Laforce Robert
Clin Neurol Neurosurg. 2013 Dec;115(12):2405-10. doi: 10.1016/j.clineuro.2013.09.031.
While recent advances in the development of neuroimaging and molecular biomarkers for studying neurodegenerative conditions have revolutionized the field, dementia remains a clinical diagnosis. No component of the diagnostic process is more crucial than obtaining a good history. Getting to know the first manifestations of the disease, tracking their evolution and functional impact, combined with a targeted neurological examination, further guides differential diagnosis. This paper summarizes the key symptoms of the behavioral and language variants of frontotemporal dementia. The behavioral variant of frontotemporal dementia (bvFTD) is characterized by severe changes in behavior and personality such as disinhibition, apathy, loss of empathy, or stereotypic behavior, leading to a loss of social competence. Executive functions are impaired, while memory and visuospatial skills are relatively better preserved. By contrast, the language variants or primary progressive aphasias (PPAs) are marked by prominent language disturbances that can be subclassified into a non-fluent/agrammatic variant (naPPA), a semantic variant (svPPA), and a logopenic variant (lvPPA). Although combined characterization of clinical, imaging, biological and genetic biomarkers is essential to establish a detailed diagnosis of such heterogeneous conditions, the author emphasizes the importance of accurate recognition of key symptoms that can lead to better identification of underlying neuropathology and appropriate treatment approaches.
虽然用于研究神经退行性疾病的神经影像学和分子生物标志物的最新进展彻底改变了该领域,但痴呆症仍然是一种临床诊断。诊断过程中没有哪个环节比获取详尽的病史更为关键。了解疾病的首发症状,追踪其演变过程和功能影响,再结合有针对性的神经学检查,能进一步指导鉴别诊断。本文总结了额颞叶痴呆行为和语言变异型的关键症状。额颞叶痴呆行为变异型(bvFTD)的特征是行为和个性发生严重变化,如脱抑制、冷漠、缺乏同理心或刻板行为,导致社交能力丧失。执行功能受损,而记忆和视觉空间技能相对保留较好。相比之下,语言变异型或原发性进行性失语症(PPA)的特征是明显的语言障碍,可细分为非流利/语法缺失型变异(naPPA)、语义型变异(svPPA)和语音迟缓型变异(lvPPA)。尽管综合临床、影像学、生物学和遗传生物标志物的特征对于建立此类异质性疾病的详细诊断至关重要,但作者强调准确识别关键症状的重要性,这有助于更好地识别潜在的神经病理学特征并采取适当的治疗方法。