Article
Clinical Neurology
Lina Zapata-Restrepo, Juan Rivas, Carlos Miranda, Bruce L. Miller, Agustin Ibanez, Isabel E. Allen, Katherine Possin
Summary: This study retrospectively reviewed medical records of 28 patients diagnosed with probable bvFTD in a public psychiatric hospital in Cali, Colombia. The majority of patients were initially misdiagnosed with a primary psychiatric condition, with a gender difference in psychiatric diagnosis but not in neuropsychiatric symptoms. These results emphasize the importance of improving bvFTD diagnosis in under-represented populations.
FRONTIERS IN NEUROLOGY
(2021)
Article
Geriatrics & Gerontology
Su Hong Kim, Yae Ji Kim, Byung Hwa Lee, Peter Lee, Ji Hyung Park, Sang Won Seo, Yong Jeong
Summary: The study investigated the behavioral reserve and associated neural substrates in participants with behavioral variant frontotemporal dementia. Analysis of neuroimaging abnormalities and behavioral assessment results revealed differences in disease progression among participants with different levels of behavioral reserve.
FRONTIERS IN AGING NEUROSCIENCE
(2022)
Review
Behavioral Sciences
Luiz Kobuti Ferreira, Olof Lindberg, Alexander F. Santillo, Lars-Olof Wahlund
Summary: Functional connectivity (FC) in behavioral variant frontotemporal dementia (bvFTD) has been summarized in this review. The most consistent finding is decreased FC within the salience network (SN), but FC changes extend beyond the SN and affect the interplay between networks. FC is associated with symptoms, functional impairment, and cognition, and the functional architecture resembles patterns of neuropathological spread.
BRAIN AND BEHAVIOR
(2022)
Article
Medicine, General & Internal
Ignacio Illan-Gala, Neus Falgas, Adit Friedberg, Sheila Castro-Suarez, Ophir Keret, Nicole Rogers, Didem Oz, Salvatore Nigro, Andrea Quattrone, Aldo Quattrone, Amy Wolf, Kyan Younes, Miguel Santos-Santos, Sergi Borrego-Ecija, Yann Cobigo, Oriol Dols-Icardo, Albert Llado, Raquel Sanchez-Valle, Jordi Clarimon, Rafael Blesa, Daniel Alcolea, Juan Fortea, Alberto Lleo, Lea T. Grinberg, Salvatore Spina, Joel H. Kramer, Gil D. Rabinovici, Adam Boxer, Maria Luisa Gorno Tempini, Bruce L. Miller, William W. Seeley, Howard J. Rosen, David C. Perry
Summary: This study demonstrates that in bvFTD, VAS scores increase the diagnostic certainty of underlying FTLD, and the MRPI shows potential for detecting participants with underlying 4R tauopathies.
Article
Psychology, Clinical
Matthew J. Y. Kang, Sarah Farrand, Andrew Evans, Wei-Hsuan Chiu, Dhamidhu Eratne, Wendy Kelso, Mark Walterfang, Dennis Velakoulis, Samantha M. Loi
Summary: A study found no significant difference in caregiver burden between younger-onset Alzheimer's disease and behavioral variant frontotemporal dementia. This could be due to similarities in patient behavior, motivation, and self-care between the two subtypes. Clinicians should screen for caregiver burden and associated factors, such as behavioral symptoms, in younger-onset dementia syndromes.
INTERNATIONAL PSYCHOGERIATRICS
(2023)
Article
Neurosciences
Chiara Cerami, Elena Perdixi, Claudia Meli, Alessandra Marcone, Michele Zamboni, Sandro Iannaccone, Alessandra Dodich
Summary: This study developed a short version of the Frontal Behavioral Inventory (mini-FBI) to identify early behavioral changes in behavioral variant of frontotemporal dementia (bvFTD) and aid in the differential diagnosis with Alzheimer's disease (AD). The mini-FBI consisted of 12 items and showed significant convergent validity with the Neuropsychiatric Inventory scores. Cluster analysis identified four clusters, with bvFTD and some AD patients showing similar behavioral patterns.
