Article
Clinical Neurology
Marina Picillo, Maria Francesca Tepedino, Filomena Abate, Sara Ponticorvo, Roberto Erro, Sofia Cuoco, Nevra Oksuz, Gianfranco Di Salle, Francesco Di Salle, Fabrizio Esposito, Maria Teresa Pellecchia, Renzo Manara, Paolo Barone
Summary: This study evaluated the asymmetry in motor and higher cortical features in PSP-RS patients and found that these features were commonly asymmetric in PSP-RS. There was also a greater degree of asymmetry in the hemispheres compared to healthy controls. These findings contribute to a better understanding of the characteristics of PSP-RS.
NEUROLOGICAL SCIENCES
(2022)
Article
Geriatrics & Gerontology
Yafei Wen, Qijie Yang, Bin Jiao, Weiwei Zhang, Jingyi Lin, Yuan Zhu, Qian Xu, Hui Zhou, Ling Weng, Xinxin Liao, Yafang Zhou, Junling Wang, Jifeng Guo, Xinxiang Yan, Hong Jiang, Beisha Tang, Lu Shen
Summary: This study characterized the clinical manifestations, MRI, and longitudinal MRI changes of progressive supranuclear palsy (PSP) in China. The results showed significant differences in clinical symptoms, MRI parameters, and longitudinal MRI changes between PSP-P and PSP-RS. Additionally, some MRI parameters could be potential markers to distinguish PSP-P from Parkinson's disease (PD).
FRONTIERS IN AGING NEUROSCIENCE
(2023)
Article
Neurosciences
Jong Hyeon Ahn, Junmo Kwon, Ji Hye Won, Kyoungseob Byeon, Jinyoung Youn, Hyunjin Park, Jin Whan Cho
Summary: This study investigated waiting impulsivity in progressive supranuclear palsy-Richardson's syndrome (PSP-RS) and introduced a novel evaluation method. The results showed that the JTG sign is a surrogate marker of waiting impulsivity in PSP-RS patients, enriching the current understanding of waiting impulsivity in PSP patients.
FRONTIERS IN NEUROSCIENCE
(2023)
Article
Clinical Neurology
Blas Couto, Ivan Martinez-Valbuena, Seojin Lee, Isabel Alfradique-Dunham, Richard J. Perrin, Joel S. Perlmutter, Carlos Cruchaga, Ain Kim, Naomi Visanji, Christine Sato, Ekaterina Rogaeva, Anthony E. Lang, Gabor G. Kovacs
Summary: This study reveals a broader range of disease courses in progressive supranuclear palsy (PSP) than previously known. Time to gait dependence and cognitive impairment are proposed as prognostic milestones. Genetic polymorphisms in TRIM11 and SLC2A13 genes are associated with longer disease duration. The study suggests a subtype of PSP with protracted course and explores its relationship with protein misfolding, seeding activity, and propagation.
EUROPEAN JOURNAL OF NEUROLOGY
(2022)
Article
Clinical Neurology
Viviana A. Martinez-Villota, Cinthia Terroba-Chambi, Sergio A. Castillo-Torres, Malco Rossi, Marcelo Merello
Summary: This study evaluated the clinical correlates of fear of falling in patients with progressive supranuclear palsy (PSP) and multiple system atrophy (MSA). The findings suggest that mobility, balance, and gait performance, as well as anxiety and depression, are determinants of fear of falling in PSP and MSA, while apathy is a determinant in MSA. These results highlight the importance of a multidisciplinary approach to fear of falling in neurodegenerative atypical parkinsonism.
EUROPEAN JOURNAL OF NEUROLOGY
(2023)
Article
Radiology, Nuclear Medicine & Medical Imaging
Priyanka Tupe-Waghmare, Archith Rajan, Shweta Prasad, Jitender Saini, Pramod Kumar Pal, Madhura Ingalhalikar
Summary: This study demonstrates the feasibility of using radiomics features extracted from T1-weighted MRI images to differentiate Parkinson's disease from atypical parkinsonian syndromes. The results showed high accuracy rates for classifiers distinguishing PD from HC, PD from APS, and a 3-way classifier for PD/MSA/PSP, highlighting the potential of radiomics in aiding clinical diagnosis in early stages of disease.
EUROPEAN RADIOLOGY
(2021)
Article
Clinical Neurology
Mike Wattjes, Hans-Juergen Huppertz, Nima Mahmoudi, Sophia Stoecklein, Sophia Rogozinski, Florian Wegner, Martin Klietz, Ivayla Apostolova, Johannes Levin, Sabrina Katzdobler, Carsten Buhmann, Andrea Quattrone, Georg Berding, Matthias Brendel, Henryk Barthel, Osama Sabri, Guenter Hoeglinger, Ralph Buchert, Alzheimers Dis Neuroimaging Initiat
Summary: This study compared the value of different MRI reading strategies and automatic classification methods in the diagnosis of PSP. The results showed that the fully automatic classification method using support vector machine performed the best, especially in the diagnosis of vPSP. Therefore, it is recommended to use machine learning methods for fully automatic classification in settings with a broad phenotypic PSP spectrum.
