Discriminating nonfluent/agrammatic and logopenic PPA variants with automatically extracted morphosyntactic measures from connected speech.

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.

Visual and social differences in dyslexia: deep phenotyping of four cases with spared phonology.

Diagnostic criteria for dyslexia describe specific reading difficulties, and single-deficit models, including the phonological deficit theory, have prevailed. Children seeking diagnosis, however, do not always show phonological deficits, and may present with strengths and challenges beyond reading. Through extensive neurological, neuropsychological, and academic evaluation, we describe four children with visuospatial, socio-emotional, and attention impairments and spared phonology, alongside long-standing reading difficulties.

Frontotemporal dementia: A unique window on the functional role of the temporal lobes.

Frontotemporal dementia (FTD) is an umbrella term covering a plethora of progressive changes in executive functions, motor abilities, behavior, and/or language. Different clinical syndromes have been described in relation to localized atrophy, informing on the functional networks that underlie these specific cognitive, emotional, and behavioral processes. These functional declines are linked with the underlying neurodegeneration of frontal and/or temporal lobes due to diverse molecular pathologies.

Automated Detection of Speech Timing Alterations in Autopsy-Confirmed Nonfluent/Agrammatic Variant Primary Progressive Aphasia.

Background And Objectives: Motor speech function, including speech timing, is a key domain for diagnosing nonfluent/agrammatic variant primary progressive aphasia (nfvPPA). Yet, standard assessments use subjective, specialist-dependent evaluations, undermining reliability and scalability. Moreover, few studies have examined relevant anatomo-clinical alterations in patients with pathologically confirmed diagnoses.

Children with developmental dyslexia show elevated parasympathetic nervous system activity at rest and greater cardiac deceleration during an empathy task.

Reading difficulties are the hallmark feature of dyslexia, but less is known about other areas of functioning. Previously, we found children with dyslexia exhibited heightened emotional reactivity, which correlated with better social skills. Whether emotional differences in dyslexia extend to the parasympathetic nervous system-an autonomic branch critical for attention, social engagement, and empathy-is unknown.

Dissociating nouns and verbs in temporal and perisylvian networks: Evidence from neurodegenerative diseases.

Naming of nouns and verbs can be selectively impaired in neurological disorders, but the specificity of the neural and cognitive correlates of such dissociation remains unclear. Functional imaging and stroke research sought to identify cortical regions selectively recruited for nouns versus verbs, yet findings are inconsistent. The present study investigated this issue in neurodegenerative diseases known to selectively affect different brain networks, thus providing new critical evidence of network specificity.

Functional and morphological correlates of developmental dyslexia: A multimodal investigation of the ventral occipitotemporal cortex.

Background And Purpose: The ventral occipitotemporal cortex (vOT) is a region crucial for reading acquisition through selective tuning to printed words. Developmental dyslexia is a disorder of reading with underlying neurobiological bases often associated with atypical neural responses to printed words. Previous studies have discovered anomalous structural development and function of the vOT in individuals with dyslexia.

Enhanced visceromotor emotional reactivity in dyslexia and its relation to salience network connectivity.

Dyslexia is a neurodevelopmental disorder mainly defined by reading difficulties. During reading, individuals with dyslexia exhibit hypoactivity in left-lateralized language systems. Lower activity in one brain circuit can be accompanied by greater activity in another, and, here, we examined whether right-hemisphere-based emotional reactivity may be elevated in dyslexia.

Verbal Semantics and the Left Dorsolateral Anterior Temporal Lobe: A Longitudinal Case of Bilateral Temporal Degeneration.

Background: Semantic variant primary progressive aphasia (svPPA), a clinical syndrome characterized by loss of semantic knowledge, is associated with neurodegeneration that starts in the anterior temporal lobe (ATL) and gradually spreads towards posterior temporal and medial frontal areas. At the earliest stages, atrophy may be predominantly lateralized to either the left or right ATL, leading to different clinical profiles with greatest impairment of word comprehension or visual/social semantics, respectively.

Methods & Procedures: We report the in-depth longitudinal investigation of cognitive and neuroanatomical features of JB, an unusual case of ATL neurodegeneration with relative sparing of left lateral ATL regions.

Speech production differences in English and Italian speakers with nonfluent variant PPA.

Objective: To understand whether the clinical phenotype of nonfluent/agrammatic primary progressive aphasia (nfvPPA) could present differences depending on the patient's native language.

Methods: In this cross-sectional study, we analyzed connected speech samples in monolingual English (nfvPPA-E) and Italian speakers (nfvPPA-I) who were diagnosed with nfvPPA and matched for age, sex, and Mini-Mental State Examination scores. Patients also received a comprehensive neuropsychological battery.

Task-Free Functional Language Networks: Reproducibility and Clinical Application.

Intrinsic connectivity networks (ICNs) identified through task-free fMRI (tf-fMRI) offer the opportunity to investigate human brain circuits involved in language processes without requiring participants to perform challenging cognitive tasks. In this study, we assessed the ability of tf-fMRI to isolate reproducible networks critical for specific language functions and often damaged in primary progressive aphasia (PPA). First, we performed whole-brain seed-based correlation analyses on tf-fMRI data to identify ICNs anchored in regions known for articulatory, phonological, and semantic processes in healthy male and female controls (HCs).

