Medical School of Puerto Rico, Rio Piedras Campus, Rio Piedras, Puerto Rico.
Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
Dement Geriatr Cogn Disord. 2021;50(1):17-28. doi: 10.1159/000513979. Epub 2021 Mar 23.
Semantic dementia (SD) is characterized by fluent speech, anomia, and loss of word and object knowledge with varying degrees of right and left anterior-medial temporal lobe hypometabolism on [18F] fluorodeoxyglucose (FDG)-PET. We assessed neurobehavioral features in SD patients across 3 FDG-PET-defined metabolic patterns and investigated progression over time.
Thirty-four patients with SD who completed FDG-PET were classified into a left- and right-dominant group based on the degree of hypometabolism in each temporal lobe. The left-dominant group was further subdivided depending on whether hypometabolism in the right temporal lobe was more or less than 2 standard deviations from controls (left+ group). Neurobehavioral characteristics determined using the Neuropsychiatric Inventory Questionnaire (NPI-Q) were compared across groups. Progression of NPI-Q scores and FDG-PET hypometabolism was assessed in 14 patients with longitudinal follow-up.
The right-dominant group performed worse on the NPI-Q and had a greater frequency of abnormal behaviors and more severe disinhibition compared to the left-dominant group. Performance on the NPI-Q and severity of disinhibition correlated with right medial and lateral, but not left, temporal lobe hypometabolism. Severity of abnormal behaviors worsened over time in most left-dominant and left+ patients but appeared to improve in the 2 right-dominant patients with longitudinal follow-up. All groups showed progressive worsening of metabolism in both temporal lobes over time, with hypometabolism spreading from anteromedial to posterior temporal regions. However, the degree of temporal lobe asymmetry remained relatively constant over time.
In SD, neurobehavioral features, especially disinhibition, are associated with right medial and lateral temporal lobe hypometabolism and commonly develop over time even in patients that present with left-dominant patterns of hypometabolism.
语义性痴呆(SD)的特点是语言流畅、命名障碍以及词语和物体知识丧失,同时伴有不同程度的右侧和左侧前内侧颞叶代谢低下。我们评估了 34 例完成氟脱氧葡萄糖(FDG)-正电子发射断层扫描(PET)的 SD 患者的神经行为特征,并研究了其随时间的进展。
根据每个颞叶代谢低下的程度,将 34 例 SD 患者分为左优势组和右优势组。左优势组进一步根据右侧颞叶代谢低下是否多于或少于对照组的 2 个标准差(左+组)进行细分。使用神经精神问卷(NPI-Q)确定神经行为特征,并比较各组之间的特征。对 14 例有纵向随访的患者进行 NPI-Q 评分和 FDG-PET 代谢低下的进展评估。
右优势组在 NPI-Q 上的表现较差,且异常行为的频率更高,抑制障碍更严重,与左优势组相比。NPI-Q 的表现和抑制障碍的严重程度与右侧内侧和外侧颞叶代谢低下相关,但与左侧颞叶代谢低下无关。大多数左优势和左+患者的异常行为严重程度随时间恶化,但在 2 例有纵向随访的右优势患者中似乎有所改善。所有组的双侧颞叶代谢均随时间逐渐恶化,代谢低下从前内侧向颞叶后部扩散。然而,颞叶不对称的程度随时间保持相对稳定。
在 SD 中,神经行为特征,尤其是抑制障碍,与右侧内侧和外侧颞叶代谢低下相关,且随着时间的推移通常会发展,即使患者表现为左优势型代谢低下模式也是如此。