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Journal of Neurology Apr 2023Behavioural variant fronto-temporal dementia (bvFTD) is characterised by a progressive change in personality in association with atrophy of the frontal and temporal...
BACKGROUND
Behavioural variant fronto-temporal dementia (bvFTD) is characterised by a progressive change in personality in association with atrophy of the frontal and temporal lobes. Whilst language impairment has been described in people with bvFTD, little is currently known about the extent or type of linguistic difficulties that occur, particularly in the genetic forms.
METHODS
Participants with genetic bvFTD along with healthy controls were recruited from the international multicentre Genetic FTD Initiative (GENFI). Linguistic symptoms were assessed using items from the Progressive Aphasia Severity Scale (PASS). Additionally, participants undertook the Boston Naming Test (BNT), modified Camel and Cactus Test (mCCT) and a category fluency test. Participants underwent a 3T volumetric T1-weighted MRI, with language network regional brain volumes measured and compared between the genetic groups and controls.
RESULTS
76% of the genetic bvFTD cohort had impairment in at least one language symptom: 83% C9orf72, 80% MAPT and 56% GRN mutation carriers. All three genetic groups had significantly impaired functional communication, decreased fluency, and impaired sentence comprehension. C9orf72 mutation carriers also had significantly impaired articulation and word retrieval as well as dysgraphia whilst the MAPT mutation group also had impaired word retrieval and single word comprehension. All three groups had difficulties with naming, semantic knowledge and verbal fluency. Atrophy in key left perisylvian language regions differed between the groups, with generalised involvement in the C9orf72 group and more focal temporal and insula involvement in the other groups. Correlates of language symptoms and test scores also differed between the groups.
CONCLUSIONS
Language deficits exist in a substantial proportion of people with familial bvFTD across all three genetic groups. Significant atrophy is seen in the dominant perisylvian language areas and correlates with language impairments within each of the genetic groups. Improved understanding of the language phenotype in the main genetic bvFTD subtypes will be helpful in future studies, particularly in clinical trials where accurate stratification and monitoring of disease progression is required.
Topics: Humans; Frontotemporal Dementia; C9orf72 Protein; Magnetic Resonance Imaging; Atrophy; Language Development Disorders; tau Proteins; Mutation
PubMed: 36538154
DOI: 10.1007/s00415-022-11512-1 -
Continuum (Minneapolis, Minn.) Aug 2014This article reviews the various types of visual dysfunction that can result from lesions of the cerebral regions beyond the striate cortex.
PURPOSE OF REVIEW
This article reviews the various types of visual dysfunction that can result from lesions of the cerebral regions beyond the striate cortex.
RECENT FINDINGS
Patients with dyschromatopsia can exhibit problems with color constancy. The apperceptive form of prosopagnosia is associated with damage to posterior occipital and fusiform gyri, and an associative/amnestic form is linked to damage to more anterior temporal regions. Pure alexia can be accompanied by a surface dysgraphia. New word-length effect criteria distinguish pure alexia from hemianopic dyslexia. Subtler problems with perception of numbers and faces can be seen in patients with pure alexia as well. Also, a developmental form of topographic disorientation, which is due to problems with forming cognitive maps of the environment, has been discovered. In Balint syndrome, added features of decreased flexibility of attention in simultanagnosia include local and global capture. Balint syndrome can affect not just localization in space, but also in time, as manifest in sequence agnosia.
SUMMARY
Lesions at intermediate levels of a processing hierarchy can cause difficulty with color perception or motion perception. At a higher level, ventral lesions of the occipitotemporal lobes can lead to a variety of problems with object recognition. Dorsal lesions of the occipitoparietal lobes can cause difficulty with spatial localization and guidance.
Topics: Adult; Aged; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Perceptual Disorders; Vision Disorders; Visual Cortex; Visual Pathways; Visual Perception
PubMed: 25099101
DOI: 10.1212/01.CON.0000453311.29519.67 -
International Journal of School &... 2019Two studies were conducted of students with and without persisting Specific Learning Disabilities (SLDs-WL) in Grades 4 to 9 (M = 11 years, 11 months) that supported the...
