169). A family history of any neuropsychiatric disorder was associated with poorer visuospatial scores, MNDbi (OR=3.14, 95% CI 1.09 to 8.99) and MND-FTD (OR=5.08, 95% CI 1.26 to 20.40). A family history of mood disorders was associated with poorer overall cognition (exp(b)=0.725, p=0.026), language, verbal fluency and visuospatial scores, and MND-FTD (OR=7.57, 95% CI 1.55 to 46.87). A family history of neurotic disorders was associated with poorer language (exp(b)=0.362, p<0.001), visuospatial scores (exp(b)=0.625, p<0.009) and MND-FTD (OR=13.75, 95% CI 1.71 to 110.86). CONCLUSION Neuropsychiatric disorders in patients and their families are associated with cognitive and behavioural changes post-MND diagnosis, with many occurring independently of MND-FTD and C9orf72 status. These findings support an overlap between MND, frontotemporal dementia and neuropsychiatric disorders, particularly mood disorders.

中文翻译：

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神经精神疾病与MND认知和行为变化之间的关系。

目的在这项基于人群的研究中，我们旨在确定神经精神病史的神经精神病史，药物治疗或家族史是否可预测运动神经元疾病（MND）的认知和/或行为障碍。方法在苏格兰临床，审核，研究和评估MND（CARE-MND）登记册上诊断为MND（pwMND）的人，于2015年1月至2018年1月被诊断，其认知和/或行为数据使用爱丁堡认知和行为ALS屏幕进行测量被包括在内。提取有关患者神经精神病学，药物治疗和神经精神病家族史的数据。我们确定了患有认知障碍（患有认知障碍的运动神经元疾病（MNDci）），行为障碍（患有行为障碍的运动神经元疾病（MNDbi），（认知和行为受损的运动神经元疾病（MNDcbi））或运动神经元疾病额颞痴呆（MND-FTD）。结果可获得305 pwMND的数据（诊断时的平均年龄= 62.26岁，SD = 11.40），其中60例（19.7％）患有神经精神疾病。有36/231（15.58％）位患者存在神经精神疾病家族史。患者病前情绪异常与冷漠感增加有关（OR = 2.78，95％CI 1.083至7.169）。任何神经精神疾病的家族史均与较差的视觉空间评分，MNDbi（OR = 3.14，95％CI 1.09至8.99）和MND-FTD（OR = 5.08，95％CI 1.26至20.40）相关。情绪障碍的家族史与较差的整体认知度（exp（b）= 0.725，p = 0.026），语言，言语流畅性和视觉空间得分以及MND-FTD（OR = 7.57，95％CI 1.55至46.87）相关。神经系统疾病的家族史与较差的语言（exp（b）= 0.362，p <0.001），视觉空间得分（exp（b）= 0.625，p <0.009）和MND-FTD（OR = 13.75，95％CI）相关1.71至110.86）。结论患者及其家属的神经精神疾病与MND诊断后的认知和行为变化有关，许多与MND-FTD和C9orf72状态无关。这些发现支持了MND，额颞痴呆和神经精神疾病，特别是情绪障碍之间的重叠。结论患者及其家属的神经精神疾病与MND诊断后的认知和行为变化有关，许多与MND-FTD和C9orf72状态无关。这些发现支持了MND，额颞痴呆和神经精神疾病，特别是情绪障碍之间的重叠。结论患者及其家属的神经精神疾病与MND诊断后的认知和行为变化有关，许多独立于MND-FTD和C9orf72状态而发生。这些发现支持了MND，额颞痴呆和神经精神疾病，特别是情绪障碍之间的重叠。