Article
Clinical Neurology
Christopher R. S. Belder, Charles R. Marshall, Jessica Jiang, Salvatore Mazzeo, Anthipa Chokesuwattanaskul, Jonathan D. Rohrer, Anna Volkmer, Chris J. D. Hardy, Jason D. Warren
Summary: This article reviews recent progress in the diagnosis and management of primary progressive aphasia, the language-led dementias. It poses six key unanswered questions and suggests that linking proteinopathies to phenotypes may help resolve the clinical complexity of this disease.
JOURNAL OF NEUROLOGY
(2023)
Article
Clinical Neurology
Mario F. Mendez, Alexander Sheppard, Diana Chavez, Kelsey A. Holiday
Summary: Approximately 1 in 10 lvPPA patients present with severely unintelligible speech, indicating impairment in language comprehension and disease awareness extending to bilateral temporoparietal areas. Jargonaphasia can be a confusing presentation of AD.
JOURNAL OF THE NEUROLOGICAL SCIENCES
(2023)
Review
Clinical Neurology
Siddharth Ramanan, Muireann Irish, Karalyn Patterson, James B. Rowe, Maria Luisa Gorno-Tempini, Matthew A. Lambon Ralph
Summary: In this article, a new clinico-anatomical model is proposed to explore the role of left temporoparietal degeneration in non-linguistic cognitive deficits in logopenic variant primary progressive aphasia. By considering research on non-linguistic dysfunction, the authors suggest that the degeneration of temporal/inferior parietal cortices and connected regions can explain a significant portion of multidimensional cognitive features.
Article
Neurosciences
Maria Luisa Mandelli, Diego L. Lorca-Puls, Sladjana Lukic, Maxime Montembeault, Andrea Gajardo-Vidal, Abigail Licata, Aaron Scheffler, Giovanni Battistella, Stephanie M. Grasso, Rian Bogley, Buddhika M. Ratnasiri, Renaud La Joie, Nidhi S. Mundada, Eduardo Europa, Gil Rabinovici, Bruce L. Miller, Jessica De Leon, Maya L. Henry, Zachary Miller, Maria Luisa Gorno-Tempini
Summary: This study identified the specific cortical loci targeted by the logopenic variant of primary progressive aphasia (lvPPA) and investigated whether atrophy spreads through predetermined networks. The results showed that two distinct brain networks anchored to different epicenters were associated with symptomatology in lvPPA and that the strength of connectivity within these networks predicted atrophy progression.
HUMAN BRAIN MAPPING
(2023)
Article
Geriatrics & Gerontology
Valeria Isella, Daniele Licciardo, Gaia Rebecchi, Francesca Ferri, Cinzia Crivellaro, Ildebrando Appollonio, Carlo Ferrarese
Summary: We validated a clinical marker for diagnosing primary progressive aphasia (PPA) due to amyloid in a clinical setting, which was previously identified in an autopsy study. The marker includes impaired digit span (DS) as an index of phonological loop dysfunction and broadened criteria for logopenic PPA. The analysis showed moderate specificity (71%) but insufficient sensitivity (41%) for the proposed marker, and poor specificity (58%) for distinguishing between A+ PPA and the A- subgroup with nonfluent PPA.
NEUROBIOLOGY OF AGING
(2023)
Article
Clinical Neurology
Binyao Huang, Xiaolu Wang, Biao Jiang, Linlin Kong, Haifeng Hou, Jiong Zhou
Summary: The logopenic variant of primary progressive aphasia is characterized by word retrieval and sentence repetition difficulties, accompanied by mild auditory comprehension and naming deficits attributed to impaired access to lexical representations. Clinical treatment with memantine hydrochloride tablet and rivastigmine transdermal patch shows efficacy in slowing down cognitive deterioration.