Two studies were conducted of students with and without persisting Specific Learning Disabilities (SLDs-WL) in Grades 4 to 9 (M = 11 years, 11 months) that supported the hypotheses that CELF 4 parent ratings for listening (language by ear), speaking (language by mouth), reading (language by eye), and writing (language by hand) were correlated with both (a) normed, standardized behavioral measures of listening, speaking, reading, and writing achievement (Study 1, 94 boys and 61 girls); and (b) fMRI connectivity or DTI white matter integrity involving brain regions for primary motor functions or motor planning and control, or motor timing in a subsample of right handers who did not wear metal (Study 2, 28 boys and 16 girls). Results of these assessment studies, which have implications for planning instruction for three SLDs-WL (dysgraphia, dyslexia, and oral and written language learning disability [OWL LD]), show that more than multisensory instruction is relevant. Language by ear, by mouth, by eye, and by hand, as well as motor planning, control, and output skills and motor timing should also be considered. Research is also reviewed that supports other processes beyond multisensory input alone that should also be considered for students with SLDs-WL.
PubMed: 32123644
DOI: 10.1080/21683603.2018.1458357 -
Brain Communications 2022The Gerstmann syndrome is a constellation of neurological deficits that include agraphia, acalculia, left-right discrimination and finger agnosia. Despite a growing...
The Gerstmann syndrome is a constellation of neurological deficits that include agraphia, acalculia, left-right discrimination and finger agnosia. Despite a growing interest in this clinical phenomenon, there remains controversy regarding the specific neuroanatomic substrates involved. Advancements in data-driven, computational modelling provides an opportunity to create a unified cortical model with greater anatomic precision based on underlying structural and functional connectivity across complex cognitive domains. A literature search was conducted for healthy task-based functional MRI and PET studies for the four cognitive domains underlying Gerstmann's tetrad using the electronic databases PubMed, Medline, and BrainMap Sleuth (2.4). Coordinate-based, meta-analytic software was utilized to gather relevant regions of interest from included studies to create an activation likelihood estimation (ALE) map for each cognitive domain. Machine-learning was used to match activated regions of the ALE to the corresponding parcel from the cortical parcellation scheme previously published under the Human Connectome Project (HCP). Diffusion spectrum imaging-based tractography was performed to determine the structural connectivity between relevant parcels in each domain on 51 healthy subjects from the HCP database. Ultimately 102 functional MRI studies met our inclusion criteria. A frontoparietal network was found to be involved in the four cognitive domains: calculation, writing, finger gnosis, and left-right orientation. There were three parcels in the left hemisphere, where the ALE of at least three cognitive domains were found to be overlapping, specifically the anterior intraparietal area, area 7 postcentral (7PC) and the medial intraparietal sulcus. These parcels surround the anteromedial portion of the intraparietal sulcus. Area 7PC was found to be involved in all four domains. These regions were extensively connected in the intraparietal sulcus, as well as with a number of surrounding large-scale brain networks involved in higher-order functions. We present a tractographic model of the four neural networks involved in the functions which are impaired in Gerstmann syndrome. We identified a 'Gerstmann Core' of extensively connected functional regions where at least three of the four networks overlap. These results provide clinically actionable and precise anatomic information which may help guide clinical translation in this region, such as during resective brain surgery in or near the intraparietal sulcus, and provides an empiric basis for future study.
PubMed: 35706977
DOI: 10.1093/braincomms/fcac140 -
Scientific Reports Apr 2021Language outcomes after speech and language therapy in post-stroke aphasia are challenging to predict. This study examines behavioral language measures and resting state...
Language outcomes after speech and language therapy in post-stroke aphasia are challenging to predict. This study examines behavioral language measures and resting state fMRI (rsfMRI) as predictors of treatment outcome. Fifty-seven patients with chronic aphasia were recruited and treated for one of three aphasia impairments: anomia, agrammatism, or dysgraphia. Treatment effect was measured by performance on a treatment-specific language measure, assessed before and after three months of language therapy. Each patient also underwent an additional 27 language assessments and a rsfMRI scan at baseline. Patient scans were decomposed into 20 components by group independent component analysis, and the fractional amplitude of low-frequency fluctuations (fALFF) was calculated for each component time series. Post-treatment performance was modelled with elastic net regression, using pre-treatment performance and either behavioral language measures or fALFF imaging predictors. Analysis showed strong performance for behavioral measures in anomia (R = 0.948, n = 28) and for fALFF predictors in agrammatism (R = 0.876, n = 11) and dysgraphia (R = 0.822, n = 18). Models of language outcomes after treatment trained using rsfMRI features may outperform models trained using behavioral language measures in some patient populations. This suggests that rsfMRI may have prognostic value for aphasia therapy outcomes.