JOURNAL OF ALZHEIMERS DISEASE
(2022)
Article
Clinical Neurology
Ignacio Illan-Gala, Kaitlin B. Casaletto, Sergi Borrego-Ecija, Eider M. Arenaza-Urquijo, Amy Wolf, Yann Cobigo, Sheng Yang M. Goh, Adam M. Staffaroni, Daniel Alcolea, Juan Fortea, Rafael Blesa, Jordi Clarimon, Maria Florencia Iulita, Anna Brugulat-Serrat, Albert Llado, Lea T. Grinberg, Katherine Possin, Katherine P. Rankin, Joel H. Kramer, Gil D. Rabinovici, Adam Boxer, William W. Seeley, Virginia E. Sturm, Maria Luisa Gorno-Tempini, Bruce L. Miller, Raquel Sanchez-Valle, David C. Perry, Alberto Lleo, Howard J. Rosen
Summary: The study revealed that women with bvFTD exhibited greater atrophy burden in the frontotemporal regions compared to men, despite having similar clinical characteristics. Additionally, women showed better executive function performance and experienced fewer changes in apathy, sleep, and appetite compared to men at a similar level of atrophy.
ALZHEIMERS & DEMENTIA
(2021)
Article
Clinical Neurology
Sheila Castro-Suarez, Erik Guevara-Silva, Cesar Caparo-Zamalloa, Victor Osorio-Marcatinco, Maria Meza-Vega, Bruce Miller, Mario Cornejo-Olivas
Summary: Neuropsychiatry medical specialists in Peru receive limited training in FTD, lacking clinical knowledge and attitudes towards bvFTD. They have limited understanding of the diagnostic criteria and assessment tools for bvFTD, and there are misconceptions regarding pharmacological and non-pharmacological management. They also lack education and support for caregivers.
FRONTIERS IN NEUROLOGY
(2022)
Review
Neurosciences
Roxane Dilcher, Charles B. Malpas, Terence J. O'Brien, Lucy Vivash
Summary: Behavioral variant frontotemporal dementia (bvFTD) is a disease within the frontotemporal lobar degeneration (FTLD) spectrum, characterized by frontal dysfunction and prominent socioemotional impairments. Social cognition, such as emotion processing and theory of mind, significantly affects daily behavior in bvFTD. Abnormal protein accumulation of tau or TDP-43 is the main cause of neurodegeneration and cognitive decline. Differential diagnosis is challenging due to heterogeneous pathology and clinicopathological overlap with other FTLD syndromes. Understanding the association between social behavior and cognition, and underlying pathology is crucial for biomarker validation, clinical trials, and practice.
JOURNAL OF ALZHEIMERS DISEASE
(2023)
Article
Neurosciences
Mariano N. Diaz-Rivera, Agustina Birba, Sol Fittipaldi, Debora Mola, Yurena Morera, Manuel de Vega, Sebastian Moguilner, Patricia Lillo, Andrea Slachevsky, Cecilia Gonzalez Campo, Agustin Ibanez, Adolfo M. Garcia
Summary: This study investigated the neural correlates of linguistic negation in healthy controls, behavioral variant frontotemporal dementia (bvFTD) patients, and Alzheimer's disease (AD) patients. The results showed that control participants displayed a delta suppression in frontocentral regions during negative sentences in a No-go condition, which is a marker of inhibition. In bvFTD patients, this suppression was selectively abolished and correlated with the volume and functional connectivity of regions involved in inhibition. This study provides insights into the neural mechanisms of negation and potential neurocognitive markers of bvFTD.
Article
Geriatrics & Gerontology
Alfonso Delgado-Alvarez, Maria Nieves Cabrera-Martin, Maria Valles-Salgado, Cristina Delgado-Alonso, Maria Jose Gil, Maria Diez-Cirarda, Jorge Matias-Guiu, Jordi A. Matias-Guiu
Summary: This study investigated the visuospatial task performance and neural mechanisms in patients with bvFTD and AD. The results showed that patients with AD performed the worst in visuospatial tasks in mild dementia, while patients with bvFTD showed higher correlations between attention and executive functioning and visuospatial performance. The visuospatial deficits in patients with bvFTD were associated with bilateral frontal regions.