MOVEMENT DISORDERS
(2023)
Article
Clinical Neurology
Arthur Cassa Macedo, Paulo Caramelli, Elisa de Paula Franca Resende, Luciano Inacio Mariano, Vitor Tumas, Antonio Lucio Teixeira, Sarah Teixeira Camargos, Francisco Eduardo Costa Cardoso, Leonardo Cruz de Souza
Summary: The study found that episodic memory impairment in PSP may be related to the damage of medial temporal structures, rather than solely due to executive dysfunction.
NEUROLOGICAL SCIENCES
(2022)
Article
Clinical Neurology
Srikanth Yadav Boini, Rohan Mahale, Seshagiri Doniparthi Venkata, Nitish Kamble, Vikram Holla, Pramod Kumar Pal, Bindu Kutty, Ravi Yadav
Summary: The study investigates the association between oculomotor abnormalities and sleep stages in progressive supranuclear palsy (PSP) using video polysomnography. The results show a significant negative correlation between vertical saccadic restriction and total sleep time and sleep efficiency. The findings suggest that oculomotor and sleep abnormalities are interconnected in PSP.
Article
Medicine, General & Internal
Piotr Alster, Michal Nieciecki, Bartosz Migda, Michal Kutylowski, Natalia Madetko, Karolina Duszynska-Was, Ingeborga Charzynska, Dariusz Koziorowski, Leszek Krolicki, Andrzej Friedman
Summary: This study examined patients with MSA-P and PSP-P, two types of atypical parkinsonism that are difficult to differentiate in the early years of development. The researchers found that extended examination using MRI and perfusion SPECT, focusing on the evaluation of the mesencephalon/pons ratio, mesencephalic volume decrease, MRPI, and frontal perfusion, may be more feasible than cognitive evaluation in the 3-6-year duration of symptoms.
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
Morgane Darricau, Taxiarchis Katsinelos, Flavio Raschella, Tomislav Milekovic, Louis Crochemore, Qin Li, Gregoire Courtine, William A. McEwan, Benjamin Dehay, Erwan Bezard, Vincent Planche
Summary: This study demonstrates that progressive supranuclear palsy (PSP) patient-derived tau aggregates can induce motor and behavioral impairments in non-human primates, showing characteristics of prion-like seeding and spreading of PSP lesions. This pilot study paves the way for using PSP tau-injected macaques as a relevant animal model to accelerate drug development for this rare and fatal neurodegenerative disease.
Article
Medicine, General & Internal
Michal Kutylowski, Piotr Alster, Natalia Madetko-Alster, Anna Marta Migda, Leszek Krolicki, Bartosz Migda
Summary: This study examined a group of patients with atypical parkinsonism using a 1.5 T MRI system and aimed to find simple and repeatable measurements that may be useful to distinguish between these diseases. The results suggest that the maximal width of the frontal horns of the lateral ventricles and Evans' Index may be useful as basic and simple measurements in the diagnostic imaging of patients with atypical parkinsonism.
Review
Clinical Neurology
Inigo Ruiz-Barrio, Andrea Horta-Barba, Ignacio Illan-Gala, Jaime Kulisevsky, Javier Pagonabarraga
Summary: This review describes important features and clues of PSP syndrome of monogenic origin, as well as a diagnostic algorithm that may be helpful in genetic diagnosis.
FRONTIERS IN NEUROLOGY
(2022)
Article
Medicine, General & Internal
Haiyan Tian, Jiuqi Wang, Renyi Feng, Rui Zhang, Han Liu, Chi Qin, Lin Meng, Yongkang Chen, Yu Fu, Dongxiao Liang, Xin Yuan, Yanping Zhai, Qingyong Zhu, Lingjing Jin, Junfang Teng, Xuebing Ding, Xuejing Wang
Summary: This clinical trial aimed to investigate the clinical efficacy of fecal microbiota transplantation (FMT) in patients with progressive supranuclear palsy-Richardson's syndrome (PSP-RS). The results showed that compared to the placebo group, FMT treatment significantly improved motor and nonmotor symptoms in PSP-RS patients, as well as reduced intestinal inflammation and enhanced intestinal barrier function.
Article
Biochemistry & Molecular Biology
Shelley L. Forrest, Jordan Hanxi Kim, Daniel R. Crockford, Katharine Huynh, Rosie Cheong, Samantha Knott, Madison A. Kane, Lars M. Ittner, Glenda M. Halliday, Jillian J. Kril
Summary: This study maps the distribution and density of astrocytes in the human frontal cortex, revealing distinct and overlapping populations of astrocytes. The findings provide a reference map for comparative studies in various disease and injury states involving astrocytes.
