Abstract
IntroductionFrontotemporal dementia (FTD) is a heterogeneous neurodegenerative disorder with multiple genetic and pathological causes. It is characterized by both cortical and subcortical atrophies, with previous studies showing early involvement of the amygdala. However, no prior study has specifically investigated the atrophy of different subnuclei of the amygdala. MethodsUsing an automated segmentation tool for T1-weighted volumetric magnetic resonance imaging, we investigated amygdalar subnuclei (AS) involvement in a cohort of 132 patients with genetic or pathologically confirmed FTD (age: mean = 61 years (standard deviation = 8); disease duration: 5 (3) years) compared with 107 age-matched controls. ResultsAS were affected in all genetic and pathological forms of FTD. MAPT mutations/FTDP-17, Pick's disease, and transactive response DNA binding protein 43 kDa type C were the forms with the smallest amygdala (35%–50% smaller than controls in the most affected hemisphere, P < .0005). In most FTD groups, medial subnuclei (particularly the superficial, accessory basal and basal/paralaminar subnuclei) tended to be affected more than the lateral subnuclei, except for the progressive supranuclear palsy group, in which the corticoamygdaloid transition area was the least-affected area. DiscussionDifferential involvement of the AS was seen in the different genetic and pathological forms of FTD. In general, the most affected subnuclei were the superficial, accessory basal and basal/paralaminar subnuclei, which form part of a network of regions that control reward and emotion regulation, functions known to be particularly affected in FTD.
Highlights
Frontotemporal dementia (FTD) is a heterogeneous neurodegenerative disorder
Stratifying by pathology, the FTDP-17, transactive response DNA binding protein 43 kDa (TDP-43) type C, and Pick’s disease groups were the most impaired, especially for the superficial (39%–47%), accessory basal nucleus (AB) (38%–50%), and basal/ paralaminar subnuclei (35%–39%, P, .0005) in the most affected hemisphere (Fig. 2B and Supplementary Table 1)
In a cohort of patients with genetic and pathologically confirmed FTD, we have shown that amygdalar subnuclei (AS) are the most affected in microtubule-associated protein tau (MAPT)/FTDP-17, Pick’s disease, and TDP-43 type C groups, with the most affected subnuclei across most groups being the superficial, AB, and basal/paralaminar ones
Summary
We aimed to investigate the pattern of atrophy of the AS in a cohort with a genetic or pathologically confirmed diagnosis of FTD to clarify whether and to what extent the AS are impaired across these different forms of FTD
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