Publications by authors named "Maria Luisa Gorno-Tempini"

Article Synopsis
  • Poor affect recognition is an early indicator of frontotemporal dementia (FTD), and this study used the Comprehensive Affect Testing System (CATS-A) to assess emotional dysfunction in FTD patients compared to healthy controls.
  • The study involved 139 FTD patients of various subtypes and tested them alongside 116 healthy controls, measuring their Affective Recognition Quotient (ARQ) to evaluate emotional recognition accuracy.
  • Results showed that the CATS-A effectively differentiated between FTD patients and healthy controls with high accuracy (AUC 0.89) and had robust internal reliability, suggesting it's a valuable clinical tool for assessing emotional dysfunction in FTD.
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Based on historic observations that children with reading disabilities were disproportionately both male and non-right-handed, and that early life insults of the left hemisphere were more frequent in boys and non-right-handed children, it was proposed that early focal neuronal injury disrupts typical patterns of motor hand and language dominance and in the process produces developmental dyslexia. To date, these theories remain controversial. We revisited these earliest theories in a contemporary manner, investigating demographics associated with reading disability, and in a subgroup with and without reading disability, compared structural imaging as well as patterns of activity during tasks of verb generation and non-word repetition using magnetoencephalography source imaging.

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Cross-linguistic studies with healthy individuals are vital, as they can reveal typologically common and different patterns while providing tailored benchmarks for patient studies. Nevertheless, cross-linguistic differences in narrative speech production, particularly among speakers of languages belonging to distinct language families, have been inadequately investigated. Using a picture description task, we analyze cross-linguistic variations in connected speech production across three linguistically diverse groups of cognitively normal participants-English, Chinese (Mandarin and Cantonese), and Italian speakers.

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Article Synopsis
  • Semantic dementia (SD) patients, particularly those with semantic variant primary progressive aphasia (svPPA) and semantic behavioral variant frontotemporal dementia (sbvFTD), exhibit challenges in identifying faces due to atrophy in the anterior temporal lobe (ATL).
  • The study involved 74 SD patients and 36 healthy controls, who underwent various face recognition and semantic processing tests, alongside structural MRI scans to assess neural correlates.
  • Findings indicated that while both patient groups struggled with semantic face tasks, they performed similarly on perceptual face tests, suggesting that perceptual deficits may not arise until later stages of the disease with more extensive ATL atrophy.
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Developmental dyslexia is typically associated with difficulties in basic auditory processing and in manipulating speech sounds. However, the neuroanatomical correlates of auditory difficulties in developmental dyslexia (DD) and their contribution to individual clinical phenotypes are still unknown. Recent intracranial electrocorticography findings associated processing of sound amplitude rises and speech sounds with posterior and middle superior temporal gyrus (STG), respectively.

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  • The study aimed to assess the clinical usability of the Comprehensive Affect Testing System (CATS) in recognizing emotional responses in Italian ALS patients.
  • A total of 96 ALS patients and 116 healthy controls participated in neuropsychological assessments, revealing that the CATS-A measures demonstrated good diagnostic accuracy for distinguishing between ALS patients with cognitive impairments and those without.
  • The findings suggested that CATS-A ARQ is an effective tool for identifying affect recognition deficits in ALS, which could indicate frontotemporal brain involvement in these patients.
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Article Synopsis
  • * Researchers used automated speech analysis on audio-recorded picture descriptions from 40 FTD patients and 22 healthy controls to identify linguistic features that could help distinguish between the two types of atrophy associated with each variant.
  • * The analysis revealed key speech features that could differentiate between FTD patients and healthy controls as well as between the two variants of FTD, suggesting potential for a non-invasive diagnostic tool that correlates with specific brain areas involved in language and
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Introduction: Frontotemporal lobar degeneration (FTLD) is associated with FTLD due to tau (FTLD-tau) or TDP (FTLD-TDP) inclusions found at autopsy. Arterial Spin Labeling (ASL) MRI is often acquired in the same session as a structural T1-weighted image (T1w), enabling detection of regional changes in cerebral blood flow (CBF). We hypothesize that ASL-T1w registration with more degrees of freedom using boundary-based registration (BBR) will better align ASL and T1w images and show increased sensitivity to regional hypoperfusion differences compared to manual registration in patient participants.

