In describing academic attainment in autism spectrum disorders (ASD), results are typically reported at the group mean level. This may mask subgroups of individuals for whom achievement is incommensurate with intellectual ability. The authors tested IQ, literacy, and mathematical abilities a large (N = 100) adolescents (14-16 years old) ASD. Seventy-three percent sample had least one area literacy or that was highly discrepant (approximately 14 standard score points) from full-scale IQ...

Abstract Despite considerable interest in improving clinical and neurobiological characterisation of frontotemporal dementia defining the role brain network disintegration its pathogenesis, information about white matter pathway alterations remains limited. Here we investigated tract damage using an unbiased, template‐based diffusion tensor imaging (DTI) protocol a cohort 27 patients with behavioral variant (bvFTD) representing both major genetic sporadic forms, relation to healthy...

Symptoms suggesting altered processing of pain and temperature have been described in dementia diseases may contribute importantly to clinical phenotypes, particularly the frontotemporal lobar degeneration spectrum, but basis for these symptoms has not characterized detail. Here we analysed using a semi-structured caregiver questionnaire recording behavioural responsiveness or cohort patients with (n = 58, 25 female, aged 52–84 years, representing major syndromes representative pathogenic...

The primary progressive aphasias (PPA) are a heterogeneous group of language-led neurodegenerative diseases resulting from large-scale brain network degeneration. White matter (WM) pathways bind networks together, and might therefore hold information about PPA pathogenesis. Here we used diffusion tensor imaging tract-based spatial statistics to compare WM tract changes between syndromes with respect Alzheimer's disease healthy controls in 33 patients (13 nonfluent/agrammatic PPA); 10...

Abstract Introduction Frontotemporal dementia (FTD) is a common cause of early-onset with significant genetic component, as underlined by the recent identification repeat expansions in gene C9ORF72 major FTD and motor neuron disease. Understanding neurobiology clinical phenomenology this novel mutation currently research focus. However, few data are available concerning longitudinal evolution Here we present neuropsychological neuroimaging on cohort patients pathological . Methods Following...

Objective Novel biomarkers for monitoring progression in neurodegenerative conditions are needed. Measurement of microstructural changes white matter (WM) using diffusion tensor imaging (DTI) may be a useful outcome measure. Here we report trajectories WM change serial DTI cohort with behavioral variant frontotemporal dementia (bvFTD). Methods Twenty‐three patients bvFTD (12 having genetic mutations), and 18 age‐matched control participants were assessed neuropsychological batteries at...

Impairments of social cognition are often leading features in frontotemporal lobar degeneration (FTLD) and likely to reflect large-scale brain network disintegration.However, the neuroanatomical basis impaired FTLD role white matter connections have not been defined.Here we assessed a cohort patients representing two core syndromes FTLD, behavioural variant dementia (bvFTD; n = 29) semantic primary progressive aphasia (svPPA; 15), relative healthy older individuals (n 37) using components...

Patients with dementia may exhibit abnormally altered liking for environmental sounds and music but such auditory hedonic responses have not been studied systematically. Here we addressed this issue in a cohort of 73 patients representing major canonical syndromes (behavioural variant frontotemporal (bvFTD), semantic (SD), progressive nonfluent aphasia (PNFA) amnestic Alzheimer's disease (AD)) using semi-structured caregiver behavioural questionnaire voxel-based morphometry (VBM) patients'...

The location and motion of sounds in space are important cues for encoding the auditory world. Spatial processing is a core component scene analysis, cognitively demanding function that vulnerable Alzheimer's disease. Here we designed novel neuropsychological battery based on virtual paradigm to assess spatial patient cohorts with clinically typical disease (n = 20) its major variant syndrome, posterior cortical atrophy 12) relation healthy older controls 26). We assessed three dimensions...

Despite considerable recent interest, the biological basis and clinical diagnosis of behavioural variant frontotemporal dementia (bvFTD) pose unresolved problems. Mentalising (the cognitive capacity to interpret behaviour oneself others in terms mental states) is impaired as a prominent feature bvFTD, consistent with involvement brain regions including ventro-medial prefrontal cortex (PFC), orbitofrontal anterior temporal lobes. Here, we investigated mentalising ability cohort patients bvFTD...

Sense of humor is potentially relevant to social functioning in dementias, but has been little studied these diseases. We designed a semi-structured informant questionnaire assess behavior and preferences patients with behavioral variant frontotemporal dementia (bvFTD; n = 15), semantic (SD; 7), progressive nonfluent aphasia (PNFA; 10), Alzheimer’s disease (AD; 16) versus healthy age-matched individuals (n 21). Altered (including frankly inappropriate) responses were significantly more...

