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
Jessica Jiang, Jeremy C. S. Johnson, Mai-Carmen Requena-Komuro, Elia Benhamou, Harri Sivasathiaseelan, Anthipa Chokesuwattanaskul, Annabel Nelson, Ross Nortley, Rimona S. Weil, Anna Volkmer, Charles R. Marshall, Doris-Eva Bamiou, Jason D. Warren, Chris J. D. Hardy
Summary: Successful communication in daily life relies on accurate decoding of speech signals, which can be degraded in challenging listening conditions. This study examines the comprehension of acoustically degraded speech in patients with Alzheimer's disease and progressive aphasia, finding that they perform worse on a degraded speech comprehension task compared to healthy controls. The impaired task performance is associated with atrophy in a fronto-temporal cortical network known to be involved in degraded speech processing.
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
Jordi A. Matias-Guiu, Vanesa Pytel, Laura Hernandez-Lorenzo, Nikil Patel, Katie A. Peterson, Jorge Matias-Guiu, Peter Garrard, Fernando Cuetos
Summary: The study adapted and validated the Spanish version of MLSE for PPA diagnosis, showing excellent internal consistency and discriminative properties. MLSE performed well in distinguishing PPA patients from healthy controls and differentiating between clinical variants.
JOURNAL OF ALZHEIMERS DISEASE
(2021)
Article
Clinical Neurology
A. Mouton, A. Plonka, R. Fabre, T. M. Tran, P. Robert, J. Macoir, V. Manera, A. Gros
Summary: This study examines the demographics and evolution of individuals with primary progressive aphasia (PPA) compared to those with Alzheimer's disease (AD) over a 7-year period in a French cohort. The study finds that PPA patients are diagnosed at a younger age, have higher education levels, and receive non-pharmacological treatments more often than AD patients. The findings support the hypothesis that PPA is diagnosed later than AD and highlight the importance of appropriate public health service policies.
ALZHEIMERS RESEARCH & THERAPY
(2022)
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
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)
Article
Behavioral Sciences
Melissa D. Stockbridge, Donna C. Tippett, Bonnie L. Breining, Emilia Vitti, Argye E. Hillis
Summary: This study retrospectively analyzed 435 evaluations of individuals with primary progressive aphasia, finding that a battery of cognitive and linguistic assessments had varying sensitivity and specificity in distinguishing different variants. Naming assessments were identified as the strongest basis for distinguishing all variants.
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
Clinical Neurology
Hulya Ulugut, Simone Stek, Lianne E. E. Wagemans, Roos J. Jutten, Maria Antoinette Keulen, Femke H. Bouwman, Niels D. Prins, Afina W. Lemstra, Welmoed Krudop, Charlotte E. Teunissen, Bart N. M. van Berckel, Rik Ossenkoppele, Frederik Barkhof, Wiesje M. van der Flier, Philip Scheltens, Yolande A. L. Pijnenburg
Summary: Primary progressive aphasia (PPA) is divided into three prototypical subtypes - semantic, non-fluent, and logopenic - each subtype progresses differently over time and has a relatively long disease duration.
JOURNAL OF NEUROLOGY
(2022)
Article
Clinical Neurology
Talita Gallas dos Reis, Thais Helena Machado, Paulo Caramelli, Francisco Scornavacca, Liana Lisboa Fernandez, Barbara Costa Beber
Summary: The study compared the initial symptoms of PPA and AD patients, finding that anomia, paraphasias, and motor speech difficulties were key symptoms differentiating PPA from AD, while memory problems were exclusive to AD. Among the PPA variants, anomia was associated with the semantic variant, while motor speech difficulties were associated with the non-fluent variant.
FRONTIERS IN NEUROLOGY
(2021)
Article
Clinical Neurology
M. -Marsel Mesulam, Christina A. Coventry, Eileen H. Bigio, Jaiashre Sridhar, Nathan Gill, Angela J. Fought, Hui Zhang, Cynthia K. Thompson, Changiz Geula, Tamar Gefen, Margaret Flanagan, Qinwen Mao, Sandra Weintraub, Emily J. Rogalski
Summary: Primary progressive aphasia is a neurodegenerative disease that selectively impairs language function. Autopsies and longitudinal studies have shown that primary progressive aphasia has various neuropathological changes, with Alzheimer's disease being the most common. Different variants of primary progressive aphasia have distinct neuropathological correlates, and word comprehension impairments are strong predictors of underlying neuropathology. Different types of primary progressive aphasia have different patterns of cortical atrophy, but all show severe damage to the left hemisphere language network. This study is important for understanding the neuropathological and clinical differences in primary progressive aphasia.
Article
Neurosciences
Nuria Montagut, Sergi Borrego-Ecija, Magdalena Castellvi, Immaculada Rico, Ramon Rene, Mircea Balasa, Albert Llado, Raquel Sanchez-Valle
Summary: The study found that errorless learning speech therapy can improve naming ability in patients with svPPA, but the improvement diminishes progressively after therapy ends, with no improvement found in comprehension tasks.
JOURNAL OF ALZHEIMERS DISEASE
(2021)
Article
Clinical Neurology
Ignacio Illan-Gala, Victor Montal, Sergi Borrego-Ecija, Maria Luisa Mandelli, Neus Falgas, Ariane E. Welch, Jordi Pegueroles, Miguel Santos-Santos, Alexandre Bejanin, Daniel Alcolea, Oriol Dols-Icardo, Olivia Belbin, M. Belen Sanchez-Saudinos, Nuria Bargallo, Sofia Gonzalez-Ortiz, Albert Llado, Rafael Blesa, Bradford C. Dickerson, Howard J. Rosen, Bruce L. Miller, Alberto Lleo, Maria Luisa Gorno-Tempini, Raquel Sanchez-Valle, Juan Fortea
Summary: Cortical mean diffusivity shows promise as a sensitive biomarker for the study of the neurodegeneration-related microstructural changes in primary progressive aphasia (PPA). Increases in cortical mean diffusivity are correlated with cortical thinning and disease severity in PPA patients.