NEUROCHEMICAL RESEARCH
(2023)
Article
Biochemistry & Molecular Biology
Zac Chatterton, Praves Lamichhane, Diba Ahmadi Rastegar, Lauren Fitzpatrick, Helene Lebhar, Christopher Marquis, Glenda Halliday, John B. Kwok
Summary: This study presents a single-cell DNA methylation sequencing method that can accurately resolve the DNA methylation profiles of individual cells. The sciEM method improves sequencing alignment rates, reduces adapter contamination, and over-estimation of DNA methylation levels. The researchers successfully applied this method to resolve single-cell DNA methylation of major cell types in the human brain.
CELL AND BIOSCIENCE
(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)
Editorial Material
Substance Abuse
Virginia Macdonald, Bradley Mathers, Keith Sabin, Angela Me, Chloe Carpentier, Kamran Niaz, Antons Mozalevskis, Niklas Luhmann, Monica Ciupagea, Fariba Soltani, Annette Verster
Review
Immunology
Caitlin E. Kennedy, Ping Teresa Yeh, Annette Verster, Niklas Luhmann, Nabeel Mangadan Konath, Maeve Brito de Mello, Rachel Baggaley, Virginia Macdonald
Summary: Counselling behavioural interventions do not have a significant impact on the prevention of HIV/STI/VH among key populations, according to a systematic review and meta-analysis. Although participants liked specific counselling interventions and intervention costs were reasonable, the evidence was limited and mostly focused on HIV.
JOURNAL OF THE INTERNATIONAL AIDS SOCIETY
(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
Multidisciplinary Sciences
Lyndal Henden, Liam G. Fearnley, Natalie Grima, Emily P. McCann, Carol Dobson-Stone, Lauren Fitzpatrick, Kathryn Friend, Lynne Hobson, Sandrine Chan Moi Fat, Dominic B. Rowe, Susan D'Silva, John B. Kwok, Glenda M. Halliday, Matthew C. Kiernan, Srestha Mazumder, Hannah C. Timmins, Margaret Zoing, Roger Pamphlett, Lorel Adams, Melanie Bahlo, Ian P. Blair, Kelly L. Williams
Summary: In this study, we analyzed whole-genome sequencing data from 608 patients with sporadic ALS, 68 patients with sporadic FTD, and 4703 matched controls using three tools. We found that 17.6% of clinically diagnosed ALS and FTD cases had expanded STR alleles reported to be pathogenic or intermediate for other neurodegenerative diseases, providing evidence for their contribution to the development of these diseases. We also identified multiple disease-relevant STR expansions associated with neurodegenerative diseases, highlighting the clinical and pathological pleiotropy of neurodegenerative disease genes and their importance in ALS and FTD.
Article
Neurosciences
Oana C. Marian, Jonathan D. Teo, Jun Yup Lee, Huitong Song, John B. Kwok, Ramon Landin-Romero, Glenda Halliday, Anthony S. Don
Summary: Heterozygous mutations in the GRN gene and hexanucleotide repeat expansions in C9orf72 are the most common genetic causes of Frontotemporal Dementia (FTD) with TDP-43 protein inclusions. Our comprehensive lipidomic analysis suggests that both C9orf72 and GRN mutations disrupt lysosomal homeostasis and result in white matter lipid loss, but GRN mutations cause more pronounced disruption to myelin lipid metabolism. The findings provide biochemical evidence supporting the use of MRI measures of white matter integrity in the diagnosis and management of FTD.
ACTA NEUROPATHOLOGICA COMMUNICATIONS
(2023)
Review
Cell Biology
Jen-Hsiang T. Hsiao, Onur Tanglay, Anne A. Li, Aysha Y. G. Strobbe, Woojin Scott Kim, Glenda M. Halliday, YuHong Fu
Summary: Multiple system atrophy (MSA) is a debilitating movement disorder with unknown etiology. It presents with characteristic parkinsonism and/or cerebellar dysfunction due to deterioration in specific brain regions. The early pathological events and development mechanisms of MSA are reviewed, focusing on the involvement of oligodendrocyte lineage cells and alpha-synuclein. This understanding will guide future research in MSA.
Letter
Clinical Neurology
Jordan Carroll, Heather Mccann, Glenda M. Halliday, John B. Kwok, Carol Dobson-Stone, Claire E. Shepherd
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
Neurosciences
Adahir Labrador-Garrido, Siying Zhong, Laura Hughes, Shikara Keshiya, Woojin S. Kim, Glenda M. Halliday, Nicolas Dzamko
Summary: This study utilized a GCase substrate probe and live cell imaging to measure lysosomal enzyme activity. The results showed heterogeneity in GCase activity among PD patients, and a significant inverse correlation between GCase activity and α-synuclein protein levels. These findings suggest the importance of GCase in PD, and demonstrate the utility of this live cell imaging assay for further research on GCase in PD.
FRONTIERS IN CELLULAR NEUROSCIENCE
(2023)
Letter
Clinical Neurology
Matthew C. Kiernan, Glenda M. Halliday, Dominic B. Rowe, Rachel H. Tan
NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY
(2023)