Article
Clinical Neurology
Yuichi Riku, Yasushi Iwasaki, Shinsuke Ishigaki, Akio Akagi, Masato Hasegawa, Kenya Nishioka, Yuanzhe Li, Miho Riku, Takeshi Ikeuchi, Yusuke Fujioka, Hiroaki Miyahara, Jun Sone, Nobutaka Hattori, Mari Yoshida, Masahisa Katsuno, Gen Sobue
Summary: Mislocalization and cytoplasmic aggregation of TDP-43 in spinal cord motor neurons have been found in various neurological disorders, suggesting mechanistic links. Additionally, the severity of TDP-43 pathology in the spinal cord correlates with the severity of 4R-tau aggregates. These findings provide insights into the pathology and potential mechanisms underlying TDP-43-related diseases.
Article
Public, Environmental & Occupational Health
Diane M. A. Swallow, Carl E. Counsell
Summary: This study estimated the prevalence of progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) in Scotland, UK. The prevalence rates were similar to previous estimates, with little change over the past 20 years.
Article
Clinical Neurology
Sami Heikkinen, Antti Cajanus, Kasper Katisko, Paivi Hartikainen, Ritva Vanninen, Annakaisa Haapasalo, Johanna Kruger, Anne M. Remes, Eino Solje
Summary: EP symptoms are associated with neuroimaging changes in PSP, CBD, and bvFTD patients, with significant pathological changes in the brain stem and SCP region in EP+ patients.
JOURNAL OF NEUROLOGY
(2022)
Review
Clinical Neurology
Katie A. Peterson, Karalyn Patterson, James B. Rowe
Summary: PSP and CBS can present changes in speech and language alongside or before motor symptoms, making their differential diagnosis challenging in the early stages. Language impairment is often an early and persistent issue in CBS and PSP, highlighting the need for improved language screening and detailed language assessments. Improved language assessment may help in differential diagnosis and inform clinical management decisions.
JOURNAL OF NEUROLOGY
(2021)
Article
Clinical Neurology
Nils Briel, Viktoria C. Ruf, Katrin Pratsch, Sigrun Roeber, Jeannine Widmann, Janina Mielke, Mario M. Dorostkar, Otto Windl, Thomas Arzberger, Jochen Herms, Felix L. Struebing
Summary: This study used chromatin data from single nuclei to identify disease-related molecular changes associated with astrocytes in the brains of CBD and PSP patients, revealing insights into the regulatory mechanisms of disease development. The research findings expand our knowledge on risk gene involvement (such as MAPT, MAPK8, and NFE2L2) and molecular pathways leading to phenotypic changes in CBD and PSP.
ACTA NEUROPATHOLOGICA
(2022)
Letter
Clinical Neurology
Chelsey ShengQi Zhao, Lei Yan, Wenqing He, Lee Cyn Ang, Qi Zhang
Summary: Neuropathological diagnosis of progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) involves interpreting tau morphology through extensive brain sampling. Subcortical tau burden can effectively differentiate between PSP and CBD, with the tau pallido-claustral ratio showing promise as a diagnostic indicator for these conditions.
Article
Clinical Neurology
Eduardo de Pablo-Fernandez, Belen Gonzalez-Herrero, Debora Cerdan Santacruz, Martin N. Rossor, Jonathan M. Schott, Tammaryn Lashley, Janice L. Holton, Nick C. Fox, Tamas Revesz, Jason D. Warren, Zane Jaunmuktane, Jonathan D. Rohrer, Thomas T. Warner
Summary: This study provides a detailed description of movement disorders in FTLD patients, finding that parkinsonism and corticobasal syndrome are common syndromes associated with FTLD, with parkinsonism presenting more often in the late stages of the disease and corticobasal syndrome as an initial symptom. The study also suggests that there is a complex pathophysiology involving structures beyond the presynaptic striatonigral system in FTLD-related movement disorders.
MOVEMENT DISORDERS
(2021)
Review
Neurosciences
Nathalie Bendstrup, Anne-Mette Hejl, Lisette Salvesen
Summary: This systematic review evaluated the utility of neurofilament light in cerebrospinal fluid (CSF) for differentiating frontotemporal dementia (FTD) from progressive supranuclear palsy (PSP). The majority of studies included in this review found a higher level of neurofilament light in CSF in patients with FTD compared to patients with PSP; however, the results were inconsistent, indicating a need for further prospective studies.