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  • Some sports and repeated head injuries (like playing football) might lead to brain problems later in life, especially conditions like Frontotemporal Dementia (FTD) and Primary Progressive Aphasia (PPA).
  • Researchers compared people with FTD/PPA to healthy ones to see how many had Traumatic Brain Injuries (TBI) and head impacts.
  • They found that people with FTD/PPA had more sports experience causing head impacts, and those with a history of head injuries had symptoms show up earlier than those without.
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Introduction: Alzheimer's disease (AD) neuropathological changes present with amnestic and nonamnestic (atypical) syndromes. The contribution of comorbid neuropathology as a substratum of atypical expression of AD remains under investigated.

Methods: We examined whether atypical AD exhibited increased comorbid neuropathology compared to typical AD and if such neuropathologies contributed to the accelerated clinical decline in atypical AD.

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Article Synopsis
  • Semantic dementia (SD) patients, including those with semantic variant primary progressive aphasia (svPPA) and semantic behavioral variant frontotemporal dementia (sbvFTD), struggle with identifying faces and known individuals due to right anterior temporal lobe (ATL) atrophy, but the presence of perceptual deficits in face recognition is still uncertain.
  • A study involving 74 SD patients and 36 cognitively healthy controls used a series of face processing tests and MRI scans to investigate the relationship between face recognition performance and brain structure.
  • Results showed that both svPPA and sbvFTD patients had significant impairments in semantic face processing tasks, but they performed well on perceptual face recognition tests, indicating that perceptual abilities
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Article Synopsis
  • A multicenter study involving 18 centers in 12 countries examined 360 patients with frontotemporal dementia (FTD) characterized by right anterior temporal lobe (RATL) atrophy to create a unified clinical description of the syndrome.
  • Key symptoms identified in patients included mental rigidity (78%), disinhibition (74%), and naming difficulties (70%), with cognitive tests revealing specific deficits in social interactions and emotional recognition, though lacking in measuring mental rigidity.
  • The findings represent the largest cohort of its kind, highlighting under-acknowledged symptoms of RATL and providing valuable insights for clinicians to improve early diagnosis and management of FTD patients.
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  • Children with dyslexia show heightened emotional sensitivity, demonstrating more robust reactions to emotional stimuli compared to their peers without dyslexia.
  • A study measured facial expressions, skin reactions, and emotional responses to a sudden loud noise, finding that children with dyslexia had greater reactions.
  • Increased physiological responses in children with dyslexia were linked to higher levels of anxiety, suggesting that their stronger emotional reactions may contribute to common anxiety symptoms in this group.
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Frontotemporal lobar degeneration with neuronal inclusions of the TAR DNA-binding protein 43 (FTLD-TDP) is a fatal neurodegenerative disorder with only a limited number of risk loci identified. We report our comprehensive genome-wide association study as part of the International FTLD-TDP Whole-Genome Sequencing Consortium, including 985 cases and 3,153 controls, and meta-analysis with the Dementia-seq cohort, compiled from 26 institutions/brain banks in the United States, Europe and Australia. We confirm as the strongest overall FTLD-TDP risk factor and identify as a novel FTLD-TDP risk factor.

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Objective: We compared the accuracy of amyloid and [F]Flortaucipir (FTP) tau positron emission tomography (PET) visual reads for distinguishing patients with mild cognitive impairment (MCI) or dementia with fluid biomarker support of Alzheimer's disease (AD).

Methods: Participants with FTP-PET, amyloid-PET, and diagnosis of dementia-AD (n = 102), MCI-AD (n = 41), non-AD diseases (n = 76), and controls (n = 20) were included. AD status was determined independent of PET by cerebrospinal fluid or plasma biomarkers.

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Article Synopsis
  • About 5% of Alzheimer’s patients show symptoms before they turn 65, which is called early-onset Alzheimer's disease.
  • There are two types: sporadic (happens by chance) and dominantly inherited (passed down from family).
  • This study looked at brain changes in both types and included tests on 134 sporadic cases, 89 inherited cases, and 102 people without Alzheimer’s to compare how they were affected.
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We present a longitudinal description of a man with the I383V variant of frontotemporal dementia (FTD). His progressive changes in behavior and language resulted in a diagnosis of the right temporal variant of FTD, also called the semantic behavioral variant (sbvFTD). We also present data from a small series of patients with the I383V variant who were enrolled in a nationwide FTD research collaboration (ALLFTD).