FRONTIERS IN NEUROLOGY
(2022)
Article
Biology
Catherine Brodeur, Emilie Belley, Lisa-Marie Deschenes, Adriana Enriquez-Rosas, Michelyne Hubert, Anik Guimond, Josee Bilodeau, Jean-Paul Soucy, Joel Macoir
Summary: This study focused on three patients with language complaints and clinical profiles consistent with posterior cortical atrophy (PCA). The results showed that PCA is characterized by visuospatial and visuoperceptual deficits, as well as primary and secondary language impairments in these patients.
Article
Biology
Eloise Da Cunha, Alexandra Plonka, Seckin Arslan, Aurelie Mouton, Tess Meyer, Philippe Robert, Fanny Meunier, Valeria Manera, Auriane Gros
Summary: The study analyzed acoustic markers to differentiate between lvPPA and AD at an early stage of the disease. The results showed that temporal and prosodic markers can effectively distinguish between the two disorders. Acoustic profile comparisons also helped discriminate different lvPPA subgroups based on their biomarkers. The study suggests that acoustic analysis could be a clinically efficient alternative to lumbar punctures for neurodegenerative diagnosis and early care.
Review
Clinical Neurology
Justina Ruksenaite, Anna Volkmer, Jessica Jiang, Jeremy C. S. Johnson, Charles R. Marshall, Jason D. Warren, Chris J. D. Hardy
Summary: This review focuses on the key issues related to the diagnosis, disease stage assessment, proteinopathy phenotyping, core auditory impairments, treatment challenges, and pathophysiological features of each major variant of primary progressive aphasia (PPA). The new findings elucidating the pathophysiology of PPA represent significant progress in understanding these diseases, with implications for diagnosis, care, management, and therapies.
CURRENT NEUROLOGY AND NEUROSCIENCE REPORTS
(2021)
Article
Neurosciences
Leighton B. N. Hinkley, Megan Thompson, Zachary A. A. Miller, Valentina Borghesani, Danielle Mizuiri, Wendy Shwe, Abigail Licata, Seigo Ninomiya, Michael Lauricella, Maria Luisa Mandelli, Bruce L. L. Miller, John Houde, Maria Luisa Gorno-Tempini, Srikantan S. S. Nagarajan
Summary: Overlapping clinical presentations in primary progressive aphasia (PPA) variants present challenges for diagnosis and understanding pathophysiology, particularly in the early stages of the disease when behavioral (speech) symptoms are not clearly evident. Divergent atrophy patterns can partially account for speech production errors in the later stages of the disease. This study explores the neural oscillation abnormalities in two PPA variants, lvPPA and nfvPPA, and finds distinct patterns of reduced cortical oscillations that are unrelated to atrophy.
HUMAN BRAIN MAPPING
(2023)
Article
Neurosciences
Davide Quaranta, Sonia Di Tella, Camillo Marra, Simona Gaudino, Federica L'Abbate, Maria Caterina Silveri
Summary: The study compared the semantic features of words produced during narrative speech in svPPA and lvPPA, finding that svPPA had lower semantic depth index (SDI) compared to lvPPA, and the SDI was positively correlated with cortical thickness in specific brain regions.
Article
Clinical Neurology
Yuki Momota, Mika Konishi, Keisuke Takahata, Taishiro Kishimoto, Toshiki Tezuka, Shogyoku Bun, Hajime Tabuchi, Daisuke Ito, Masaru Mimura
Summary: This article reports a patient with logopenic variant primary progressive aphasia (lv-PPA) who was diagnosed with non-Alzheimer's disease (AD) tauopathy based on the results of multiple examinations. This case underscores the clinicopathologically heterogeneous nature of this syndrome.
FRONTIERS IN NEUROLOGY
(2022)
Review
Cell Biology
Francesca Conca, Valentina Esposito, Giada Giusto, Stefano F. Cappa, Eleonora Catricala
Summary: This study comprehensively analyzed the characteristics of lv-PPA and found a consistent core profile, characterized by linguistic and mild non-linguistic deficits, predominantly affecting the left temporal and parietal regions. However, variations in lv-PPA profiles may occur due to heterogeneity in neuropsychological assessment tools and diagnostic criteria adopted.
