Clinical and pathological heterogeneity is common in patients with frontotemporal lobar degeneration (FTLD) pathology. This investigated clinical or imaging characteristics that differentiate FTLD-TDP from FTLD-tau, FTLD-TDP subtypes from each other, or pathological stages of FTLD-TDP. Initial clinical, neuropsychological and neuroimaging characteristics were compared between pathologically defined FTLD-tau and FTLD-TDP groups. Voxel-based morphometry analyses contrasted grey matter atrophy patterns. Twenty-six FTLD-TDP, 28 FTLD-tau and 78 controls were included. Amyotrophic lateral sclerosis features, when present, were highly specific FTLD-TDP, which displayed greater cortical and subcortical atrophy than FTLD-tau. FTLD-TDP-43 type B had significantly shorter survival than type A. Type A patients were more cognitively impaired than type B, and basal ganglia atrophy appeared to distinguish type A from type B. Age at onset and survival duration were comparable between stages II and IV. In conclusion, Amyotrophic lateral sclerosis features may be useful in distinguishing FTLD-TDP from FTLD-tau. TDP-43 type A and B appear to present with distinct profiles. The relationship between clinical features and pathological staging in FTLD-TDP-43 is complex, and TDP-43 subtyping may have more clinical utility.

临床和病理异质性在额颞叶变性 (FTLD) 病理患者中很常见。这研究了区分 FTLD-TDP 与 FTLD-tau、FTLD-TDP 亚型或 FTLD-TDP 病理阶段的临床或影像学特征。比较病理定义的 FTLD-tau 组和 FTLD-TDP 组的初始临床、神经心理学和神经影像学特征。基于体素的形态测量分析对比灰质萎缩模式。包括 26 个 FTLD-TDP、28 个 FTLD-tau 和 78 个对照。肌萎缩侧索硬化症的特征，当存在时，是高度特异性的 FTLD-TDP，其表现出比 FTLD-tau 更大的皮质和皮质下萎缩。FTLD-TDP-43 B 型的生存期明显短于 A 型。A 型患者的认知障碍比 B 型更严重，和基底节萎缩似乎可以区分 A 型和 B 型。II 期和 IV 期的发病年龄和存活时间相当。总之，肌萎缩侧索硬化特征可能有助于区分 FTLD-TDP 和 FTLD-tau。TDP-43 A 型和 B 型似乎呈现出不同的轮廓。FTLD-TDP-43 的临床特征和病理分期之间的关系很复杂，TDP-43 亚型可能具有更多的临床效用。