1,721,261 research outputs found

    Assessment of psychiatric changes in C9ORF72 frontotemporal dementia

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    Recent neuroimaging evidence highlights cerebellar atrophy as one feature of frontotemporal dementia (FTD) with C9ORF72 mutation. Interestingly, C9ORF72 patients do not present with classic cerebellar symptoms, such as ataxia, but have instead a higher incidence of psychiatric changes compared to sporadic FTD. To date there exists no objective tool to assess such psychiatric changes due to cerebellar dysfunction. In the previous edition of Alzheimer's Research & Treatment, Downey and colleagues present a novel task, including a new apparatus, that targets such psychiatric disturbances. In the task participants are required to make self-other attributions, which have been shown to be dependent on the cerebellum in functional neuroimaging in healthy subjects. The data Downey and colleagues present on a case of C9ORF72 compared to four age-matched controls reveal that the patient shows impaired judgement only for other induced actions. These findings highlight the sensitivity of such a simple task to tap into potential cerebellar dysfunction in C9ORF72. Future studies are needed to now to determine whether this task is mediated solely via the cerebellum and is disease specific to C9ORF72. Nevertheless, this study is an important first step in the development of cerebellar-specific tasks tapping into psychiatric dysfunction, which will inform future diagnosis and disease management of patients with cerebellar dysfunction, and in particular C9ORF72

    Beyond and below the cortex: The contribution of striatal dysfunction to cognition and behaviour in neurodegeneration

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    Investigations of cognitive and behavioural changes in neurodegeneration have been mostly focussed on how cortical changes can explain these symptoms. In the proposed review, we will argue that the striatum has been overlooked as a critical nexus in understanding the generation of such symptoms. Although the striatum is historically more associated with motor dysfunction, there is increasing evidence from functional neuroimaging studies in the healthy that striatal regions modulate behaviour and cognition. This should not be surprising, as the striatum has strong anatomical connections to many cortical regions including the frontal, temporal and insula lobes, as well as some subcortical regions (amygdala, hippocampus). To date, however, it is largely unclear to what extent striatal regions are affected in many neurodegenerative conditions - and if so, how striatal dysfunction can potentially influence cognition and behaviour. The proposed review will examine the existing evidence of striatal changes across selected neurodegenerative conditions (Parkinson's disease, progressive supranuclear palsy, Huntington's disease, motor neuron disease, frontotemporal dementia and Alzheimer's disease), and will document their link with the cognitive and behavioural impairments observed. Thus, by reviewing the varying degrees of cortical and striatal changes in these conditions, we can start outlining the contributions of the striatal nexus to cognitive and behavioural symptoms. In turn, this knowledge will inform future studies investigating corticostriatal networks and also diagnostic strategies, disease management and future therapeutics of neurodegenerative conditions

    Inhibitory dysfunction in frontotemporal dementia:A review

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    Failure of inhibitory control is an early and consistent feature in patients suffering from frontotemporal dementia (FTD). This appears because of their pervasive ventromedial prefrontal atrophy - particularly in the orbitofrontal cortex - which has been linked to inhibitory dysfunction in studies on human and monkey lesions. However, the range of measures currently available to assess inhibitory processes in FTD is limited, and, as such, inhibitory dysfunction in FTD remains relatively underexplored. Subjective caregiver questionnaires are useful for defining disinhibition as it manifests behaviorally; however, endorsement of symptoms can vary largely across patients as it is contingent on the perceptiveness of the caregiver. The few objective neuropsychological tasks that tap directly into inhibitory functioning have potential, although they mostly rely on intact language and semantics, which can confound performance in FTD patients. An emergent possibility is to explore inhibitory functioning in FTD through nonverbal experimental tasks. Adaptation of such experimental tasks into clinical tools is a promising avenue for exploring one of the earliest behavioral features in FTD patients and concomitantly tap into their prevalent orbitofrontal cortex dysfunction. We suggest that improved characterization of early inhibitory dysfunction may facilitate more accurate diagnosis of FTD

    Feasibility and reliability of online vs in-person cognitive testing in healthy older people

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    BackgroundEarly evidence in using online cognitive assessments show that they could offer a feasible and resource-efficient alternative to in-person clinical assessments in evaluating cognitive performance, yet there is currently little understanding about how these assessments relate to traditional, in-person cognitive tests.ObjectivesIn this preliminary study, we assess the feasibility and reliability of NeurOn, a novel online cognitive assessment tool. NeurOn measures various cognitive domains including processing speed, executive functioning, spatial working memory, episodic memory, attentional control, visuospatial functioning, and spatial orientation.DesignThirty-two participants (mean age: 70.19) completed two testing sessions, unsupervised online and in-person, one-week apart. Participants were randomised in the order of testing appointments. For both sessions, participants completed questionnaires prior to a cognitive assessment. Test-retest reliability and concurrent validity of the online cognitive battery was assessed using intraclass correlation coefficients (ICCs) and correlational analysis, respectively. This was conducted by comparing performance in repeated tasks across testing sessions as well as with traditional, in-person cognitive tests.ResultsGlobal cognition in the NeurOn battery moderately validated against MoCA performance, and the battery demonstrated moderate test-retest reliability. Concurrent validity was found only between the online and paper versions of the Trail Making Test -A, as well as global cognitive performance between online and in-person testing sessions.ConclusionsThe NeurOn cognitive battery provides a promising tool for measuring cognitive performance online both longitudinally and across short retesting intervals within healthy older adults. When considering cost-effectiveness, flexible administration, and improved accessibility for wider populations, online cognitive assessments show promise for future screening of neurodegenerative diseases
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