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.
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
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.
Review
Clinical Neurology
Shane Lyons, Dominic Trepel, Tim Lynch, Richard Walsh, Sean O'Dowd
Summary: This study conducted a systematic review to investigate the incidence and prevalence of progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). The findings showed heterogeneity in the epidemiology of these conditions, highlighting the need for further research to understand their true burden.
JOURNAL OF NEUROLOGY
(2023)
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, 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
Neurosciences
David J. Whiteside, Duncan Street, Alexander G. Murley, P. Simon Jones, Maura Malpetti, Boyd C. P. Ghosh, Ian Coyle-Gilchrist, Alexander Gerhard, Michele T. T. Hu, Johannes C. Klein, P. Nigel Leigh, Alistair Church, David J. Burn, Huw R. Morris, James B. Rowe, Timothy Rittman
Summary: The study found that the differences in network connectivity between PSP and CBS patients are associated with disease severity, survival time, and rate of change in clinical severity. This finding is important for understanding the prognosis factors of PSP and CBS.
HUMAN BRAIN MAPPING
(2023)
Article
Clinical Neurology
Yuya Saito, Koji Kamagata, Peter A. Wijeratne, Christina Andica, Wataru Uchida, Kaito Takabayashi, Shohei Fujita, Toshiaki Akashi, Akihiko Wada, Keigo Shimoji, Masaaki Hori, Yoshitaka Masutani, Daniel C. Alexander, Shigeki Aoki
Summary: This study used a novel machine learning technique called SuStaIn to reveal the difference in brain atrophy progression patterns between patients with corticobasal syndrome (CBD-CBS) and those with Richardson's syndrome (PSP-RS). The results showed high accuracy and sensitivity in classifying these diseases based on structural brain MRI data. SuStaIn has potential for improving our understanding of disease mechanisms, accurately stratifying patients, and providing prognoses for patients with CBD and PSP-RS.
FRONTIERS IN NEUROLOGY
(2022)
Article
Geriatrics & Gerontology
Katie A. Peterson, P. Simon Jones, Nikil Patel, Kamen A. Tsvetanov, Ruth Ingram, Stefano F. Cappa, Matthew A. Lambon Ralph, Karalyn Patterson, Peter Garrard, James B. Rowe
Summary: Speech and language impairments in patients with PSP, CBS, and nfvPPA show similarities in cortical thickness reduction in bilateral frontal regions and striatal volume reduction. Multivariate analysis reveals associations between different speech and language domains and atrophy of specific brain regions.
FRONTIERS IN AGING NEUROSCIENCE
(2021)
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
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
Cecile Hubsch, Marc Ziegler Ivan Mari, Teddy Leguillier, Marie Lebouteux, Jean-Philippe Brandel
Summary: This study investigated the effects of continuous subcutaneous apomorphine infusion (CSAI) on patients with Progressive Supranuclear Palsy (PSP) and Corticobasal Degeneration (CBD). The results suggest that CSAI can improve the quality of life and reduce pain in these patients.
PARKINSONISM & RELATED DISORDERS
(2022)
Article
Neurosciences
Natalia Parjane, Sunghye Cho, Sharon Ash, Katheryn A. Q. Cousins, Sanjana Shellikeri, Mark Liberman, Leslie M. Shaw, David J. Irwin, Murray Grossman, Naomi Nevler
Summary: The study found that speech features of PSPS-CBS overlap with those of naPPA, showing some commonalities but also differences. Both groups of patients exhibited consistent characteristics in speech, including shorter speech segments, more pauses, and slower speech rate, with speech features correlating with CSF pTau levels.
JOURNAL OF ALZHEIMERS DISEASE
(2021)
Article
Clinical Neurology
Benedict D. Michael, Dean Walton, Erica Westenberg, David Garcia-Azorin, Bhagteshwar Singh, Arina A. Tamborska, M. Netravathi, Mashina Chomba, Greta K. Wood, Ava Easton, Omar K. Siddiqi, Thomas A. Jackson, Thomas A. Pollak, Timothy R. Nicholson, Shalini Nair, Gerome Breen, Kameshwar Prasad, Kiran T. Thakur, Sherry H. -Y. Chou, Erich Schmutzhard, Jennifer A. Frontera, Raimund Helbok, Alessandro Padovani, David K. Menon, Tom Solomon, Andrea S. Winkler
Summary: Encephalopathy is a common and challenging condition among hospitalized COVID-19 patients, which can have a negative impact on prognosis. This review provides practical guidance on the management of COVID-19 encephalopathy through consensus agreement of the Global COVID-19 Neuro Research Coalition, covering definition, epidemiology, pathophysiology, clinical assessment, investigation, and both acute and long-term management.
JOURNAL OF NEUROPSYCHIATRY AND CLINICAL NEUROSCIENCES
(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
Marco Bolchini, Francesca Schiano di Cola, Giulia Ceccardi, Salvatore Caratozzolo, Paolo Liberini, Renata Rao, Alessandro Padovani
Summary: The aim of this study was to evaluate the efficacy of erenumab 140 mg in the treatment of migraine disability and intensity in patients with high-frequency episodic migraine. The results showed a significant and progressive improvement in migraine pain intensity and disability during re-treatment.