AGEING RESEARCH REVIEWS
(2022)
Article
Behavioral Sciences
Anja Staiger, Matthias L. Schroeter, Wolfram Ziegler, Theresa Schoelderle, Sarah Anderl-Straub, Adrian Danek, Thomas Duning, Klaus Fassbender, Klaus Fliessbach, Holger Jahn, Elisabeth Kasper, Johannes Kornhuber, Bernhard Landwehrmeyer, Martin Lauer, Jolina Lombardi, Albert Ludolph, Felix Mueller-Sarnowski, Maryna Polyakova, Catharina Prix, Johannes Prudlo, Frank Regenbrecht, Carola Rossmeier, Anja Schneider, Jens Wiltfang, Markus Otto, Janine Diehl-Schmid
Summary: The study revealed varying prevalence of MSDs among different types of PPA patients, with the highest proportion observed in nfvPPA patients. It also confirmed the presence of MSDs in svPPA and lvPPA patients.
Article
Neurosciences
David Foxe, Sau Chi Cheung, Nicholas J. Cordato, James R. Burrell, Rebekah M. Ahmed, Cathleen Taylor-Rubin, Muireann Irish, Olivier Piguet
Summary: Impaired verbal 'phonological' short-term memory is a key feature of logopenic variant of primary progressive aphasia. Distinguishing between different PPA variants can be challenging due to overlapping language profiles, and a combination of verbal short-term and working memory measures is crucial for understanding language disturbances in PPA. Additionally, visuospatial span tasks are essential for assessing PPA without language contamination.
Article
Geriatrics & Gerontology
Alinka C. Fisher, Sau C. Cheung, Claire M. C. O'Connor, Olivier Piguet
Summary: This pilot study investigated the acceptability and usefulness of a Positive Behaviour Support (PBS) education program for family carers of individuals with behavioural-variant frontotemporal dementia (bvFTD). The results indicate that the program was helpful in improving the carers' capability in providing behaviour support, with positive changes observed in their approach.
JOURNAL OF GERIATRIC PSYCHIATRY AND NEUROLOGY
(2023)
Review
Psychology, Clinical
Jan Van den Stock, Maxime Bertoux, Janine Diehl-Schmid, Olivier Piguet, Katherine P. Rankin, Florence Pasquier, Simon Ducharme, Yolande Pijnenburg, Fiona Kumfor
Summary: Dodich et al. reviewed the clinical use of social cognition assessment in behavioral variant frontotemporal dementia and proposed an initiative to address the limitations in their study.
NEUROPSYCHOLOGY REVIEW
(2023)
Article
Psychology
Marianne Jarsch, Olivier Piguet, Manfred Berres, Constantin Sluka, Anna Semenkova, Reto W. Kressig, Andreas U. Monsch, Skye McDonald, Marc Sollberger
Summary: This study aimed to develop the first German-language adaptation of TASIT-SIM, an ecologically valid test for assessing Theory of Mind (ToM) abilities in healthy adults. Thirteen scenes were selected and filmed at different intensities to determine the best materials for the test. Through Rasch analysis, intensity versions were chosen that showed optimal performance in participants with medium ToM abilities. In conclusion, the Basel Version of the Awareness of Social Inference Test - Theory of Mind (BASIT-ToM) was developed, incorporating the strengths of TASIT-SIM and addressing its limitations. Further validation of BASIT-ToM is needed in both healthy and clinical populations.
JOURNAL OF NEUROPSYCHOLOGY
(2023)
Review
Psychology, Experimental
Muireann Irish
Summary: Autobiographical memory is a crucial aspect of human cognition that allows us to recall significant events from our personal past and envision future goals and behavior. Dementia syndromes provide valuable insights into the cognitive neuroarchitecture of autobiographical memory, and the disruption of this memory function varies across different types of dementia.
