Theme 11 Cognitive and psychological assessment and support

IF 2.5 4区医学 Q2 CLINICAL NEUROLOGY

Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration Pub Date : 2019-10-31 DOI:10.1080/21678421.2019.1647000

R. Ahmed, E. Devenney, C. Strikwerda-Brown, J. Hodges, O. Piguet, M. Kiernan

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引用次数: 2

Abstract

Background: Within the Amyotrophic Lateral Sclerosis (ALS)-Frontotemporal dementia (FTD) spectrum there is considerable heterogeneity in clinical presentation and survival.Objectives: The current study aimed to examine how initial symptoms (motor compared to cognitive) may affect survival, with specific focus on structural cognitive and behavioural differences between ALS-FTD and bvFTD  cohorts.Methods: Cognitive and behavioural profiles were examined in 98 patients (59 ALS-FTD and 39 bvFTD patients). The initial presentation of ALS-FTD was categorized into either motor or cognitive, based on symptoms combined with carer reports. Survival was calculated from initial symptom onset. Brain atrophy patterns on MRI were examined using a verified visual rating scale.Results: In the ALS-FTD group, 69% were categorized as having an initial cognitive presentation and 31% a motor presentation. Those patients with motor presentation of ALS-FTD experienced a significantly shorter survival of 33 months, compared to 63 months (p<0.007) in those with a cognitive presentation of ALS-FTD. On cognitive testing there were no differences between motor versus cognitive onset ALS-FTD. When compared to bvFTD, ALS-FTD, particularly the cognitive presentation, was characterized by reduced language function (p<0.001), verbal fluency (p = 0.001), and naming (p = 0.007). Both cognitive and motor presentation ALS-FTD had poorer emotion processing (p = 0.01) compared to bvFTD. On structural imaging analyses both motor and cognitive onset ALS-FTD patients had greater motor cortex and dorsal lateral prefrontal cortex atrophy compared to bvFTD patients. Increased motor cortex atrophy was associated with 1.5-fold reduction in survival.Discussion and conclusions: In ALS-FTD those with an initial motor presentation have a much faster progression than those with a cognitive presentation, despite having similar overall cognitive impairment, suggesting that disease progression in ALS-FTD may be critically linked to physiological and motor changes. Survival is also associated with motor cortex atrophy which is increased in ALS-FTD.These results provide further suggestions in relation to the categorization of clinical trial patients into fast and slow progressors.

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主题11认知和心理评估与支持

背景:在肌萎缩性侧索硬化症(ALS)-额颞叶痴呆(FTD)谱系中，临床表现和生存率存在相当大的异质性。目的:目前的研究旨在检查初始症状(运动与认知相比)如何影响生存，特别关注ALS-FTD和bvFTD 队列之间的结构性认知和行为差异。方法:对98例患者(59例ALS-FTD和39例bvFTD)的认知和行为特征进行分析。ALS-FTD的最初表现根据症状和护理人员报告分为运动或认知两类。生存率从最初症状开始计算。脑萎缩模式的MRI检查使用验证视觉评定量表。结果:在ALS-FTD组中，69%被归类为初始认知表现，31%被归类为运动表现。运动表现的ALS-FTD患者的生存期为33个月，而认知表现的ALS-FTD患者的生存期为63个月(p<0.007)。在认知测试中，运动型和认知型ALS-FTD之间没有差异。与bvFTD相比，ALS-FTD，尤其是认知表现，以语言功能下降(p<0.001)、言语流畅(p = 0.001)和命名(p = 0.007)为特征。与bvFTD相比，ALS-FTD在认知和运动表现方面的情绪加工较差(p = 0.01)。在结构成像分析中，与bvFTD患者相比，运动和认知发病的ALS-FTD患者有更大的运动皮层和背外侧前额叶皮层萎缩。运动皮质萎缩加剧与生存期降低1.5倍相关。讨论和结论:在ALS-FTD中，尽管具有相似的整体认知障碍，但具有初始运动表现的患者比具有认知表现的患者进展得快得多，这表明ALS-FTD的疾病进展可能与生理和运动变化密切相关。生存也与ALS-FTD中增加的运动皮质萎缩有关。这些结果为将临床试验患者分为快速进展者和缓慢进展者提供了进一步的建议。

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来源期刊

Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration CLINICAL NEUROLOGY-

CiteScore

5.40

自引率

10.70%

发文量

期刊介绍： Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration is an exciting new initiative. It represents a timely expansion of the journal Amyotrophic Lateral Sclerosis in response to the clinical, imaging pathological and genetic overlap between ALS and frontotemporal dementia. The expanded journal provides outstanding coverage of research in a wide range of issues related to motor neuron diseases, especially ALS (Lou Gehrig’s disease) and cognitive decline associated with frontotemporal degeneration. The journal also covers related disorders of the neuroaxis when relevant to these core conditions.