EUROPEAN NEUROLOGY
(2023)
Article
Neurosciences
Enrico Premi, Juergen Dukart, Irene Mattioli, Ilenia Libri, Marta Pengo, Yasmine Gadola, Maria Cotelli, Rosa Manenti, Giuliano Binetti, Stefano Gazzina, Antonella Alberici, Mauro Magoni, Giacomo Koch, Roberto Gasparotti, Alessandro Padovani, Barbara Borroni
Summary: Primary progressive aphasias (PPAs) are a group of neurodegenerative diseases characterized by language impairment and possible presence of other symptoms. In this study, researchers used the JuSpace toolbox to analyze the correlation between brain changes in PPA patients and various neurotransmitter systems. The results showed significant associations between voxel-based brain changes and the distribution of serotonin, dopamine, and glutamatergic pathways. Additionally, disease severity and behavioral symptoms were negatively correlated with specific neurotransmitter systems. This pilot study highlights the potential of JuSpace in assessing neurotransmitter deficits in neurodegenerative dementias and gaining insight into disease mechanisms and associated clinical features.
HUMAN BRAIN MAPPING
(2023)
Article
Clinical Neurology
Andrea Morotti, Qi Li, Valentina Mazzoleni, Jawed Nawabi, Frieder Schlunk, Federico Mazzacane, Giorgio Busto, Elisa Scola, Laura Brancaleoni, Sebastiano Giacomozzi, Luigi Simonetti, Michele Laudisi, Anna Cavallini, Andrea Zini, Ilaria Casetta, Enrico Fainardi, Dar Dowlatshahi, Alessandro Padovani, Francesco Arba
Summary: This study found that non-contrast computed tomography (NCCT) features are related to hematoma expansion (HE) in intracerebral hemorrhage (ICH), but the diagnostic accuracy is influenced by the time of examination. Early examination can better identify hypodensities and heterogeneous density in patients with HE, while late examination is more accurate in identifying irregular shape. These findings may improve the utilization of NCCT in future studies and clinical trials targeting HE.
INTERNATIONAL JOURNAL OF STROKE
(2023)
News Item
Clinical Neurology
Alessandro Padovani, Andrea Pilotto
Summary: A new study provides evidence for a link between COVID-19 and long-term neurological syndromes, emphasizing the need for further research and the development of strategies.
NATURE REVIEWS NEUROLOGY
(2023)
Article
Clinical Neurology
Lize C. Jiskoot, Lucy L. Russell, Georgia Peakman, Rhian S. Convery, Caroline V. Greaves, Martina Bocchetta, Jackie M. Poos, Harro Seelaar, Lucia A. A. Giannini, John C. van Swieten, Rick van Minkelen, Yolande A. L. Pijnenburg, James B. Rowe, Barbara Borroni, Daniela Galimberti, Mario Masellis, Carmela Tartaglia, Elizabeth Finger, Chris R. Butler, Caroline Graff, Robert Laforce Jr, Raquel Sanchez-Valle, Alexandre de Mendonca, Fermin Moreno, Matthis Synofzik, Rik Vandenberghe, Simon Ducharme, Isabelle le Ber, Johannes Levin, Markus Otto, Florence Pasquier, Isabel Santana, David M. Cash, David Thomas, Jonathan D. Rohrer
Summary: The Benson Complex Figure Test (BCFT) shows potential as a sensitive cognitive marker for frontotemporal dementia (FTD). Differences were found in BCFT Copy, Recall, and Recognition scores between presymptomatic and symptomatic FTD mutation carriers, with correlations to cognitive function and neuroimaging results. These findings suggest limited potential of BCFT as a cognitive biomarker for upcoming clinical trials in presymptomatic to early-stage FTD.
JOURNAL OF THE NEUROLOGICAL SCIENCES
(2023)
Article
Clinical Neurology
Silvia Paola Caminiti, Andrea Pilotto, Enrico Premi, Alice Galli, Elisabetta Ferrari, Stefano Gipponi, Elisabetta Cottini, Barbara Paghera, Daniela Perani, Alessandro Padovani
Summary: This study aimed to investigate the dynamic changes of local and long-distance dopaminergic networks in the DLB continuum. Brain SPECT was used to measure brain dopamine transporter (DAT) density, and the results showed that DLB patients had similar basal ganglia deficits as compared to the control group. However, DLB patients in the dementia stage exhibited more connectivity losses, while the prodromal group showed increased connectivity.
