Discipline of Pathology, Sydney Medical School, The University of Sydney, Sydney, NSW 2206, Australia.
Acta Neuropathol Commun. 2013 Jul 5;1:30. doi: 10.1186/2051-5960-1-30.
Early atrophy of the cingulate cortex is a feature of both behavioral variant frontotemporal dementia (bvFTD) and Alzheimer's disease (AD), with degeneration of the anterior cingulate region increasingly recognized as a strong predictor of bvFTD. The total number of neurons in this region, rather than the density of neurons, is associated with mood disturbance in other dementias, although there are no data on the extent and magnitude of neuronal loss in patients with bvFTD. While the density of small populations of neurons in this region has been assessed, it is unlikely that the degree of atrophy of the cingulate cortex seen in bvFTD can be explained by the loss of these subpopulations. This suggests that there is more generalized degeneration of neurons in this region in bvFTD.The present study assesses total neuronal number, as well as characteristic pathologies, in the anterior and posterior cingulate cortices of pathologically confirmed bvFTD (N = 11) and AD (N = 9) patients compared with age-matched controls (N = 14). The bvFTD cohort comprised 5 cases with tau pathology (Pick's disease), and 6 with TDP-43 pathology.
At postmortem, atrophy was detected in the anterior and posterior cingulate cortices of bvFTD cases, but only in the posterior cingulate cortex of AD cases. As predicted, there was a significant reduction in both the density and total number of neurons in the anterior but not the posterior cingulate cortex of bvFTD cases with the opposite observed for the AD cases. Importantly, neuronal loss in the anterior cingulate cortex was only observed in cases with tau pathology.
This study confirms significant neuronal loss in the posterior but not anterior cingulate cortex in AD, and demonstrates that significant neuron loss in bvFTD occurs only in the anterior cingulate cortex but only in cases with tau pathology compared with cases with TDP pathology. We propose that significant neurodegeneration in the anterior cingulate cortex may be useful in differentiating the pathological subtypes in vivo.
扣带回皮质的早期萎缩是行为变异额颞叶痴呆（bvFTD）和阿尔茨海默病（AD）的特征，越来越多的人认为前扣带回区域的退化是 bvFTD 的强有力预测指标。该区域神经元的总数，而不是神经元的密度，与其他痴呆症中的情绪障碍有关，尽管在 bvFTD 患者中，没有关于神经元丢失程度和幅度的数据。虽然已经评估了该区域中小部分神经元的密度，但 bvFTD 中看到的扣带回皮质萎缩程度不太可能仅仅是这些亚群丢失的结果。这表明在 bvFTD 中，该区域的神经元存在更广泛的退行性变化。本研究评估了病理证实的 bvFTD（N=11）和 AD（N=9）患者与年龄匹配的对照组（N=14）的前扣带回和后扣带回皮质中的总神经元数量以及特征性病理学。bvFTD 队列包括 5 例 tau 病理学（Pick 病）和 6 例 TDP-43 病理学。
在尸检时，在 bvFTD 病例的前扣带回和后扣带回皮质中检测到萎缩，但仅在 AD 病例的后扣带回皮质中检测到萎缩。正如预期的那样，在前扣带回皮质中，bvFTD 病例的神经元密度和总数均显著降低，而 AD 病例则相反。重要的是，只有在前扣带回皮质中观察到 tau 病理学的病例才观察到神经元丢失。
本研究证实 AD 中后扣带回皮质的神经元丢失显著，但前扣带回皮质没有丢失，并且表明 bvFTD 中仅在前扣带回皮质中发生显著的神经元丢失，但只有在 tau 病理学的情况下，与 TDP 病理学的病例相比。我们提出，前扣带回皮质的显著神经退行性变可能有助于在体内区分病理亚型。
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