JOURNAL OF ALZHEIMERS DISEASE
(2022)
Article
Medicine, General & Internal
Eun Joo Chung, Hwa Jin Cho, Wooyoung Jang, Dae Young Hur, Yeong Seok Kim, Kyung-Hwa Lee, Sang Jin Kim
Summary: We report a case of pathologically confirmed corticobasal degeneration (CBD) in a 68-year-old man with clinical suspicion of progressive supranuclear palsy (PSP). Despite overlapping clinical features, the presence of astrocytic plaques led to the final diagnosis of CBD.
JOURNAL OF KOREAN MEDICAL SCIENCE
(2022)
Article
Clinical Neurology
Diane M. A. Swallow, Carl E. Counsell
Summary: Misdiagnosis and delayed diagnosis are common in PSP and CBD. This study evaluated the diagnostic process from symptom onset to death and found that the duration and complexity of the diagnostic journey were greater in PSP/CBD compared to PD.
JOURNAL OF NEUROLOGY
(2023)
Article
Behavioral Sciences
Leonardo Cruz de Souza, Maxime Bertoux, Ratko Radakovic, Michael Hornberger, Luciano Inacio Mariano, Elisa de Paula Franca Resende, Francois Quesque, Henrique Cerqueira Guimaraes, Leandro Boson Gambogi, Vitor Tumas, Sarah Teixeira Camargos, Francisco Eduardo Costa Cardoso, Antonio Lucio Teixeira, Paulo Caramelli
Summary: This study used a simplified version of the Social and Emotional Assessment (Mini-SEA) to evaluate the social cognition abilities of patients with behavioral variant frontotemporal dementia (bvFTD) and progressive supranuclear palsy (PSP). The results revealed significant impairments in social cognition and brain changes in these two conditions compared to controls. Additionally, similar neural patterns were observed in PSP and bvFTD patients, suggesting shared clinical, cognitive, and neuroimaging features.
Article
Clinical Neurology
Shunsuke Koga, Xiaolai Zhou, Dennis W. Dickson
Summary: By analyzing tau pathology scores in different brain regions of a large number of PSP and CBD cases and establishing a decision tree classifier, it was found that the severity of tau lesions in certain brain regions is crucial for distinguishing between PSP and CBD.
NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY
(2021)
Article
Clinical Neurology
Nils Briel, Katrin Pratsch, Sigrun Roeber, Thomas Arzberger, Jochen Herms
Summary: Research suggests that pTau in astrocytes plays an important role in maintaining synaptic integrity in Progressive Supranuclear Palsy (PSP) and Corticobasal Degeneration (CBD), and may be associated with cognitive dysfunction in CBD.
Article
Clinical Neurology
Blas Couto, Susan Fox, Maria Carmela Tartaglia, Ekaterina Rogaeva, Jeffrey Antwi, Puja Bhakta, Gabor G. Kovacs, Anthony E. E. Lang
Summary: The study focuses on the development and initial experience of the Rossy PSP Centre in Canada, which aims to advance clinical and basic research in progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). The study collects extensive demographic and longitudinal clinical information using standardized forms, as well as biofluids for genetic analysis and neuroimaging research protocols. The preliminary results show a typical distribution of phenotypes, demographics, and response to symptomatic treatments in the cohort. Future steps include enrolling patients in earlier stages, developing biomarkers, and fast-tracking well-characterized patients into clinical trials.