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Article Synopsis
  • Bilingualism is believed to enhance executive functioning and may delay dementia symptoms, but its effects on cognitive reserve in frontotemporal dementia (FTD) haven't been widely studied.
  • The researchers hypothesized that bilingual individuals with behavioral variant FTD would show an older age of symptom onset compared to monolinguals but didn't expect this for other types of primary progressive aphasia (PPA).
  • Contrary to expectations, the study found no significant differences in age at symptom onset or neuropsychological performance between bilingual and monolingual patients across all FTD variants, suggesting bilingualism does not provide the expected protective effect in this group.
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Objective: Brain areas implicated in semantic memory can be damaged in patients with epilepsy (PWE). However, it is challenging to delineate semantic processing deficits from acoustic, linguistic, and other verbal aspects in current neuropsychological assessments. We developed a new Visual-based Semantic Association Task (ViSAT) to evaluate nonverbal semantic processing in PWE.

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Models of speech perception are centered around a hierarchy in which auditory representations in the thalamus propagate to primary auditory cortex, then to the lateral temporal cortex, and finally through dorsal and ventral pathways to sites in the frontal lobe. However, evidence for short latency speech responses and low-level spectrotemporal representations in frontal cortex raises the question of whether speech-evoked activity in frontal cortex strictly reflects downstream processing from lateral temporal cortex or whether there are direct parallel pathways from the thalamus or primary auditory cortex to the frontal lobe that supplement the traditional hierarchical architecture. Here, we used high-density direct cortical recordings, high-resolution diffusion tractography, and hemodynamic functional connectivity to evaluate for evidence of direct parallel inputs to frontal cortex from low-level areas.

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Background And Objectives: Cavum septum pellucidum (CSP) is a common but nonspecific MRI finding in individuals with prior head trauma. The type and extent of head trauma related to CSP, CSP features specific to head trauma, and the impact of brain atrophy on CSP are unknown. We evaluated CSP cross-sectionally and longitudinally in healthy and clinically impaired older adults who underwent detailed lifetime head trauma characterization.

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Morphosyntactic assessments are important for characterizing individuals with nonfluent/agrammatic variant primary progressive aphasia (nfvPPA). Yet, standard tests are subject to examiner bias and often fail to differentiate between nfvPPA and logopenic variant PPA (lvPPA). Moreover, relevant neural signatures remain underexplored.

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Article Synopsis
  • The study focused on primary progressive aphasia (PPA) in native Chinese speakers, highlighting the unique challenges posed by the classifier system in Chinese compared to Indo-European languages.
  • Results showed that both semantic variant (sv) PPA and logopenic variant (lv) PPA patients struggled significantly with classifier production, with lvPPA patients performing better in recognition tasks.
  • The findings indicate that classifier processing could serve as a linguistic marker for distinguishing between different PPA variants, with performance linked to specific brain regions involved in language and visual processing.
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Here we build from the central strength of the existing definition of dyslexia-its emphasis on neurobiological origins-and proffer a set of seven core principles for a new, more comprehensive conceptualization of dyslexia. These principles derive from two major research directions: (1) the still evolving history of attempts to explain dyslexia, including in varied writing systems; and (2) the study of the reading brain circuit, its development, and its genetic and environmental influences. What emerges from connecting these two directions is a dynamic conceptualization of dyslexia that incorporates the extensive research on the heterogeneity of dyslexia and the interdependent contributions of multiple biological and socio-cultural risk and preventive factors.

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Article Synopsis
  • - The study examines the relationship between brain atrophy and functional connectivity in Alzheimer's disease (AD) and frontotemporal dementia (FTD) using MRI data from 221 patients and 100 healthy individuals.
  • - Three main structure-function components were identified: overall atrophy related to general hypo- and hyper-connectivity, and syndrome-specific atrophy linked to connectivity changes around brain lesions.
  • - The findings suggest that changes in brain connectivity are tied to cognitive deficits, with specific alterations in brain activity patterns that might explain the observed atrophy-driven connectivity issues.
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