WILEY INTERDISCIPLINARY REVIEWS-COGNITIVE SCIENCE
(2023)
Article
Behavioral Sciences
Stephanie Wong, Grace Wei, Masud Husain, John R. Hodges, Olivier Piguet, Muireann Irish, Fiona Kumfor
Summary: This study found a significant link between impaired social reward learning and emotional apathy in dementia, suggesting a shared neurobiological basis between the two. Understanding the neurocognitive mechanisms of reward processing can help improve the identification and treatment of emotional apathy in dementia.
COGNITIVE AFFECTIVE & BEHAVIORAL NEUROSCIENCE
(2023)
Article
Neuroimaging
Nga Yan Tse, Martina Bocchetta, Emily G. Todd, Emma M. Devenney, Sicong Tu, Jashelle Caga, John R. Hodges, Glenda M. Halliday, Muireann Irish, Matthew C. Kiernan, Olivier Piguet, Jonathan D. Rohrer, Rebekah M. Ahmed
Summary: Through studying a large sample of 211 participants, it was found that different subregions of the hypothalamus are correlated with cognitive and behavioral impairments. Patients with ALS, mixed ALS-FTD, and bvFTD all showed hypothalamic involvement. Cognitive and behavioral symptoms were correlated with bilateral involvement of the anterior inferior, anterior superior, and posterior hypothalamic subregions. The anterior superior and superior tuberal subregions displayed the greatest volume loss in bvFTD and ALS-FTD, and ALS, respectively, and were associated with specific neuropeptide expression abnormalities.
NEUROIMAGE-CLINICAL
(2023)
Article
Behavioral Sciences
Jessica L. Hazelton, Sol Fittipaldi, Matias Fraile-Vazquez, Marion Sourty, Agustina Legaz, Anna L. Hudson, Indira Garcia Cordero, Paula C. Salamone, Adrian Yoris, Agustin Ibanez, Olivier Piguet, Fiona Kumfor
Summary: This study investigates the disease-specific mechanisms underlying emotion recognition difficulties in bvFTD, AD, and PD patients. The findings suggest that interoceptive accuracy and cognitive abilities may contribute to emotion recognition impairments. In particular, bvFTD patients show worse interoceptive accuracy, while AD and PD patients show worse cognition.
Article
Behavioral Sciences
V. Lenglin, S. Wong, C. O'Callaghan, S. Erzinclioglu, M. Hornberger, T. Lebouvier, O. Piguet, S. Bourgeois-Gironde, M. Bertoux
Summary: Recent evidence from psycho-economics shows that the zero-price effect (ZPE) leads to an increase in subjective utility when the price of an item decreases to zero. This effect is attributed to an affective heuristic where the free status of an item biases choice towards that item. The study explored the role of the ventromedial prefrontal cortex (vmPFC) in the ZPE and found that patients with vmPFC lesion or behavioural-variant frontotemporal dementia showed reduced zero-related changes of preference for gift-cards but not for food items, suggesting altered affective evaluations in these patients.
Article
Psychology, Clinical
Kimberly Gressie, Fiona Kumfor, Her Teng, David Foxe, Emma Devenney, Rebekah M. Ahmed, Olivier Piguet
Summary: The study aims to compare the patterns of errors in facial emotion recognition in frontotemporal dementia (FTD) subtypes with Alzheimer's disease (AD) and healthy controls. The results show that all clinical groups perform worse than controls, with bvFTD and SD-right being particularly impaired. The patterns of errors are similar across all clinical groups.