Topics: Aged; Aphasia; Brain; Brain Mapping; Female; Humans; Language Therapy; Magnetic Resonance Imaging; Male; Middle Aged; Patient-Specific Modeling; Prognosis; Stroke; Stroke Rehabilitation; Treatment Outcome
PubMed: 33875733
DOI: 10.1038/s41598-021-88022-z -
PloS One 2022Adults with specific learning disabilities (e.g., dyslexia, dysgraphia, and dyscalculia) reveal limitations in daily functioning in various life domains. Following...
Adults with specific learning disabilities (e.g., dyslexia, dysgraphia, and dyscalculia) reveal limitations in daily functioning in various life domains. Following previous evidence of deficient executive functions and unique sensory patterns in this population, this study examined how relationships between these two domains are expressed in daily functioning. Participants included 55 adults with specific learning disabilities and 55 controls matched by age, gender, socioeconomic status, and education. Participants completed a sociodemographic questionnaire, the Behavioral Rating Inventory of Executive Functions-adult version, and the Adolescent/Adult Sensory Profile. Results indicated significant relationships between executive functions (per the Behavioral Rating Inventory of Executive Functions-adult version) and sensory patterns (per the Adolescent/Adult Sensory Profile) as reflected in daily functioning. The low sensory registration pattern predicted 12% to 16% of the variance in the behavioral regulation index, metacognitive index, and general executive composite scores and was a significant predictor of specific executive function abilities. Results indicated that the difficulties of adults with specific learning disabilities in using executive function abilities efficiently might be tied to a high sensory threshold and passive self-regulation strategies. A deeper understanding of this population's sensory-executive mechanisms may improve evaluation and intervention processes. This understanding can consequently increase executive abilities for improved daily functioning and life satisfaction.
Topics: Adolescent; Adult; Executive Function; Humans; Learning Disabilities; Surveys and Questionnaires
PubMed: 35390062
DOI: 10.1371/journal.pone.0266385 -
Brain and Language Jan 2017We used fMRI to examine the neural substrates of sublexical phoneme-grapheme conversion during spelling in a group of healthy young adults. Participants performed a...
We used fMRI to examine the neural substrates of sublexical phoneme-grapheme conversion during spelling in a group of healthy young adults. Participants performed a writing-to-dictation task involving irregular words (e.g., choir), plausible nonwords (e.g., kroid), and a control task of drawing familiar geometric shapes (e.g., squares). Written production of both irregular words and nonwords engaged a left-hemisphere perisylvian network associated with reading/spelling and phonological processing skills. Effects of lexicality, manifested by increased activation during nonword relative to irregular word spelling, were noted in anterior perisylvian regions (posterior inferior frontal gyrus/operculum/precentral gyrus/insula), and in left ventral occipito-temporal cortex. In addition to enhanced neural responses within domain-specific components of the language network, the increased cognitive demands associated with spelling nonwords engaged domain-general frontoparietal cortical networks involved in selective attention and executive control. These results elucidate the neural substrates of sublexical processing during written language production and complement lesion-deficit correlation studies of phonological agraphia.
Topics: Adult; Agraphia; Brain Mapping; Cerebral Cortex; Female; Functional Laterality; Humans; Language; Magnetic Resonance Imaging; Male; Middle Aged; Occipital Lobe; Reading; Temporal Lobe; Writing; Young Adult
PubMed: 27838547
DOI: 10.1016/j.bandl.2016.10.001 -
Medicina (Kaunas, Lithuania) May 2022Recent advances in chemotherapy have led to the emergence of new types of anticancer agents. With these advances, cases of side effects that have not been witnessed in...
