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Synaptic loss occurs early in many neurodegenerative diseases and contributes to cognitive impairment even in the absence of gross atrophy. Currently, for human disease there are few formal models to explain how cortical networks underlying cognition are affected by synaptic loss. We advocate that biophysical models of neurophysiology offer both a bridge from preclinical to clinical models of pathology and quantitative assays for experimental medicine. Such biophysical models can also disclose hidden neuronal dynamics generating neurophysiological observations such as EEG and magnetoencephalography. Here, we augment a biophysically informed mesoscale model of human cortical function by inclusion of synaptic density estimates as captured by 11C-UCB-J PET, and provide insights into how regional synapse loss affects neurophysiology. We use the primary tauopathy of progressive supranuclear palsy (Richardson's syndrome) as an exemplar condition, with high clinicopathological correlations. Progressive supranuclear palsy causes a marked change in cortical neurophysiology in the presence of mild cortical atrophy and is associated with a decline in cognitive functions associated with the frontal lobe. Using parametric empirical Bayesian inversion of a conductance-based canonical microcircuit model of magnetoencephalography data, we show that the inclusion of regional synaptic density-as a subject-specific prior on laminar-specific neuronal populations-markedly increases model evidence. Specifically, model comparison suggests that a reduction in synaptic density in inferior frontal cortex affects superficial and granular layer glutamatergic excitation. This predicted individual differences in behaviour, demonstrating the link between synaptic loss, neurophysiology and cognitive deficits. The method we demonstrate is not restricted to progressive supranuclear palsy or the effects of synaptic loss: such pathology-enriched dynamic causal models can be used to assess the mechanisms of other neurological disorders, with diverse non-invasive measures of pathology, and is suitable to test the effects of experimental pharmacology.
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http://dx.doi.org/10.1093/brain/awac471 | DOI Listing |
JAMA Neurol
September 2025
Center for Neurodegenerative Diseases and the Aging Brain, University of Bari 'Aldo Moro,' "Pia Fondazione Cardinale G. Panico," Tricase, Lecce, Italy.
Importance: Comprehensive incidence and prevalence rates of frontotemporal dementia are currently not available.
Objective: To estimate the incidence and prevalence of frontotemporal dementia and its clinical variants in the overall population and age subgroups.
Data Sources And Study Selection: We systematically searched PubMed, EMBASE, and Scopus between January 1, 1990, and October 22, 2024, for population-based studies estimating the incidence and/or prevalence of FTD.
J Neurochem
September 2025
Center for Translational Research in Neurodegenerative Disease, University of Florida, Gainesville, Florida, USA.
The two most prominent post-translational modifications of pathologic tau are Ser/Thr/Tyr phosphorylation and Lys acetylation. Whether acetylation impacts the susceptibility of tau to templated seeding in diseases like Alzheimer's disease (AD) and Progressive Supranuclear Palsy (PSP) is largely uncharacterized. Towards this, we examined how acetylation mimicking or nullifying mutations on five sites of tau (K311, K353, K369, K370, K375), located within the tau filament core, influenced the susceptibility of P301L (PL) tau to seeds from AD (AD-tau) or PSP (PSP-tau) brain donors in HEK293T cells.
View Article and Find Full Text PDFSleep Med
August 2025
Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan Province, 610041, PR China. Electronic address:
Background: Progressive supranuclear palsy (PSP) is a rapidly advancing tauopathy, and amongst rapid eye movement (REM) sleep behavior disorder (RBD) has been observed. Prior studies were limited by small samples and inconsistent results; meanwhile, research is lacking since the updated criteria for PSP. This cross-sectional study aimed to assess RBD in a sizable PSP cohort.
View Article and Find Full Text PDFJ Neural Transm (Vienna)
September 2025
Edmond J. Safra Program in Parkinson's Disease, the Rossy Progressive Supranuclear Palsy Centre, and the Morton and Gloria Shulman Movement Disorders Clinic, Toronto Western Hospital, Toronto, ON, Canada.
Parkinson's disease (PD) is increasingly recognized as a heterogeneous neurodegenerative entity with diverse clinical presentations, genetic contributors, and neuropathological features. Central to its pathogenesis is misfolded and aggregated α-synuclein, which collectively form Lewy pathology. Recent advances in biomarker and genetic research have enabled biologically grounded models of PD classification, diagnosis and staging.
View Article and Find Full Text PDFMov Disord Clin Pract
September 2025
UCL Queen Square Institute of Neurology, London, UK.
Background: Progressive Supranuclear Palsy (PSP) is a rare and severe neurodegenerative tauopathy characterized by diverse clinical phenotypes, including Richardson's syndrome (PSP-RS), PSP-parkinsonism (PSP-P), PSP-progressive gait freezing (PSP-PGF), and PSP-corticobasal syndrome (PSP-CBS). Significant geographic variation exists in prevalence, clinical presentations, and prognosis.
Objectives: This global review aims to systematically evaluate the epidemiological variation, clinical phenotypes, diagnostic practices, and management strategies for PSP, focusing on regional disparities and identifying influencing genetic and environmental factors.