FRONTIERS IN AGING NEUROSCIENCE
(2022)
Article
Clinical Neurology
Nilton Custodio, Rosa Montesinos, Lizardo Cruzado, Eder Herrera-Perez, Virgilio E. Failoc-Rojas, Maritza Pintado-Caipa, Wendy G. Seminario, Jose Cuenca, Carlos Gamboa, Monica M. Diaz
Summary: This study demonstrated that integrating brief socio-cognitive tests and behavioral assessments can aid in distinguishing patients with bvFTD from those with AD, especially in individuals with low educational levels. Social and behavioral cognition tasks showed high sensitivity and moderate specificity in discriminating between bvFTD and AD.
FRONTIERS IN NEUROLOGY
(2021)
Article
Neurosciences
Meghan Healey, Erica Howard, Molly Ungrady, Christopher A. Olm, Naomi Nevler, David J. Irwin, Murray Grossman
Summary: Indirect speech acts, such as responding to a question about the time with a statement like "I forgot to wear my watch today," are common in daily conversations but have been understudied in current neurobiological models of language. This study found that patients with behavioral variant frontotemporal dementia (bvFTD) showed impairments in understanding indirect speech acts compared to brain-damaged controls with amnestic mild cognitive impairment (MCI), which may be due to their social and executive limitations. MRI imaging revealed cortical thinning in language-associated regions as well as fronto-parietal regions in bvFTD patients, highlighting a language-mediated social disorder in this population.
FRONTIERS IN HUMAN NEUROSCIENCE
(2021)
Article
Clinical Neurology
Adit Friedberg, Eliana Marisa Ramos, Zhongan Yang, Luke W. Bonham, Jennifer S. Yokoyama, Peter A. Ljubenkov, Kyan Younes, Daniel H. Geschwind, Bruce L. Miller
Summary: CSF1R-related leukoencephalopathy is a neurodegenerative disease caused by mutations in CSF1R. This study describes the prodromal and early symptoms of a patient with this disease and identifies a novel pathogenic variant. The findings contribute to the understanding of early manifestations of CSF1R-related leukoencephalopathy and may facilitate early detection and treatment.
FRONTIERS IN NEUROLOGY
(2022)
Article
Clinical Neurology
Gustavo Campos de Franca, Henrique Carneiro de Barros Barreto, Thiago Paranhos, Julio Cesar Nunes, Ricardo de Oliveira-Souza
Summary: This case report describes a 95-year-old woman with degenerative dementia who experienced a radical change in personality and eventually developed catatonic stupor. Magnetic resonance imaging revealed asymmetric frontotemporal and parietal atrophy. The case highlights the link between frontotemporal injury and behavioral disinhibition, as well as the involvement of the parietal cortex in the development of catatonic stupor.
FRONTIERS IN NEUROLOGY
(2022)
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)
Review
Clinical Neurology
Bonnie Yin Ka Lam, Yuan Cai, Rufus Akinyemi, Geert Jan Biessels, Hilde van den Brink, Christopher Chen, Chin Wai Cheung, King Ngai Chow, Henry Kwun Hang Chung, Marco Duering, Siu Ting Fu, Deborah Gustafson, Saima Hilal, Vincent Ming Ho Hui, Rajesh Kalaria, Sang Yun Kim, Maggie Li Man Lam, Frank Erik de Leeuw, Ami Sin Man Li, Hugh Stephen Markus, Anna Marseglia, Huijing Zheng, John O'Brien, Leonardo Pantoni, Perminder Singh Sachdev, Eric E. Smith, Joanna Wardlaw, Vincent Chung Tong Mok
Summary: This study systematically reviewed the prevalence of cerebral small vessel disease (cSVD) in low- and middle-income countries (LMICs). The results demonstrated a high prevalence of cSVD in LMICs. There is a need for more data from different regions to support the findings, as there is currently bias in the available studies.