Differential intrinsic functional connectivity changes in semantic variant primary progressive aphasia.

The semantic variant of primary progressive aphasia (svPPA) is a clinical syndrome characterized by semantic memory deficits with relatively preserved motor speech, syntax, and phonology. There is consistent evidence linking focal neurodegeneration of the anterior temporal lobes (ATL) to the semantic deficits observed in svPPA. Less is known about large-scale functional connectivity changes in this syndrome, particularly regarding the interplay between affected and spared language networks that leads to the unique cognitive dissociations typical of svPPA.

Altered topology of the functional speech production network in non-fluent/agrammatic variant of PPA.

Non-fluent/agrammatic primary progressive aphasia (nfvPPA) is caused by neurodegeneration within the left fronto-insular speech and language production network (SPN). Graph theory is a branch of mathematics that studies network architecture (topology) by quantifying features based on its elements (nodes and connections). This approach has been recently applied to neuroimaging data to explore the complex architecture of the brain connectome, though few studies have exploited this technique in PPA.

Frontotemporal dementia.

Frontotemporal dementia (FTD) is a neurodegenerative disorder characterized by progressive changes in behavior, personality, and language with involvement of the frontal and temporal regions of the brain. About 40% of FTD cases have a positive family history, and about 10% of these cases are inherited in an autosomal-dominant pattern. These gene defects present with distinct clinical phenotypes.

Defects of mutant DNMT1 are linked to a spectrum of neurological disorders.

We report a broader than previously appreciated clinical spectrum for hereditary sensory and autonomic neuropathy type 1E (HSAN1E) and a potential pathogenic mechanism for DNA methyltransferase (DNMT1) mutations. The clinical presentations and genetic characteristics of nine newly identified HSAN1E kinships (45 affected subjects) were investigated. Five novel mutations of DNMT1 were discovered; p.

Elicitation of specific syntactic structures in primary progressive aphasia.

Many patients with primary progressive aphasia (PPA) are impaired in syntactic production. Because most previous studies of expressive syntax in PPA have relied on quantitative analysis of connected speech samples, which is a relatively unconstrained task, it is not well understood which specific syntactic structures are most challenging for these patients. We used an elicited syntactic production task to identify which syntactic structures pose difficulties for 31 patients with three variants of PPA: non-fluent/agrammatic, semantic and logopenic.

Syntactic processing depends on dorsal language tracts.

Frontal and temporal language areas involved in syntactic processing are connected by several dorsal and ventral tracts, but the functional roles of the different tracts are not well understood. To identify which white matter tract(s) are important for syntactic processing, we examined the relationship between white matter damage and syntactic deficits in patients with primary progressive aphasia, using multimodal neuroimaging and neurolinguistic assessment. Diffusion tensor imaging showed that microstructural damage to left hemisphere dorsal tracts--the superior longitudinal fasciculus including its arcuate component--was strongly associated with deficits in comprehension and production of syntax.

Lives in isolation: stories and struggles of low-income African American women with panic disorder.

Research evidence points to the existence of racial-ethnic disparities in both access to and quality of mental health services for African Americans with panic disorder. Current panic disorder evaluation and treatment paradigms are not responsive to the needs of many African Americans. The primary individual, social, and health-care system factors that limit African Americans' access to care and response to treatment are not well understood.

Adult glial precursor proliferation in mutant SOD1G93A mice.

The focus of most neurodegenerative disease studies has been on neuronal death in particular subpopulations of the central nervous system. The associated response of glial populations has been ascribed the term "reactive astrocytosis." This has been defined as the proliferation of astrocytes accompanied by cellular hypertrophy and changes in gene expression following injury to the central nervous system.

Neural regions essential for distinct cognitive processes underlying picture naming.

We hypothesized that distinct cognitive processes underlying oral and written picture naming depend on intact function of different, but overlapping, regions of the left hemisphere cortex, such that the distribution of tissue dysfunction in various areas can predict the component of the naming process that is disrupted. To test this hypothesis, we evaluated 116 individuals within 24 h of acute ischaemic stroke using a battery of oral and written naming and other lexical tests, and with magnetic resonance diffusion and perfusion imaging to identify the areas of tissue dysfunction. Discriminant function analysis, using the degree of hypoperfusion in various Brodmann's areas--BA 22 (including Wernicke's area), BA 44 (part of Broca's area), BA 45 (part of Broca's area), BA 21 (inferior temporal cortex), BA 37 (posterior, inferior temporal/fusiform gyrus), BA 38 (anterior temporal cortex) and BA 39 (angular gyrus)--as discriminant variables, classified patients on the basis of the primary component of the naming process that was impaired (defined as visual, semantics, modality-independent lexical access, phonological word form, orthographic word form and motor speech by the pattern of performance and types of errors across lexical tasks).