INTERNATIONAL PSYCHOGERIATRICS
(2023)
Article
Clinical Neurology
Anna Semenkova, Olivier Piguet, Andreas Johnen, Matthias L. Schroeter, Jannis Godulla, Christoph Linnemann, Markus Muhlhauser, Thomas Sauer, Markus Baumgartner, Sarah Anderl-Straub, Markus Otto, Ansgar Felbecker, Reto W. Kressig, Manfred Berres, Marc Sollberger
Summary: Based on the revised diagnostic criteria, the Behavioural Dysfunction Questionnaire (BDQ) was developed to discriminate between behavioural variant frontotemporal dementia (bvFTD) and non-bvFTD patients. BDQ-scores without any time criterion were found highly discriminatory between early-stage bvFTD and non-bvFTD groups, suggesting its potential for improving early diagnosis of bvFTD.
JOURNAL OF NEUROLOGY
(2023)
Article
Clinical Neurology
A. Skeggs, G. Wei, R. Landin-Romero, J. R. Hodges, O. Piguet, Fiona Kumfor
Summary: This study aims to characterise the clinical profiles of patients with behavioural-variant frontotemporal dementia (bvFTD) from culturally diverse backgrounds. The results showed differences in clinical features, cognitive test performance, and cognitive reserve among patients from different cultural and linguistic backgrounds. The study also found that higher cognitive reserve is associated with lower neural integrity in frontal-temporal regions.
JOURNAL OF NEUROLOGY
(2023)
Article
Multidisciplinary Sciences
Hiu Chuen Lok, Jared S. Katzeff, John R. Hodges, Olivier Piguet, YuHong Fu, Glenda M. Halliday, Woojin Scott Kim
Summary: Neuroinflammation is a key feature of frontotemporal dementia (FTD), characterized by the degeneration of the frontal and temporal lobes. This study examined 48 cytokines in FTD serum and brain tissues to identify common dysregulation pathways. The results suggest the potential importance of the NLRP3 inflammasome in FTD.
SCIENTIFIC REPORTS
(2023)
Editorial Material
Clinical Neurology
Kristina Horne, Muireann Irish
BRAIN COMMUNICATIONS
(2023)
Article
Psychology, Clinical
Alice Powell, Ben C. P. Lam, David Foxe, Jacqueline C. T. Close, Perminder S. Sachdev, Henry Brodaty
Summary: This study aims to investigate the frequency of exceptional cognition in Australian older adults using different definitions and to explore its relationship with function, brain imaging markers, and incident dementia. The frequency of super-aging varied depending on the definition used and was associated with better functional performance and lower rates of incident dementia. The study highlights the importance of consistency in defining super-aging for better characterization of this minority group.
INTERNATIONAL PSYCHOGERIATRICS
(2023)
Article
Clinical Neurology
Kristina Horne, Rebekah M. Ahmed, Olivier Piguet, Muireann Irish
Summary: This study demonstrates the link between motivational changes and behavioral rigidity in frontotemporal dementia (FTD). The researchers found that FTD patients with severe behavioral rigidity also had a lack of motivation and decreased hedonic tone. By identifying candidate mechanisms of behavioral rigidity, these findings can inform targeted interventions to manage inflexible patterns of thought and behavior in daily life.
EUROPEAN JOURNAL OF NEUROLOGY
(2023)
Article
Behavioral Sciences
Andrea Gajardo-Vidal, Maxime Montembeault, Diego L. Lorca-Puls, Abigail E. Licata, Rian Bogley, Sabrina Erlhoff, Buddhika Ratnasiri, Zoe Ezzes, Giovanni Battistella, Elena Tsoy, Christa Watson Pereira, Jessica Deleon, Boon Lead Tee, Maya L. Henry, Zachary A. Miller, Katherine P. Rankin, Maria Luisa Mandelli, Katherine L. Possin, Maria Luisa Gorno-Tempini
Summary: This study investigates the potential differences in processing speed and neural correlates among the three variants of Primary Progressive Aphasia (PPA). The findings reveal that non-verbal cognitive abilities, such as processing speed, are significantly impacted in nfvPPA and lvPPA patients compared to healthy controls and svPPA patients. Neuroimaging results confirm the importance of fronto-parietal regions associated with processing speed and executive control.