Auditory scene analysis is a demanding computational process that performed automatically and efficiently by the healthy brain but vulnerable to neurodegenerative pathology of Alzheimer's disease. Here we assessed functional neuroanatomy auditory in disease using well-known 'cocktail party effect' as model paradigm whereby stored templates for objects (e.g., hearing one's spoken name) are used segregate 'foreground' 'background'. Patients with typical amnestic (n = 13) age-matched...

Humour is a complex cognitive and emotional construct that vulnerable in neurodegenerative diseases, notably the frontotemporal lobar degenerations. However, humour processing these diseases has been little studied. Here we assessed patients with behavioural variant dementia (n = 22, mean age 67 years, four female) semantic 11, five relative to healthy individuals 21, 66 11 female), using joint neuroanatomical approach. We created novel neuropsychological test requiring decision about...

Despite much recent interest in music and dementia, perception has not been widely studied across dementia syndromes using an information processing approach. Here we addressed this issue a cohort of 30 patients representing major typical Alzheimer's disease (AD, n = 16), logopenic aphasia (LPA, Alzheimer variant syndrome; 5), progressive nonfluent (PNFA; 9) relation to 19 healthy age-matched individuals. We designed novel neuropsychological battery assess musical patterns the dimensions...

<h3>Background</h3> Mutations in C9ORF72 are an important cause of frontotemporal dementia (FTD) and motor neuron disease. Accumulating evidence suggests that FTD associated with mutations (C9ORF72-FTD) is distinguished clinically by early prominent neuropsychiatric features might collectively reflect deranged body schema processing. However, the pathophysiology C9ORF72-FTD has not been elucidated. <h3>Methods</h3> We undertook a detailed neurophysiological investigation five patients...

Crowding is a breakdown in the ability to identify objects clutter, and major constraint on object recognition. particularly impairs perception peripheral, amblyopic possibly developing vision. Here we argue that crowding also critical factor limiting central vision of individuals with neurodegeneration occipital cortices. In current study, posterior cortical atrophy (n = 26), typical Alzheimer's disease 17) healthy control subjects 14) completed centrally-presented tests letter...

Emotional behavioral disturbances are hallmarks of many dementias but their pathophysiology is poorly understood. Here we addressed this issue using the paradigm emotionally salient sounds.

The current study examined motor timing in frontotemporal dementia (FTD), which manifests as progressive deterioration social, behavioural and cognitive functions. Twenty-patients fulfilling consensus clinical criteria for variant FTD (bvFTD), 11 patients semantic-variant primary aphasia (svPPA), four nonfluent/agrammatic (naPPA), eight Alzheimer׳s disease (AD), 31 controls were assessed on both an externally- self-paced finger-tapping task requiring maintenance of a regular, 1500 ms beat...

Abnormal responsiveness to salient sensory signals is often a prominent feature of dementia diseases, particularly the frontotemporal lobar degenerations, but has been little studied. Here we assessed processing one important class signals, looming sounds, in canonical syndromes. We manipulated tones using intensity cues create percepts approaching ("looming") or less withdrawing sounds. Pupil dilatation responses and behavioral rating these stimuli were compared patients fulfilling...

Recognition of nonverbal sounds in semantic dementia and other syndromes anterior temporal lobe degeneration may determine clinical symptoms help to define phenotypic profiles. However, auditory function has not been widely studied these syndromes. Here we investigated processing two key domains - environmental melodies patients with (SD group; n=9) atrophy presenting behavioural decline (TL n=7, including four cases MAPT mutations) relation healthy older controls (n=20). We assessed...

Deficits of auditory scene analysis accompany Alzheimer's disease (AD). However, the functional neuroanatomy spatial sound processing has not been defined in AD. We addressed this using a "sparse" fMRI virtual paradigm 14 patients with typical AD relation to 16 healthy age-matched individuals. Sound stimulus sequences discretely varied perceived location and pitch source factorial design. was associated loss differentiated cortical profiles at prescribed threshold, significant group...

Memory for music has attracted much recent interest in Alzheimer's disease but the underlying brain mechanisms have not been defined patients directly. Here we addressed this issue an cohort using activation fMRI of two core musical memory systems. We studied 34 with younger onset led either by episodic decline (typical disease) or visuospatial impairment (posterior cortical atrophy) relation to 19 age-matched healthy individuals. designed a novel paradigm based on passive listening melodies...