Dementia Reseach Centre, Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, UK.
London School of Hygiene & Tropical Medicine, London, UK.
J Neurol Neurosurg Psychiatry. 2023 May;94(5):357-368. doi: 10.1136/jnnp-2022-330152. Epub 2023 Jan 10.
Current clinical rating scales in frontotemporal dementia (FTD) often do not incorporate neuropsychiatric features and may therefore inadequately measure disease stage.
832 participants from the Genetic FTD Initiative (GENFI) were recruited: 522 mutation carriers and 310 mutation-negative controls. The standardised GENFI clinical questionnaire assessed the frequency and severity of 14 neuropsychiatric symptoms: visual, auditory, and tactile hallucinations, delusions, depression, anxiety, irritability/lability, agitation/aggression, euphoria/elation, aberrant motor behaviour, hypersexuality, hyperreligiosity, impaired sleep, and altered sense of humour. A principal component analysis (PCA) was performed to identify key groupings of neuropsychiatric and behavioural items in order to create a new neuropsychiatric module that could be used as an addition to the Clinical Dementia Rating (CDR) plus National Alzheimer's Coordinating Center Behaviour and Language Domains (NACC FTLD) rating scale.
Overall, 46.4% of mutation carriers had neuropsychiatric symptoms (51.6% , 40.8% , 46.6% ) compared with 24.5% of controls. Anxiety and depression were the most common in all genetic groups but fluctuated longitudinally and loaded separately in the PCA. Hallucinations and delusions loaded together, with the remaining neuropsychiatric symptoms loading with the core behavioural features of FTD. These results suggest using a single 'psychosis' neuropsychiatric module consisting of hallucinations and delusions. Adding this to the CDR plus NACC FTLD, called the CDR plus NACC FTLD-N, leads to a number of participants being scored more severely, including those who were previously considered asymptomatic now being scored as prodromal.
Neuropsychiatric symptoms occur in mutation carriers at all disease stages across all three genetic groups. However, only psychosis features provided additional staging benefit to the CDR plus NACC FTLD. Inclusion of these features brings us closer to optimising the rating scale for use in trials.
目前用于额颞叶痴呆（FTD）的临床评估量表往往不包含神经精神症状，因此可能无法充分评估疾病阶段。
从遗传额颞叶痴呆倡议（GENFI）招募了 832 名参与者：522 名突变携带者和 310 名突变阴性对照。标准化的 GENFI 临床问卷评估了 14 种神经精神症状的频率和严重程度：视觉、听觉和触觉幻觉、妄想、抑郁、焦虑、易怒/不稳定、激越/攻击、欣快/兴奋、异常运动行为、性欲亢进、宗教过度、睡眠障碍和幽默感改变。进行了主成分分析（PCA），以确定神经精神和行为项目的关键分组，以便创建一个新的神经精神模块，可以作为临床痴呆评分（CDR）加国家阿尔茨海默病协调中心行为和语言领域（NACC FTLD）评分的补充。
总体而言，与对照组相比，46.4%的突变携带者出现神经精神症状（51.6%、40.8%、46.6%）。焦虑和抑郁在所有遗传组中最为常见，但在纵向变化且在 PCA 中单独加载。幻觉和妄想一起加载，其余神经精神症状与 FTD 的核心行为特征一起加载。这些结果表明，使用包含幻觉和妄想的单一“精神病”神经精神模块。将其添加到 CDR 加 NACC FTLD 中，称为 CDR 加 NACC FTLD-N，导致许多参与者被评为更严重，包括以前被认为无症状的参与者现在被评为前驱期。
在所有三个遗传组中，在疾病的所有阶段，突变携带者都会出现神经精神症状。然而，只有精神病特征为 CDR 加 NACC FTLD 提供了额外的分期获益。包含这些特征使我们更接近优化该量表以用于试验。
