Cognitive decline heralds onset of symptomatic inherited prion disease
AbstractThe clinical effectiveness of any disease-modifying treatment for prion disease, as for other neurodegenerative disorders, will depend on early treatment before damage to neural tissue is irrevocable. Thus, there is a need to identify markers that predict disease onset in healthy at-risk individuals. Whilst imaging and neurophysiological biomarkers have shown limited use in this regard, we recently reported progressive neurophysiological changes in individuals with the inherited prion disease mutation P102L. We have also previously demonstrated a signature pattern of fronto-parietal dysfunction in mild prion diseas...
Source: Brain - April 9, 2021 Category: Neurology Source Type: research

Co-pathologies in Alzheimer ’s disease: just multiple pathologies or partners in crime?
This scientific commentary refers to ‘The development and convergence of co-pathologies in Alzheimer’s disease’, by Robinsonet al. (doi:10.1093/brain/awaa438). (Source: Brain)
Source: Brain - April 9, 2021 Category: Neurology Source Type: research

DBS for refractory epilepsy: is closed-loop stimulation of the medial septum the way forward?
This scientific commentary refers to ‘Closed-loop stimulation of the medial septum terminates epileptic seizures’, by Takeuchiet al. (doi:10.1093/brain/awaa450),and‘Medial septal GABAergic neurons reduce seizure duration upon optogenetic closed-loop stimulation’, by Hristovaet al. (doi:10.1093/brain/awab042). (Source: Brain)
Source: Brain - April 9, 2021 Category: Neurology Source Type: research

A step forward in understanding the role of sleep and its link to neurodegeneration
This scientific commentary refers to ‘Sleep deprivation impairs molecular clearance from the human brain’ by Eideet al. (doi:10.1093/brain/awaa443). (Source: Brain)
Source: Brain - April 9, 2021 Category: Neurology Source Type: research

Taking others into account: combining directly experienced and indirect information in schizophrenia
AbstractAn abnormality in inference, resulting in distorted internal models of the world, has been argued to be a common mechanism underlying the heterogeneous psychopathology in schizophrenia. However, findings have been mixed as to wherein the abnormality lies and have typically failed to find convincing relations to symptoms. The limited and inconsistent findings may have been due to methodological limitations of the experimental design, such as conflating other factors (e.g. comprehension) with the inferential process of interest, and a failure to adequately assess and model the key aspects of the inferential process. ...
Source: Brain - April 8, 2021 Category: Neurology Source Type: research

Progression in Parkinson ’s disease: a potpourri of plots and probabilities
This scientific commentary refers to ‘Sequence of clinical and neurodegeneration events in Parkinson’s disease progression’ by Oxtobyet al. (doi:10.1093/brain/awaa461). (Source: Brain)
Source: Brain - April 8, 2021 Category: Neurology Source Type: research

Subclinical anterior optic pathway involvement in early multiple sclerosis and clinically isolated syndromes
AbstractOptical coherence tomography (OCT) is gaining increasing relevance in the assessment of patients with multiple sclerosis. Converging evidence point to the view that neuro-retinal changes, in eyes without acute optic neuritis, reflect inflammatory and neurodegenerative processes taking place throughout the CNS. The present study aims at exploring the usefulness of OCT as a marker of inflammation and disease burden in the earliest phases of the disease. Thus, a cohort of 150 consecutive patients underwent clinical, neurophysiological and brain MRI assessment as well as lumbar puncture as part of their diagnostic work...
Source: Brain - April 8, 2021 Category: Neurology Source Type: research

Reply: From ‘loose fitting’ to high-performance, uncertainty-aware brain-age modelling
We appreciate the opportunity to defend our original claims and continue the discussion about the effects of regularization on deep learning-based brain age prediction. While we understand that this is a relatively unexplored topic and further inquiry is certainly needed to validate our results, we refute several of the claims presented in the letter fromHahnet al. (2021). (Source: Brain)
Source: Brain - April 7, 2021 Category: Neurology Source Type: research

From ‘loose fitting’ to high-performance, uncertainty-aware brain-age modelling
In brain-age modelling, a machine learning model is trained on a normative, usually healthy group of individuals to predict chronological age from neuroimaging data. This model is then applied to new data and the difference between predicted and chronological age —termed the brain-age gap (BAG)—is taken as a measure of deviation from ‘normal ageing’. This new area of research has generated large interest over the last decade and accelerated ageing has been associated with many different disorders and pathologies (Franke and Gaser, 2019). (Source: Brain)
Source: Brain - April 7, 2021 Category: Neurology Source Type: research

Corrigendum to: Monitoring the progression of Alzheimer's disease with τ-PET
Dietmar Rudolf Thal and Rik Vandenberghe. Monitoring the progression of Alzheimer ’s disease with τ-PET.Brain. 2016;139:1318 –1320. doi:10.1093/brain/aww057 (Source: Brain)
Source: Brain - April 6, 2021 Category: Neurology Source Type: research

Neuroinflammation is highest in areas of disease progression in semantic dementia
AbstractDespite epidemiological and genetic data linking semantic dementia to inflammation, the topography of neuroinflammation in semantic dementia, also known as the semantic variant of primary progressive aphasia, remains unclear. The pathology starts at the tip of the left temporal lobe where, in addition to cortical atrophy, a strong signal appears with the tau PET tracer18F-flortaucipir, even though the disease is not typically associated with tau but with TDP-43 protein aggregates. Here, we characterized the topography of inflammation in semantic variant primary progressive aphasia using high-resolution PET and the ...
Source: Brain - April 6, 2021 Category: Neurology Source Type: research

Identification of multiple system atrophy mimicking Parkinson ’s disease or progressive supranuclear palsy
AbstractWe studied a subset of patients with autopsy-confirmed multiple system atrophy who presented a clinical picture that closely resembled either Parkinson ’s disease or progressive supranuclear palsy. These mimics are not captured by the current diagnostic criteria for multiple system atrophy. Among 218 autopsy-proven multiple system atrophy cases reviewed, 177 (81.2%) were clinically diagnosed and pathologically confirmed as multiple system atrophy (i.e. typical cases), while the remaining 41 (18.8%) had received an alternative clinical diagnosis, including Parkinson’s disease (i.e. Parkinson’s disease mimics;n...
Source: Brain - April 5, 2021 Category: Neurology Source Type: research

Guillain-Barr é syndrome, SARS-CoV-2 and molecular mimicry
(Source: Brain)
Source: Brain - April 3, 2021 Category: Neurology Source Type: research

Currently available data regarding the potential association between COVID-19 and Guillain-Barr é syndrome
This study forms part of the Unusual Manifestations of Covid-19 (UMC-19) project, a retrospective, case-controlled, emergency department-based, multicentre study investigating the potential relationship between COVID and 10 different entities that could be influenced by SARS-Cov-2 infection.3 Our study showed that GBS is rarely observed as a form of COVID-19 presentation ( ∼0.15‰ cases). Nevertheless, in comparison with non-COVID-19 patients coming to the emergency department, patients with COVID presented a significantly higher relative frequency of GBS [odds ratio (OR) = 6.30, 95% confidence interval (CI) = 3.18–12...
Source: Brain - April 3, 2021 Category: Neurology Source Type: research

Reply: Guillain-Barr é syndrome, SARS-CoV-2 and molecular mimicry and Ongoing challenges in unravelling the association between COVID-19 and Guillain-Barré syndrome and Unclear association between COVID-19 and Guillain-Barré syndrome and Currently available data regarding the potential association between COVID-19 an d Guillain-Barré syndrome
(Source: Brain)
Source: Brain - April 3, 2021 Category: Neurology Source Type: research