Article
Behavioral Sciences
Holger Wiese, Tsvetomila Popova, Maya Schipper, Deni Zakriev, Mike Burton, Andrew W. Young
Summary: Previous experiments have shown that brief exposure to unfamiliar individuals leads to the formation of new facial representations, which undergo changes and consolidation within the first day after learning.
Article
Behavioral Sciences
Astrid Prochnow, Xianzhen Zhou, Foroogh Ghorbani, Paul Wendiggensen, Veit Roessner, Bernhard Hommel, Christian Beste
Summary: Individuals organize events in their environment by partitioning them into discrete units. This study reveals that the neural activity in the brain plays a critical role in this process, reflecting the key elements of event segmentation.
Article
Behavioral Sciences
Zhenzhen Huo, Zhiyi Chen, Rong Zhang, Junye Xu, Tingyong Feng
Summary: Procrastination has adverse effects on personal growth and social development. Reward sensitivity is positively correlated with procrastination. This study used VBM and RSFC analyses to investigate the neural substrates underlying the association between reward sensitivity and procrastination. The results showed that the functional connectivity of the right parahippocampal gyrus-precuneus mediated the relationship between reward sensitivity and procrastination.
Article
Behavioral Sciences
Stefano Lasaponara, Gabriele Scozia, Silvana Lozito, Mario Pinto, David Conversi, Marco Costanzi, Tim Vriens, Massimo Silvetti, Fabrizio Doricchi
Summary: Cholinergic (Ach), Noradrenergic (NE), and Dopaminergic (DA) pathways are crucial in regulating spatial attention and determining inter-individual differences in temperamental traits. This study found that temperamental traits predict individual differences in the ability to orient spatial attention based on the probabilistic association between cues and targets. These findings highlight the importance of considering temperamental and personality traits in social and professional environments where attention control is essential.
Article
Behavioral Sciences
Darren J. Yeo, Courtney Pollack, Benjamin N. Conrad, Gavin R. Price
Summary: The processing of numerals as visual objects is supported by an Inferior Temporal Numeral Area (ITNA) in the bilateral inferior temporal gyri (ITG). Extant findings suggest some degree of hemispheric asymmetry in how the bilateral ITNAs process numerals. The study found that digit sensitivity did not differ between ITNAs, and digit sensitivity in both left and right ITNAs was associated with calculation skills. The study also revealed a right lateralization in engagement in alphanumeric categorization, and that the right ITNA showed greater discriminability between digits and letters.
Review
Behavioral Sciences
Beste Gulsuna, Abuzer Gungor, Alp O. Borcer, Ugur Ture
Summary: The fiber dissection technique has been used to study the internal structures of the brain, with less focus on white matter. The sagittal stratum, a white matter structure, has not received enough attention and has been a subject of controversy. Recent studies suggest potential functions of the sagittal stratum, emphasizing the importance of understanding this structure accurately.
Article
Behavioral Sciences
Nora Geiser, Brigitte Charlotte Kaufmann, Samuel Elia Johannes Knobel, Dario Cazzoli, Tobias Nef, Thomas Nyffeler
Summary: This study compared the effects of auditory and visual motion stimulation on spatial neglect and found that both interventions were equally effective in improving neglect. Multimodal motion stimulation also improved neglect, but did not show greater improvement than unimodal auditory or visual motion stimulation alone.
Article
Behavioral Sciences
Anna E. Hughes, Anna Nowakowska, Alasdair D. F. Clarke
Summary: This study examines the relationship between search slopes and search efficiency in visual search tasks, introduces the Target Contrast Signal (TCS) Theory, and extends it to a Bayesian multi-level framework. The findings demonstrate that TCS can predict data well, but distinguishing between contrast combination models proves to be difficult.