CANADIAN JOURNAL OF NEUROLOGICAL SCIENCES
(2023)
Article
Clinical Neurology
Leonidas Chouliaras, Alan Thomas, Maura Malpetti, Paul Donaghy, Joseph Kane, Elijah Mak, George Savulich, Maria A. Prats-Sedano, Amanda J. Heslegrave, Henrik Zetterberg, Li Su, James Benedict Rowe, John Tiernan O'Brien
Summary: This study compared plasma biomarkers for neurodegenerative diseases in different patient groups and found that p-tau181 was elevated in MCI+AD patients with high accuracy in differentiating between different types of dementia. NfL was elevated in all dementia groups, while GFAP was elevated in MCI+AD and LBD.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
(2022)
Article
Clinical Neurology
Kelly M. Sunderland, Derek Beaton, Stephen R. Arnott, Peter Kleinstiver, Donna Kwan, Jane M. Lawrence-Dewar, Joel Ramirez, Brian Tan, Robert Bartha, Sandra E. Black, Michael Borrie, Donald Brien, Leanne K. Casaubon, Brian C. Coe, Benjamin Cornish, Allison A. Dilliott, Dar Dowlatshahi, Elizabeth Finger, Corinne Fischer, Andrew Frank, Julia Fraser, Morris Freedman, Barry Greenberg, David A. Grimes, Ayman Hassan, Wendy Hatch, Robert A. Hegele, Christopher Hudson, Mandar Jog, Sanjeev Kumar, Anthony Lang, Brian Levine, Wendy Lou, Jennifer Mandzia, Connie Marras, William McIlroy, Manuel Montero-Odasso, David G. Munoz, Douglas P. Munoz, Joseph B. Orange, David S. Park, Stephen H. Pasternak, Frederico Pieruccini-Faria, Tarek K. Rajji, Angela C. Roberts, John F. Robinson, Ekaterina Rogaeva, Demetrios J. Sahlas, Gustavo Saposnik, Christopher J. M. Scott, Dallas Seitz, Christen Shoesmith, Thomas D. L. Steeves, Michael J. Strong, Stephen C. Strother, Richard H. Swartz, Sean Symons, David F. Tang-Wai, Maria Carmela Tartaglia, Angela K. Troyer, John Turnbull, Lorne Zinman, Paula M. McLaughlin, Mario Masellis, Malcolm A. Binns
Summary: This is a multi-site, longitudinal, observational cohort study that aims to understand the impact of neurodegenerative and cerebrovascular diseases on dementia presentation. The study recruited 520 participants with prevalent neurodegenerative and cerebrovascular diseases and comprehensively assessed them. Results showed that the participants were predominantly White males with higher education levels. The data will be shared with the global scientific community to identify markers of disease severity, progression, and therapy targets.
ALZHEIMERS & DEMENTIA
(2023)
Review
Clinical Neurology
Maria Carmela Tartaglia, Ian R. A. Mackenzie
Summary: Frontotemporal dementia is a devastating neurodegenerative condition for which there is currently no effective treatment. Recent molecular discoveries provide a foundation for the development of biomarkers and targeted therapies.
CANADIAN JOURNAL OF NEUROLOGICAL SCIENCES
(2023)
Article
Rheumatology
Oshrat E. Tayer-Shifman, Kimberley Yuen, Robin Green, Mahta Kakvan, Patricia Katz, Kathleen S. Bingham, Juan Pablo Diaz-Martinez, Lesley Ruttan, Joan E. Wither, Maria Carmela Tartaglia, Jiandong Su, Dennisse Bonilla, May Y. Choi, Simone Appenzeller, Michelle Barraclough, Dorcas E. Beaton, Zahi Touma
Summary: The Montreal Cognitive Assessment (MoCA) does not have adequate concurrent criterion validity to accurately identify cognitive impairment (CI) in systemic lupus erythematosus (SLE) patients. The inclusion of MoCA in the Automated Neuropsychological Assessment Metrics (ANAM) does not significantly improve the accuracy of ANAM.
ARTHRITIS CARE & RESEARCH
(2023)
Article
Geriatrics & Gerontology
Duygu Hunerli-Gunduz, Yagmur Ozbek Isbitiren, Hakan Uzunlar, Berrin Cavusoglu, Berril Donmez Colakoglu, Emel Adag, Bahar Guntekin, Gorsev G. Yener
Summary: Parkinson's disease (PD) is a neurodegenerative disorder with cognitive impairment as a nonmotor manifestation. This study examined the differences between PD-MCI and PD-CN patients using visual event-related oscillations (EROs) and subcortical structural measures. The results showed that both PD-MCI and PD-CN patients had abnormal EROs compared to healthy controls, but the abnormalities were more pronounced in PD-MCI. Additionally, PD-MCI showed atrophy in multiple brain regions while PD-CN had reduced hippocampal volumes. These findings suggest complex interactions between electrophysiological, neuropsychological, and structural parameters in PD.