INTERNATIONAL JOURNAL OF STROKE
(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)
Correction
Clinical Neurology
YuHong Fu, Liche Zhou, Hongyun Li, Jen-Hsiang T. Hsiao, Binyin Li, Onur Tanglay, Andrew D. Auwyang, Elinor Wang, Jieyao Feng, Woojin S. Kim, Jun Liu, Glenda M. Halliday
ACTA NEUROPATHOLOGICA
(2023)
Letter
Clinical Neurology
Srestha Mazumder, Heather McCann, Susan D'Silva, Sarah Furlong, Claire E. Shepherd, Jillian J. Kril, Glenda M. Halliday, Dominic B. Rowe, Matthew C. Kiernan, Rachel H. Tan
Article
Clinical Neurology
Yannie Soo, Annaelle Zietz, Brian Yiu, Vincent C. T. Mok, Alexandros A. Polymeris, David Seiffge, Gareth Ambler, Duncan Wilson, Thomas Wai Hong Leung, Suk Fung Tsang, Winnie Chu, Jill Abrigo, Cyrus Cheng, Keon-Joo Lee, Jae-Sung Lim, Masayuki Shiozawa, Masatoshi Koga, Hugues Chabriat, Michael Hennerici, Yuen Kwun Wong, Henry Mak, Roger Collet, Shigeru Inamura, Kazuhisa Yoshifuji, Ethem Murat Arsava, Solveig Horstmann, Jan Purrucker, Bonnie Y. K. Lam, Adrian Wong, Young Dae Kim, Tae-Jin Song, Robin Lemmens, Sebastian Eppinger, Thomas Gattringer, Ender Uysal, Derya Selcuk Demirelli, Natan M. Bornstein, Einor Ben Assayag, Hen Hallevi, Jeremy Molad, Masashi Nishihara, Jun Tanaka, Shelagh B. Coutts, L. Jaap Kappelle, Rustam Al-Shahi Salman, Rolf Jager, Gregory Y. H. Lip, Martina B. Goeldlin, Leonidas D. Panos, Jean-Louis Mas, Laurence Legrand, Chris Karayiannis, Thanh Phan, Maximilian Bellut, Francesca Chappell, Stephen Makin, Derek Hayden, David Williams, Dianne H. K. van Dam-Nolen, Paul J. Nederkoorn, Carmen Barbato, Simone Browning, Kim Wiegertjes, Anil Man Tuladhar, Anne-Marie Mendyk, Sebastian Kohler, Robert van Oostenburgge, Ying Zhou, Chao Xu, Saima Hilal, Bibek Gyanwali, Christopher Chen, Min Lou, Julie Staals, Regis Bordet, Nagaendran Kandiah, Frank-Erik de Leeuw, Robert Simister, Jeroen Hendrikse, Joanna Wardlaw, Peter Kelly, Felix Fluri, Velandai Srikanth, David Calvet, Simon Jung, Vincent I. H. Kwa, Eric E. Smith, Hideo Hara, Yusuke Yakushiji, Dilek Necioglu Orken, Franz Fazekas, Vincent Thijs, Ji-Hoe Heo, Roland Veltkamp, Hakan Ay, Toshio Imaizumi, Kui Kai Lau, Eric Jouvent, Kazunori Toyoda, Sohei Yoshimura, Hee-Joon Bae, Joan Marti-Fabregas, Luis Prats-Sanchez, Philippe Lyrer, Jonathan Best, David Werring, Stefan T. Engelter, Nils Peters
Summary: This study aimed to evaluate the risks of intracranial hemorrhage and ischemic stroke associated with microbleeds in atrial fibrillation patients. The results showed that the presence of microbleeds increased the relative risk of hemorrhage and stroke. In patients taking a combination of anticoagulant and antiplatelet therapy, the risk of intracranial hemorrhage was higher than that of ischemic stroke.