ALZHEIMERS RESEARCH & THERAPY
(2022)
Article
Immunology
Aitana Sogorb-Esteve, Imogen J. Swift, Ione O. C. Woollacott, Jason D. Warren, Henrik Zetterberg, Jonathan D. Rohrer
Summary: This study investigated the role of chemokines in primary progressive aphasias, revealing differential alterations in cerebrospinal fluid (CSF) and plasma across the PPA variants. The results suggest a potential role for neuroinflammation in these poorly understood sporadic disorders, indicating a potential therapeutic target for the future.
JOURNAL OF NEUROINFLAMMATION
(2021)
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
Geriatrics & Gerontology
Sarah N. Kraeutner, Cristina Rubino, Jennifer K. Ferris, Shie Rinat, Lauren Penko, Larissa Chiu, Brian Greeley, Christina B. Jones, Beverley C. Larssen, Lara A. Boyd
Summary: This study examined the age-related changes in brain function and baseline brain structure that support motor skill acquisition. The findings showed that older adults experienced decreases in functional connectivity during motor skill acquisition, while younger adults experienced increases. Additionally, regardless of age group, lower baseline microstructure in a frontoparietal tract was associated with slower motor skill acquisition.
NEUROBIOLOGY OF AGING
(2024)
Article
Geriatrics & Gerontology
Karen Nuytemans, Farid Rajabli, Melissa Jean-Francois, Jiji Thulaseedhara Kurup, Larry D. Adams, Takiyah D. Starks, Patrice L. Whitehead, Brian W. Kunkle, Allison Caban-Holt, Jonathan L. Haines, Michael L. Cuccaro, Jeffery M. Vance, Goldie S. Byrd, Gary W. Beecham, Christiane Reitz, Margaret A. Pericak-Vance
Summary: This study conducted genetic research on African American AD families and identified a significant linkage signal associated with AD, highlighting the importance of diverse population-level genetic data in understanding the genetic determinants of AD.
NEUROBIOLOGY OF AGING
(2024)
Article
Geriatrics & Gerontology
Kazuya Suwabe, Ryuta Kuwamizu, Kazuki Hyodo, Toru Yoshikawa, Takeshi Otsuki, Asako Zempo-Miyaki, Michael A. Yassa, Hideaki Soya
Summary: Physical exercise has a positive impact on hippocampal memory decline with aging. Recent studies have shown that even light exercise can improve memory and this improvement is mediated by the ascending arousal system. This study aimed to investigate the effects of light-intensity exercise on hippocampal memory function in healthy older adults and found that pupil dilation during exercise played a role in the memory improvement.
NEUROBIOLOGY OF AGING
(2024)
Article
Geriatrics & Gerontology
Ajay Sood, Ana Werneck Capuano, Robert Smith Wilson, Lisa Laverne Barnes, Alifiya Kapasi, David Alan Bennett, Zoe Arvanitakis
Summary: The objective of this study was to explore the impact of metformin on cognition and brain pathology. The results showed that metformin users had slower decline in global cognition, episodic memory, and semantic memory compared to non-users. However, the relationship between metformin use and certain brain pathology remains uncertain.
NEUROBIOLOGY OF AGING
(2024)
Article
Geriatrics & Gerontology
Brian N. Lee, Junwen Wang, Molly A. Hall, Dokyoon Kim, Shana D. Stites, Li Shen
Summary: Alzheimer's disease (AD) is a neurodegenerative disorder characterized by memory and functional impairments. This study analyzed participants from the Alzheimer's Disease Neuroimaging Initiative and found differential associations between cerebral spinal fluid (CSF)/neuroimaging biomarkers and cognitive/functional outcomes, as well as variations between sexes. These findings suggest that sex differences may play a role in the development of AD.
NEUROBIOLOGY OF AGING
(2024)
Article
Geriatrics & Gerontology
Madeline R. Hale, Rebecca Langhough, Lianlian Du, Bruce P. Hermann, Carol A. Van Hulle, Margherita Carboni, Gwendlyn Kollmorgenj, Kristin E. Basche, Davide Bruno, Leah Sanson-Miles, Erin M. Jonaitis, Nathaniel A. Chin, Ozioma C. Okonkwo, Barbara B. Bendlin, Cynthia M. Carlsson, Henrik Zetterberg, Kaj Blennow, Tobey J. Betthauser, Sterling C. Johnson, Kimberly D. Mueller
Summary: This study demonstrates a relationship between cerebrospinal fluid biomarkers and the ability to recall proper names in the preclinical phase of Alzheimer's disease.
NEUROBIOLOGY OF AGING
(2024)
Article
Geriatrics & Gerontology
Thomas T. Austin, Christian L. Thomas, Ben Warren
Summary: This study investigated the effects of age on the robustness and resilience of auditory system using the desert locust. The researchers found that gene expression changes were mainly influenced by age rather than noise exposure. Both young and aged locusts were able to recover their auditory nerve function within 48 hours of noise exposure, but the recovery of transduction current magnitude was impaired in aged locusts. Key genes responsible for robustness to noise exposure in young locusts and potential candidates for compensatory mechanisms in auditory neurons of aged locusts were identified.
NEUROBIOLOGY OF AGING
(2024)