NEUROBIOLOGY OF AGING
(2023)
Article
Clinical Neurology
Gianina Toller, Yann Cobigo, Patrick Callahan, Brian S. Appleby, Danielle Brushaber, Kimiko Domoto-Reilly, Leah K. Forsberg, Nupur Ghoshal, Jonathan Graff-Radford, Neil R. Graff-Radford, Murray Grossman, Hilary W. Heuer, John Kornak, Walter Kremers, Maria Lapid, Gabriel Leger, Irene Litvan, Ian R. Mackenzie, Maria B. Pascual, Eliana M. Ramos, Katya Rascovsky, Julio C. Rojas, Adam M. Staffaroni, Maria C. Tartaglia, Arthur Toga, Sandra Weintraub, Zbigniew K. Wszolek, Brad F. Boeve, Adam L. Boxer, Howard J. Rosen, Katherine P. Rankin
Summary: This study modeled the changes in empathy in behavioral variant frontotemporal dementia (bvFTD) and found that empathic concern and perspective taking decline in the early stages of the disease regardless of genetic factors. The loss of empathy is progressive and correlates with subcortical atrophy.
ALZHEIMERS & DEMENTIA
(2023)
Article
Clinical Neurology
David J. Whiteside, Maura Malpetti, P. Simon Jones, Boyd C. P. Ghosh, Ian Coyle-Gilchrist, John C. van Swieten, Harro Seelaar, Lize Jiskoot, Barbara Borroni, Raquel Sanchez-Valle, Fermin Moreno, Robert Laforce, Caroline Graff, Matthis Synofzik, Daniela Galimberti, Mario Masellis, Maria Carmela Tartaglia, Elizabeth Finger, Rik Vandenberghe, Alexandre de Mendonca, Fabrizio Tagliavini, Chris R. Butler, Isabel Santana, Isabelle Le Ber, Alexander Gerhard, Simon Ducharme, Johannes Levin, Adrian Danek, Markus Otto, Sandro Sorbi, Florence Pasquier, Arabella Bouzigues, Lucy L. Russell, Jonathan D. Rohrer, James B. Rowe, Timothy Rittman
Summary: This study investigated the role of changes in functional networks in predicting cognitive decline and conversion to symptomatic disease in familial frontotemporal dementia (FTD). The study found a characteristic pattern of dynamic network changes in FTD, which were correlated with neuropsychological impairment. Among presymptomatic mutation carriers, this pattern of network dynamics was more prominent in those who later converted to the symptomatic phase. Baseline network dynamic changes predicted future cognitive decline in symptomatic participants and older presymptomatic participants.
ALZHEIMERS & DEMENTIA
(2023)
Article
Clinical Neurology
Ezgi Fide, Hasan Polat, Gorsev Yener, Mehmet Sirac Ozerdem
Summary: This study investigated the complexity changes in brain signals of Alzheimer's disease (AD) patients using the permutation entropy method. The findings showed regional alterations in the complexity profiles of different frequency bands, which were associated with cognitive scores. The results suggest that entropy measures can be useful in assessing medication effects in AD.
Article
Clinical Neurology
Blas Couto, Susan Fox, Maria Carmela Tartaglia, Ekaterina Rogaeva, Jeffrey Antwi, Puja Bhakta, Gabor G. Kovacs, Anthony E. E. Lang
Summary: The study focuses on the development and initial experience of the Rossy PSP Centre in Canada, which aims to advance clinical and basic research in progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). The study collects extensive demographic and longitudinal clinical information using standardized forms, as well as biofluids for genetic analysis and neuroimaging research protocols. The preliminary results show a typical distribution of phenotypes, demographics, and response to symptomatic treatments in the cohort. Future steps include enrolling patients in earlier stages, developing biomarkers, and fast-tracking well-characterized patients into clinical trials.
CANADIAN JOURNAL OF NEUROLOGICAL SCIENCES
(2023)
Article
Clinical Neurology
Claudio Del Percio, Susanna Lopez, Giuseppe Noce, Roberta Lizio, Federico Tucci, Andrea Soricelli, Raffaele Ferri, Flavio Nobili, Dario Arnaldi, Francesco Fama, Carla Buttinelli, Franco Giubilei, Moira Marizzoni, Bahar Guntekin, Gorsev Yener, Fabrizio Stocchi, Laura Vacca, Giovanni B. Frisoni, Claudio Babiloni
Summary: Abnormalities in rsEEG rhythms can be used to monitor and evaluate the progression of Alzheimer's disease. This study suggests that these abnormalities can be observed using fewer scalp electrodes in patients with mild cognitive impairment due to AD. The findings show that specific bipolar channels exhibit power density changes in ADMCI patients, and these changes can accurately classify ADMCI individuals.
