Abstract：
BACKGROUND: Although frontotemporal dementia (FTD) with right anterior temporal lobe (RATL) predominance has been recognized, a uniform description of the syndrome is still missing. This multicenter study aims to establish a cohesive clinical phenotype.
METHODS: Retrospective clinical data from 18 centers across 12 countries yielded 360 FTD patients with predominant RATL atrophy through initial neuroimaging assessments.
RESULTS: Common symptoms included mental rigidity/preoccupations (78%), disinhibition/socially inappropriate behavior (74%), naming/word-finding difficulties (70%), memory deficits (67%), apathy (65%), loss of empathy (65%), and face-recognition deficits (60%). Real-life examples unveiled impairments regarding landmarks, smells, sounds, tastes, and bodily sensations (74%). Cognitive test scores indicated deficits in emotion, people, social interactions, and visual semantics however, lacked objective assessments for mental rigidity and preoccupations.
CONCLUSIONS: This study cumulates the largest RATL cohort unveiling unique RATL symptoms subdued in prior diagnostic guidelines. Our novel approach, combining real-life examples with cognitive tests, offers clinicians a comprehensive toolkit for managing these patients.
CONCLUSIONS: This project is the first international collaboration and largest reported cohort. Further efforts are warranted for precise nomenclature reflecting neural mechanisms. Our results will serve as a clinical guideline for early and accurate diagnoses.
METHODS: Retrospective clinical data from 18 centers across 12 countries yielded 360 FTD patients with predominant RATL atrophy through initial neuroimaging assessments.
RESULTS: Common symptoms included mental rigidity/preoccupations (78%), disinhibition/socially inappropriate behavior (74%), naming/word-finding difficulties (70%), memory deficits (67%), apathy (65%), loss of empathy (65%), and face-recognition deficits (60%). Real-life examples unveiled impairments regarding landmarks, smells, sounds, tastes, and bodily sensations (74%). Cognitive test scores indicated deficits in emotion, people, social interactions, and visual semantics however, lacked objective assessments for mental rigidity and preoccupations.
CONCLUSIONS: This study cumulates the largest RATL cohort unveiling unique RATL symptoms subdued in prior diagnostic guidelines. Our novel approach, combining real-life examples with cognitive tests, offers clinicians a comprehensive toolkit for managing these patients.
CONCLUSIONS: This project is the first international collaboration and largest reported cohort. Further efforts are warranted for precise nomenclature reflecting neural mechanisms. Our results will serve as a clinical guideline for early and accurate diagnoses.
摘要：
背景：尽管已经认识到以右颞叶（RATL）为主的额颞叶痴呆（FTD），对这种综合症的统一描述仍然缺失。这项多中心研究旨在建立一个有凝聚力的临床表型。
方法：来自12个国家的18个中心的回顾性临床数据通过初始神经影像学评估得出360例FTD患者的主要RATL萎缩。
结果：常见症状包括精神僵硬/专注（78％），抑制/社会不当行为（74%），命名/单词查找困难（70％），记忆缺陷（67%），冷漠（65%），失去同理心（65%），面部识别缺陷（60%）。现实生活中的例子揭示了关于地标的损伤，闻起来,声音,口味，和身体感觉（74％）。认知测试分数表明情绪缺陷，人,社交互动,然而，视觉语义，缺乏对精神僵化和专注的客观评估。
结论：本研究收集了最大的RATL队列，揭示了先前诊断指南中抑制的独特RATL症状。我们新颖的方法，将现实生活中的例子与认知测试相结合，为临床医生提供了一个全面的工具包来管理这些患者。
结论：该项目是第一个国际合作和最大的报告队列。对于反映神经机制的精确命名法，需要进一步的努力。我们的结果将作为早期和准确诊断的临床指南。
方法：来自12个国家的18个中心的回顾性临床数据通过初始神经影像学评估得出360例FTD患者的主要RATL萎缩。
结果：常见症状包括精神僵硬/专注（78％），抑制/社会不当行为（74%），命名/单词查找困难（70％），记忆缺陷（67%），冷漠（65%），失去同理心（65%），面部识别缺陷（60%）。现实生活中的例子揭示了关于地标的损伤，闻起来,声音,口味，和身体感觉（74％）。认知测试分数表明情绪缺陷，人,社交互动,然而，视觉语义，缺乏对精神僵化和专注的客观评估。
结论：本研究收集了最大的RATL队列，揭示了先前诊断指南中抑制的独特RATL症状。我们新颖的方法，将现实生活中的例子与认知测试相结合，为临床医生提供了一个全面的工具包来管理这些患者。
结论：该项目是第一个国际合作和最大的报告队列。对于反映神经机制的精确命名法，需要进一步的努力。我们的结果将作为早期和准确诊断的临床指南。