ANNALS OF NEUROLOGY
(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
Clinical Neurology
Yue Yang, Dominic Rowe, Heather McCann, Claire E. Shepherd, Jillian J. Kril, Matthew C. Kiernan, Glenda M. Halliday, Rachel H. Tan
Summary: This study compared the pathology of ALS patients who received CuATSM and riluzole with those who only received riluzole, and found no significant difference in neuron density or TDP-43 burden. However, CuATSM treatment led to the presence of p62-immunoreactive astrocytes in the motor cortex and reduced Iba1 density in the spinal cord. There was no significant difference in astrocytic activity and SOD1 immunoreactivity with CuATSM treatment.
NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY
(2023)
Article
Multidisciplinary Sciences
Priscilla Youssef, Laura Hughes, Woojin S. Kim, Glenda M. Halliday, Simon J. G. Lewis, Antony Cooper, Nicolas Dzamko
Summary: Objective biomarkers for Parkinson's Disease (PD) could aid early and specific diagnosis, effective monitoring of disease progression, and improved design and interpretation of clinical trials. In this study, the potential of the SIMOA neurology 4-plex-A marker panel, along with plasma alpha-synuclein, as biomarkers for PD diagnosis and prognosis was assessed. The levels of NFL and GFAP in plasma positively correlated with alpha-synuclein levels, while as disease state biomarkers, NFL and GFAP showed correlation with motor severity and stage.
SCIENTIFIC REPORTS
(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)
Article
Clinical Neurology
Monica Pinkerton, Guinevere Lourenco, Maria Torres Pacheco, Glenda M. Halliday, Matthew C. Kiernan, Rachel H. Tan
Summary: This study aimed to assess the pathology of p62 in sporadic ALS patients and determine its association with disease pathology. The results identified more cytoplasmic p62 aggregates in patients with a shorter disease duration. The findings support further research on p62 as a potential prognostic biomarker in ALS.
JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY
(2023)
Letter
Clinical Neurology
Claire E. Shepherd, Simon J. G. Lewis, Stephen Tisch, Heather McCann, Ben Jonker, Glenda M. Halliday
JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY
(2023)
Article
Clinical Neurology
Andrew J. Affleck, Perminder S. Sachdev, Glenda M. Halliday
Summary: This study examines the impact of antihypertensive medication use on the severity of neuropathological cerebrovascular disease in older individuals. The results suggest that antihypertensive medication use is associated with less severe white matter small vessel disease. However, there is no significant relationship between medication use and infarction, lacunes, or cerebral amyloid angiopathy. Antihypertensive medication use reduces white matter rarefaction and Aβ propagation through the brain, specifically in individuals with moderate to severe white matter small vessel disease.
NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY
(2023)
Article
Cell Biology
Rui Qi, Esther Sammler, Claudia P. Gonzalez-Hunt, Ivana Barraza, Nicholas Pena, Jeremy P. Rouanet, Yahaira Naaldijk, Steven Goodson, Marie Fuzzati, Fabio Blandini, Kirk I. Erickson, Andrea M. Weinstein, Michael W. Lutz, John B. Kwok, Glenda M. Halliday, Nicolas Dzamko, Shalini Padmanabhan, Roy N. Alcalay, Cheryl Waters, Penelope Hogarth, Tanya Simuni, Danielle Smith, Connie Marras, Francesca Tonelli, Dario R. Alessi, Andrew B. West, Sruti Shiva, Sabine Hilfiker, Laurie H. Sanders
Summary: Parkinson's disease (PD) is a common neurodegenerative movement disorder in which mitochondrial dysfunction plays a key role. This study developed a PCR-based assay called Mito DNADX to accurately quantify mitochondrial DNA (mtDNA) damage. The assay revealed increased mtDNA damage in peripheral blood cells from PD patients and those with a PD-associated mutation. In addition, it was found that LRRK2 kinase inhibitors could mitigate mtDNA damage in animal models and patient-derived cells. This study suggests that quantifying mtDNA damage may serve as a potential biomarker for PD and for assessing the effectiveness of LRRK2 kinase inhibitors.
SCIENCE TRANSLATIONAL MEDICINE
(2023)