CLINICAL EEG AND NEUROSCIENCE
(2023)
Article
Neurosciences
Ezgi Fide, Deniz Yerlikaya, Bahar Guntekin, Claudio Babiloni, Gorsev G. Yener
Summary: This study investigated the abnormal functional connectivity in patients with mild cognitive impairment (MCI) and dementia due to Alzheimer's disease (ADD) using imaginary part of coherency (ICoh) derived from event-related electroencephalographic oscillations. The results showed increased midline frontal and temporal theta coherence in the MCI group, decreased intra-hemispheric coherence in the ADD group, and normal inter-hemispheric coherence in both ADD and MCI groups. These findings highlight the disrupted functional connectivity in ADD and MCI patients, especially in the low-frequency bands.
COGNITIVE NEURODYNAMICS
(2023)
Review
Geriatrics & Gerontology
Burcu Akpinar Soylemez, Ecem Ozgul, Ozlem Kucukguclu, Gorsev Yener
Summary: This study aimed to investigate the effects of telehealth-based interventions on self-efficacy levels, caregiving burden, stress, depression, and quality of life for family caregivers of individuals with dementia. A systematic review and meta-analysis were conducted, and the results showed that telehealth applications can improve the self-efficacy levels of caregivers. However, there was no significant impact on caregiving burden, stress, depression, or quality of life.
Article
Geriatrics & Gerontology
Susanna Lopez, Claudio Del Percio, Roberta Lizio, Giuseppe Noce, Alessandro Padovani, Flavio Nobili, Dario Arnaldi, Francesco Fama, Davide V. Moretti, Annachiara Cagnin, Giacomo Koch, Alberto Benussi, Marco Onofrj, Barbara Borroni, Andrea Soricelli, Raffaele Ferri, Carla Buttinelli, Franco Giubilei, Bahar Guntekin, Gorsev Yener, Fabrizio Stocchi, Laura Vacca, Laura Bonanni, Claudio Babiloni
Summary: This study compared the hubs of interdependencies in elderly individuals with and without Alzheimer's disease dementia (ADD) using rsEEG rhythms. The results showed that ADD patients have lower connectivity centrality and directionality compared to normal elderly individuals.
FRONTIERS IN AGING NEUROSCIENCE
(2023)
Article
Neurosciences
Fatma Kubra Cekok, Turhan Kahraman, Arzu Genc, Gozde Duran, Berril Donmez Colakoglu, Deniz Yerlikaya, Gorsev Yener
Summary: There is a significant association between executive and physical function in people with Parkinson's disease, with patients exhibiting poorer executive and physical functions compared to healthy individuals. Further studies should investigate whether treating one of these dysfunctions affects the other.
SOMATOSENSORY AND MOTOR RESEARCH
(2023)
Article
Clinical Neurology
Ozge Akgul, Ezgi Fide, Fatih Ozel, Koksal Alptekin, Emre Bora, Berna Binnur Akdede, Gorsev Yener
Summary: This study examined the amplitude and latency of event-related potential (ERP) P300 in schizophrenia patients compared to healthy controls during a Monetary Incentive Delay Task (MID). Results showed that the schizophrenia group had enhanced P300 amplitudes and delayed peak latency in the punishment condition, which were also associated with negative symptoms.
CLINICAL EEG AND NEUROSCIENCE
(2023)
Article
Neurosciences
Kerem Kemik, Emel Ada, Berrin Cavusoglu, Cansu Aykac, Derya Durusu Emek-Savas, Gorsev Yener
Summary: This study investigated neural activity changes in patients with Amnestic mild cognitive impairment (aMCI) using functional magnetic resonance imaging (fMRI). The results showed alterations in the visual network in resting-state and task-based fMRI, indicating that these changes may serve as early biomarkers for aMCI.
CNS NEUROSCIENCE & THERAPEUTICS
(2023)