PARKINSONISM & RELATED DISORDERS
(2023)
Article
Clinical Neurology
Kiran Samra, Amy M. MacDougall, Arabella Bouzigues, Martina Bocchetta, David M. Cash, Caroline Greaves, Rhian S. Convery, Chris Hardy, John C. van Swieten, Harro Seelaar, Lize C. Jiskoot, Fermin Moreno, Raquel Sanchez-Valle, Robert Laforce, Caroline Graff, Mario Masellis, Maria Carmela Tartaglia, James B. Rowe, Barbara Borroni, Elizabeth Finger, Matthis Synofzik, Daniela Galimberti, Rik Vandenberghe, Alexandre de Mendonca, Chris R. Butler, Alexander Gerhard, Simon Ducharme, Isabelle Le Ber, Isabel Santana, Florence Pasquier, Johannes Levin, Markus Otto, Sandro Sorbi, Jason D. Warren, Jonathan D. Rohrer, Lucy L. Russell
Summary: Samra et al. report that progranulin mutations are the most common genetic cause of primary progressive aphasia, with two subtypes observed. Revised criteria for primary progressive aphasia should take into account genetic phenotypes. Primary progressive aphasia is typically sporadic, but can also be genetic.
BRAIN COMMUNICATIONS
(2023)
Article
Clinical Neurology
Martina Bocchetta, Emily G. Todd, Arabella Bouzigues, David M. Cash, Jennifer M. Nicholas, Rhian S. Convery, Lucy L. Russell, David L. Thomas, Ian B. Malone, Juan Eugenio Iglesias, John C. van Swieten, Lize C. Jiskoot, Harro Seelaar, Barbara Borroni, Daniela Galimberti, Raquel Sanchez-Valle, Robert Laforce, Fermin Moreno, Matthis Synofzik, Caroline Graff, Mario Masellis, Maria Carmela Tartaglia, James B. Rowe, Rik Vandenberghe, Elizabeth Finger, Fabrizio Tagliavini, Alexandre de Mendonca, Isabel Santana, Chris R. Butler, Simon Ducharme, Alexander Gerhard, Adrian Danek, Johannes Levin, Markus Otto, Sandro Sorbi, Isabelle Le Ber, Florence Pasquier, Jonathan D. Rohrer
Summary: The study quantified brain anomalies on MRI in individuals with C9orf72, MAPT, and GRN mutations. The identified imaging markers associated with clinical and behavioral changes in presymptomatic carriers over one year, providing important data for participant stratification in trials. Biomarkers predicting disease progression in genetic frontotemporal dementia are urgently needed.
BRAIN COMMUNICATIONS
(2023)
Article
Medical Laboratory Technology
Francisco Meda, Joel Simren, Barbara Borroni, Valentina Cantoni, Silvana Archetti, Giorgio Biasiotto, Ulf Andreasson, Kaj Blennow, Hlin Kvartsberg, Henrik Zetterberg
Summary: This study aimed to analytically and clinically validate a commercial progranulin kit for clinical use. The results showed that the kit has excellent stability and diagnostic performance in both analytical and clinical validation. This easy-to-use test can help identify potential GRN mutation carriers and guide further evaluation.
CLINICAL CHEMISTRY AND LABORATORY MEDICINE
(2023)
Article
Medicine, General & Internal
Barbara Risi, Stefano Cotti Piccinelli, Stefano Gazzina, Beatrice Labella, Filomena Caria, Simona Damioli, Loris Poli, Alessandro Padovani, Massimiliano Filosto
Summary: The MUNIX technique can estimate the number and size of surviving motor units (MUs). Previous studies found correlations between MUNIX and several clinical measures in ALS patients, but its potential as a predictor of disease progression rate (DPR) has not been thoroughly evaluated. Our study aimed to investigate the ability of MUNIX to predict DPR at a six-month follow up. The results showed that MUNIX could predict DPR in ALS patients and could be used as a prognostic tool.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Obstetrics & Gynecology
Margaret Mascherpa, Anna Fichera, Rossana Orabona, Daniela Recupero, Barbara Borroni, Franco Edoardo Odicino, Federico Prefumo
Summary: An optimal materno-neonatal outcome was achieved in a pregnancy involving inherited episodic ataxia through a multidisciplinary team managing the timing and mode of delivery.
INTERNATIONAL JOURNAL OF GYNECOLOGY & OBSTETRICS
(2023)
Article
Clinical Neurology
Alice Galli, Andrea Pilotto, Benedetta Chiarini, Marcello Giunta, Daniele Corbo, Clara Tirloni, Daniel Ferreira, Enrico Premi, Alessandro Lupini, Cinzia Zatti, Laura Bonanni, Pietro Tiraboschi, Roberto Gasparotti, Alessandro Padovani
Summary: This study found that prodromal DLB and full-blown dementia stages are characterized by reduced occipital and posterior-temporal volume and thickness. Occipital atrophy is more sensitive than insular atrophy in differentiating prodromal DLB and healthy controls. The structural damage in the frontotemporal area increases along with occipital atrophy from prodromal DLB to full-blown dementia.
ALZHEIMER'S & DEMENTIA: DIAGNOSIS, ASSESSMENT & DISEASE MONITORING
(2023)
Article
Radiology, Nuclear Medicine & Medical Imaging
Andrea Morotti, Giorgio Busto, Gregoire Boulouis, Elisa Scola, Alessandro Padovani, Ilaria Casetta, Enrico Fainardi
Summary: The combined analysis of non-contrast CT and CT perfusion markers improves the prediction of hematoma expansion and outcome in intracerebral hemorrhage.
EUROPEAN RADIOLOGY
(2023)