Recent advances in chemotherapy have led to the emergence of new types of anticancer agents. With these advances, cases of side effects that have not been witnessed in the past have emerged. The systems of side effect evaluation and their grading have been based on the existing knowledge, such as the CTCAE (Common Terminology Standard for Adverse Events) for evaluating adverse drug reactions in cancer chemotherapy clinical trials. Therefore, new types of side effects may be overlooked or underestimated. Blinatumomab is a bispecific T-cell-engager (BiTE) antibody with specificity for CD19 on B cells and CD3 on T cells. Neurological events, such as neuropathy and encephalopathy, are serious side effects of BiTE antibodies. We encountered a case of a 62-year-old woman who experienced short-term memory impairment and dysgraphia after the first blinatumomab administration for Philadelphia chromosome negative (Ph-) B-cell acute lymphoblastic leukemia (ALL). The CTCAE does not include dysgraphia as a classifier for antibody therapies, such as blinatumomab, and immune effector cell-associated neurotoxicity syndrome, which is defined as a Chimeric antigen receptor T cell therapy-related toxicity; dysgraphia is included in the list of symptoms but is not graded. In this case, the severity of dysgraphia differed depending on the complexity of the letters examined. There is no report that the severity of dysgraphia depends on the letters' complexity, and therefore, it may be overlooked when using simple letters. We have reported the characteristics of dysgraphia in this case and the differences observed when judging different letters.
Topics: Agraphia; Antibodies, Bispecific; Antigens, CD19; Antineoplastic Agents; Female; Handwriting; Humans; Middle Aged
PubMed: 35743996
DOI: 10.3390/medicina58060733 -
BMC Psychiatry Feb 2024Children with specific learning disabilities (SLDs) and their parents experience many problems that may influence their interactions. The study aimed to evaluate the...
BACKGROUND
Children with specific learning disabilities (SLDs) and their parents experience many problems that may influence their interactions. The study aimed to evaluate the maternal acceptance/rejection status of children with SLDs and their associations with sociodemographic characteristics, and problem behaviors.
METHODS
The Parental Acceptance-Rejection/Control Questionnaire (PARQ/C) and Strengths and Difficulties Questionnaire (SDQ) were applied to the mothers with children aged 7-17 years with a diagnosis of SLD.
RESULTS
Among 266 children enrolled, the mean age was 10.2 years, and 61.7% were male, the mean score was 30.4 for warmth/affection, 25.8 for hostility/aggression, 22.9 for indifference/neglect, 16.3 for undifferentiated rejection, 95.4 for the total PARQ, and 40.8 for the control scales. Generalized linear models revealed that maternal depression, poor family income, parental smoking, and presence of dysgraphia, and poor total difficulties and prosocial scores of SDQ subscales were associated with the maternal acceptance-rejection. There was an interaction between the maternal control subscale and the school success of the child.
CONCLUSION
Mothers of children with SLDs had high maternal rejection scores which were associated with unfavorable characteristics of child and family. Early detection and giving appropriate support of these cases could improve the mother's relationship with her SLD child.
Topics: Humans; Child; Female; Male; Parents; Mothers; Parent-Child Relations; Surveys and Questionnaires; Learning Disabilities
PubMed: 38350976
DOI: 10.1186/s12888-024-05584-8 -
Frontiers in Psychiatry 2021Writing disorders are frequent and impairing. However, social robots may help to improve children's motivation and to propose enjoyable and tailored activities. Here, we...
Writing disorders are frequent and impairing. However, social robots may help to improve children's motivation and to propose enjoyable and tailored activities. Here, we have used the scenario in which a child is asked to teach a robot how to write via demonstration on a tablet, combined with a series of games we developed to train specifically pressure, tilt, speed, and letter liaison controls. This setup was proposed to a 10-year-old boy with a complex neurodevelopmental disorder combining phonological disorder, attention deficit/hyperactivity disorder, dyslexia, and developmental coordination disorder with severe dysgraphia. Writing impairments were severe and limited his participation in classroom activities despite 2 years of specific support in school and professional speech and motor remediation. We implemented the setup during his occupational therapy for 20 consecutive weekly sessions. We found that his motivation was restored; avoidance behaviors disappeared both during sessions and at school; handwriting quality and posture improved dramatically. In conclusion, treating dysgraphia using child-robot interaction is feasible and improves writing. Larger clinical studies are required to confirm that children with dysgraphia could benefit from this setup.
PubMed: 33716812
DOI: 10.3389/fpsyt.2021.596055