METHODS: We retrospectively reviewed medical records of 26 autopsied FTLD patients (six FTLD-FET, ten FTLD-Tau, and ten FTLD-TDP) who had completed antemortem FDG-PET. We evaluated five regions, caudate nucleus, medial frontal cortex, lateral frontal cortex, and medial temporal using a 0-3 visual rating scale and validated our findings quantitatively using CORTEX-ID suite Z scores.
RESULTS: Of the six FTLD-FET cases (three females) with median age at onset = 36, three were atypical FTLD-U (aFTLD-U) and three were neuronal intermediate filament inclusion disease (NIFID). bvFTD was the most common presentation. Four of the six FTLD cases (3 aFTLD-U + 1 NIFID) showed prominent caudate hypometabolism relatively early in the disease course. FTLD-tau and FTLD-TDP did not show early prominent caudate hypometabolism. Hypometabolism in medial and lateral temporal cortex was associated with FTLD-TDP, while FTLD-tau had normal-minimal regional metabolism.
CONCLUSIONS: Prominent caudate hypometabolism, especially early in the disease course, appears to be a hallmark feature of the aFTLD-U subtype of FTLD-FET. Assessing caudate and temporal hypometabolism on FDG-PET will help to differentiate FTLD-FET from FTLD-tau and FTLD-TDP.
方法:我们回顾性回顾了26例尸检FTLD患者的医疗记录(6例FTLD-FET,十个FTLD-Tau,和10名FTLD-TDP)完成了生前FDG-PET。我们评估了五个地区,尾状核,内侧额叶皮质,外侧额叶皮质,和内侧时间使用0-3视觉评分量表,并使用CORTEX-ID套件Z评分定量验证我们的发现。
结果:在发病中位年龄为36岁的6例FTLD-FET病例(3例女性)中,3例为非典型FTLD-U(aFTLD-U),3例为神经元中间丝包涵体病(NIFID)。bvFTD是最常见的介绍。6例FTLD病例中的4例(3aFTLD-U1NIFID)在病程早期显示出明显的尾状低代谢。FTLD-tau和FTLD-TDP未显示早期明显的尾状低代谢。内侧和外侧颞叶皮质的低代谢与FTLD-TDP相关,而FTLD-tau具有正常-最小的区域代谢。
结论:明显的尾状低代谢,尤其是在病程早期,似乎是FTLD-FET的aFTLD-U亚型的标志性特征。在FDG-PET上评估尾状和时间低代谢将有助于区分FTLD-FET与FTLD-tau和FTLD-TDP。