Abstract：
OBJECTIVE: Frontotemporal dementia (FTD) patients frequently present with psychosis, which complicates diagnosis and management. In this study, we aim to examine the relationship between psychosis and the most common genetic mutations predisposing to FTD, and in the different pathological subtypes of FTD.
METHODS: We conducted a systematic review, searching the literature up to December 2022, and reviewed 50 articles that met our inclusion criteria. From the reviewed articles, we extracted and summarized data regarding the frequency of psychosis and patient characteristics in each major genetic and pathological subtype of FTD.
RESULTS: Among FTD patients with confirmed genetic mutations or pathological diagnosss, the frequency of psychosis was 24.2%. Among the genetic mutation carriers, C9orf72 mutation carriers had the highest frequency of psychosis (31.4%), whereas GRN (15.0%) and MAPT (9.2%) mutation carriers had lower frequencies of psychosis. MAPT mutation carriers notably developed psychosis at a younger age compared to other genetic groups. The most common psychotic symptoms were delusions among C9orf72 carriers and visual hallucinations among GRN mutation carriers. Among the pathological subtypes, 30% of patients with FUS pathology, 25.3% of patients with TDP-43 pathology, and 16.4% of patients with tau pathology developed psychosis. In the TDP-43 group, subtype B pathology was the most common subtype reported in association with psychosis.
CONCLUSIONS: Our systematic review suggests a high frequency of psychosis in specific subgroups of FTD patients. Further studies are required to understand the structural and biological underpinnings of psychosis in FTD.
METHODS: We conducted a systematic review, searching the literature up to December 2022, and reviewed 50 articles that met our inclusion criteria. From the reviewed articles, we extracted and summarized data regarding the frequency of psychosis and patient characteristics in each major genetic and pathological subtype of FTD.
RESULTS: Among FTD patients with confirmed genetic mutations or pathological diagnosss, the frequency of psychosis was 24.2%. Among the genetic mutation carriers, C9orf72 mutation carriers had the highest frequency of psychosis (31.4%), whereas GRN (15.0%) and MAPT (9.2%) mutation carriers had lower frequencies of psychosis. MAPT mutation carriers notably developed psychosis at a younger age compared to other genetic groups. The most common psychotic symptoms were delusions among C9orf72 carriers and visual hallucinations among GRN mutation carriers. Among the pathological subtypes, 30% of patients with FUS pathology, 25.3% of patients with TDP-43 pathology, and 16.4% of patients with tau pathology developed psychosis. In the TDP-43 group, subtype B pathology was the most common subtype reported in association with psychosis.
CONCLUSIONS: Our systematic review suggests a high frequency of psychosis in specific subgroups of FTD patients. Further studies are required to understand the structural and biological underpinnings of psychosis in FTD.
摘要：
目的：额颞叶痴呆（FTD）患者常出现精神病，这使得诊断和管理复杂化。在这项研究中,我们的目的是研究精神病与FTD最常见的基因突变之间的关系，以及FTD的不同病理亚型。
方法：我们进行了系统评价，搜索截至2022年12月的文献，并回顾了50篇符合我们纳入标准的文章。从评论的文章中，我们提取并总结了FTD的每个主要遗传和病理亚型的精神病发生率和患者特征的数据.
结果：在确诊基因突变或病理诊断的FTD患者中，精神病的发生率为24.2%。在基因突变携带者中，C9orf72突变携带者出现精神病的频率最高(31.4%),而GRN(15.0%)和MAPT(9.2%)突变携带者的精神病发生率较低.与其他遗传群体相比，MAPT突变携带者在更年轻的时候就出现了精神病。最常见的精神病症状是C9orf72携带者的妄想和GRN突变携带者的视觉幻觉。在病理亚型中，30%的患者有FUS病理，25.3%的患者有TDP-43病理，16.4%的tau病理患者出现精神病。在TDP-43组中，B型亚型病理学是与精神病相关的最常见亚型.
结论：我们的系统评价表明，FTD患者的特定亚组中精神病的发生率很高。需要进一步的研究来了解FTD中精神病的结构和生物学基础。
方法：我们进行了系统评价，搜索截至2022年12月的文献，并回顾了50篇符合我们纳入标准的文章。从评论的文章中，我们提取并总结了FTD的每个主要遗传和病理亚型的精神病发生率和患者特征的数据.
结果：在确诊基因突变或病理诊断的FTD患者中，精神病的发生率为24.2%。在基因突变携带者中，C9orf72突变携带者出现精神病的频率最高(31.4%),而GRN(15.0%)和MAPT(9.2%)突变携带者的精神病发生率较低.与其他遗传群体相比，MAPT突变携带者在更年轻的时候就出现了精神病。最常见的精神病症状是C9orf72携带者的妄想和GRN突变携带者的视觉幻觉。在病理亚型中，30%的患者有FUS病理，25.3%的患者有TDP-43病理，16.4%的tau病理患者出现精神病。在TDP-43组中，B型亚型病理学是与精神病相关的最常见亚型.
结论：我们的系统评价表明，FTD患者的特定亚组中精神病的发生率很高。需要进一步的研究来了解FTD中精神病的结构和生物学基础。
