Kiwamu Matsuoka1,2, Yuhei Takado1, Kenji Tagai1, Manabu Kubota3, Yasunori Sano1, Keisuke Takahata1, Maiko Ono1, Chie Seki1, Hideki Matsumoto1,4, Hironobu Endo1, Hitoshi Shinotoh1, Jamie Near5, Kazunori Kawamura1, Ming-Rong Zhang1, Hitoshi Shimada1, and Makoto Higuchi1
1National Institute of Radiological Sciences, National Institutes for Quantum and Radiological Science and Technology, Chiba, Japan, 2Department of Psychiatry, Nara Medical University, Kashihara, Japan, 3Department of Psychiatry, Kyoto University Graduate School of Medicine, Kyoto, Japan, 4Department of Oral and Maxillofacial Radiology, Tokyo Dental College, the city of Chiyoda-ku, Japan, 5Douglas Mental Health University Institute and Department of Psychiatry, McGill University, Montréal, QC, Canada
Synopsis
Apathy is characterized by lack of motivation. We
investigated the mechanisms underlying apathy in progressive supranuclear palsy
(PSP), which is characterized by tau aggregate accumulations causing oxidative
stress in the brain. Using magnetic resonance spectroscopy and tau positron
emission tomography, we found associations of apathy levels with
glutathione levels in the posterior
cingulate cortex (PCC), tau aggregate
accumulation levels in the angular gyrus/PCC, and atrophy of the right inferior frontal gyrus and anterior cingulate
cortex. The vulnerability of the
anterior and posterior brain regions where apathy is related is suggested as possible
underlying mechanisms for apathy in PSP.
INTRODUCTION
Progressive supranuclear
palsy (PSP) is one of the primary
tauopathies. 1
More than half of patients with PSP present apathy, defined by lack of
motivation. 2 While we previously reported
that apathy levels are associated with tau aggregates in the anterior brain
regions in Alzheimer’s disease 3, the underlying mechanism of apathy in relation to tau aggregates
in PSP patients remains unknown. We hypothesized that tau aggregates cause oxidative
stress (OS),
leading to the vulnerability of brain regions where apathy is related.
To test this hypothesis, we performed positron emission tomography
(PET) scans with a tau ligand, 18F-PM-PBB3 4, magnetic resonance
spectroscopy (MRS) scans to measure levels of the antioxidant glutathione (GSH) 5, and
volumetry.METHODS
We
enrolled 20 PSP patients and 23 healthy controls (HCs) confirmed to be amyloid-negative. We performed MRS using a short TE spin-echo full-intensity acquired
localized single voxel spectroscopy (SPECIAL) sequence (TR/TE/number of
excitations = 3000 ms / 8.5 ms / 128), 18F-PM-PBB3 PET scans,
and examined three-dimensional T1-weighted images. Given the importance of both the anterior and posterior cingulate
cortex for apathy and GSH 6, 7, volumes of interest for MRS were
manually placed on the anterior cingulate cortex (ACC, 30
× 20 × 20 mm2) and posterior cingulate cortex (PCC, 20 × 20 ×
20 mm2) regions (Figure
1). We analyzed MRS data using LCModel software for a linear combination of
model fitting with a basis set including GSH and corrected metabolite values
for CSF partial volumes. We generated standardized
uptake value ratio (SUVR) images of 18F-PM-PBB3 PET with
cerebellar gray matter (GM) as a reference, using data at 90-110 min. We
performed voxel-based morphometry (VBM) about the
associations of apathy scale (AS) scores with 18F-PM-PBB3
SUVRs and GM volumes in PSP patients. T-tests, χ2-tests, and
analysis of covariance were used to compare the two groups and Pearson partial
correlation and Spearman's partial rank-order correlation were used to evaluate
the associations of AS scores. Path analysis was used for models explaining apathy. This study was approved by the
Certified Review Board.RESULTS
The demographic profiles were summarized. MRS:
The average Cramér–Rao lower bound of
GSH measurements were 6.1% and 6.9% in ACC and PCC, respectively. While there
was no significant difference between the PCC GSH levels of PSP patients and
HCs (p = 0.87), partial correlation analyses with age and PSP Rating Scale
(PSPRS) scores showed negative correlations between the PCC GSH levels and AS
scores (r = -0.62, p = 0.014) (Figure 2a).
The GSH levels in the ACC in PSP patients did not show significant associations
with AS scores (p = 0.87). 18F-PM-PBB3 PET: In PSP
patients, a positive association was found between AS scores and 18F-PM-PBB3
SUVRs in the bilateral angular gyrus (AG) and PCC using age and PSPRS scores as
nuisance variables (Figure 2b and 3). We found significantly higher 18F-PM-PBB3
SUVRs in the bilateral AG in PSP patients compared with HCs using age as
nuisance variables (the left AG: p = 0.006; the right AG: p = 0.004). Partial correlation analyses with age and PSPRS scores suggested that there
were negative correlations of the GSH levels in the PCC with 18F-PM-PBB3
SUVRs in the PCC and AG (the PCC: r = -0.63, p = 0.012; the AG: r = -0.64, p = 0.010) (Figure 2c-d). Volumetry:
In the VBM using age, PSPRS scores, and TBV as nuisance
variables in PSP patients, we found negative associations between AS scores and
the GM volumes in the right inferior frontal gyrus (IFG) and ACC (Figure 4). Path analysis: Path analysis generated a model between
the AS scores, the 18F-PM-PBB3 SUVRs in the AG, the GSH levels in the PCC, and the GM volumes
in the right IFG satisfied the criteria for a good fit (χ2 (2) =
2.07, p = 0.36, CFI = 1.00, NFI = 0.91,
RMSEA = 0.046) (Figure 5).DISCUSSION and CONCLUSION
We found associations of apathy with tau
accumulation levels in the AG/PCC and GSH
levels in the PCC, in addition to
atrophy of the right IFG and ACC, which have key roles in the reward system closely
related to apathy 8. Because the AG and PCC have been reported to be
associated with action initiation 9 and
self-reference 10, respectively, the
deterioration of these functions might also be related to apathy. 7 We also found negative
associations between 18F-PM-PBB3 SUVRs and GSH levels, suggesting a link between tau aggregate accumulations and OS, in line with previous evidence that aggregates initiate
inflammation which causes reactive oxygen species production. 11, 12 Our model generated by path analysis suggests
that the depletion of GSH in the PCC is associated with abnormal tau aggregation
in the AG and PCC region. Antioxidant GSH might serve as the resilience factor
of the neural system by limiting the damage from OS caused by tau aggregate
accumulation. 13 In conclusion, these findings suggest a potential
link between tau aggregates, OS, and brain atrophy in both the anterior and
posterior brain regions as underlying brain mechanisms for apathy in PSP
patients.Acknowledgements
The authors thank all patients and their
caregivers for participation in this study, clinical research coordinators, PET
and MRI operators, and research ethics advisers at QST for their assistance to
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