Results 51 to 60 of about 16,389 (183)

Agnosia for accents in primary progressive aphasia

Neuropsychologia, 2013
As an example of complex auditory signal processing, the analysis of accented speech is potentially vulnerable in the progressive aphasias. However, the brain basis of accent processing and the effects of neurodegenerative disease on this processing are not well understood.
Fletcher, PD, Downey, LE, Agustus, JL, Hailstone, JC, Tyndall, MH, Cifelli, A, Schott, JM, Warrington, EK, Warren, JD   +8 more
openaire   +3 more sources

A 57‐Year‐Old Male With Behavioral Variant Frontotemporal Dementia and MATR3 and NOS3 Mutations

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT This report presents a case of behavioral variant frontotemporal dementia caused by mutations in the MATR3 and NOS3 genes, aiming to analyze its clinical manifestations and genetic characteristics. For a case presenting with personality changes and gait abnormalities as the initial symptoms, this study conducted a comprehensive analysis of its
Feifei Lin, Saie Huang
wiley   +1 more source

Stage‐Dependent β‐Synuclein Links MRI and Cognitive Decline in Alzheimer's Disease

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Synaptic degeneration drives cognitive decline in Alzheimer's disease (AD), but synaptic biomarkers are scarce. Brain‐enriched β‐synuclein emerged as a synaptic damage marker. We investigated its diagnostic, prognostic, and structural correlates across the AD continuum.
Ulaş Ay, Merve Alaylioglu, Erdi Sahin, Sanem Sultan Yoruk Oner, Gulce Iskan, Gulce Cosku Yilmaz Cakan, Gozde Kizilates‐Evin, Emre Hari, Elif Kurt, Erdinc Dursun, Basar Bilgic, Hasmet Hanagasi, Hakan Gurvit, Tamer Demiralp, Duygu Gezen‐Ak, Bedia Samanci   +15 more
wiley   +1 more source

Case Report: Barely Able to Speak, Can’t Stop Echoing: Echolalic Dynamic Aphasia in Progressive Supranuclear Palsy

Frontiers in Aging Neuroscience, 2021
The diagnostic criteria for progressive supranuclear palsy (PSP) incorporate two speech-language disturbances (SLDs), non-fluent/agrammatic primary progressive aphasia and progressive apraxia of speech, but overlook the inclusion of other SLDs, including
Marcelo L. Berthier, Marcelo L. Berthier, Marcelo L. Berthier, Florencia Hoet, Florencia Hoet, Florencia Hoet, Álvaro Beltrán-Corbellini, Daniel Santana-Moreno, Lisa Edelkraut, Lisa Edelkraut, Lisa Edelkraut, Lisa Edelkraut, Guadalupe Dávila, Guadalupe Dávila, Guadalupe Dávila, Guadalupe Dávila   +15 more
doaj   +1 more source

Severe ADEM‐Like Neuroinflammatory Disease and Cerebrovascular Fragility With Recurrent Pseudoaneurysms and Moyamoya in a Familial Germline CBL Mutation: Expanding the Clinical Phenotype

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Heterozygous germline variants in CBL disrupt its function as a negative regulator of the Ras/MAPK pathway, classically predisposing to Juvenile myelomonocytic leukemia (JMML) and moyamoya. We describe two affected siblings carrying a paternally inherited CBL variant (c.1210 T> C, p.
Michal Bar‐Hakim, Anat Abramovich, Sara Via‐Dorembus, Gali Heimer, Bruria Ben‐Zeev, Etai Adam, Assaf A. Barg, Jonathan Roth, Basheer Sheick‐Yousif, Rony Beeri Berkowiz, Michal Feldon, Haim Bassan, Moran Hausman‐Kedem   +12 more
wiley   +1 more source

Gephyrin Neurological Autoimmunity

Annals of Neurology, EarlyView.
Gephyrin is a postsynaptic scaffold protein essential for inhibitory neurotransmission. Gephyrin‐immunoglobulin G (IgG) was reported, decades ago, in a single case of paraneoplastic stiff‐person‐like syndrome, but its broader clinical relevance remains unknown.
Maria Chiara Pantuliano, Friederike A. Arlt, Naomi Arenson, Irena Dujmovic Basuroski, Divyanshu Dubey, Connie E. Lesnick, John R. Mills, Sean J. Pittock, Anastasia Zekeridou, Michael A. Lane, Andrew McKeon   +10 more
wiley   +1 more source

Case report: Behavioral variant FTD confounding a language variant FTD in a case of PSP-CBS

Frontiers in Dementia
Frontotemporal dementia (FTD) occurs in two main clinical subtypes, which can transition into one another: the behavioral variant (bvFTD) and the language variant (primary progressive aphasia; PPA).
Alexandra V. Jürs, Alexandra V. Jürs, Elisabeth Kasper, Manuela Neumann, Manuela Neumann, Jens Kurth, Bernd J. Krause, Daniel Cantré, Johannes Prudlo, Johannes Prudlo   +9 more
doaj   +1 more source

Efficacy, safety and cost‐effectiveness of CAR‐T therapy

British Journal of Clinical Pharmacology, EarlyView.
CAR T‐cells demonstrate high efficacy in blood cancers, including ALL, MM and DLBCL. Innovations target solid tumours despite challenges such as antigen escape. Combination therapies enhance the delivery and infiltration of CAR T cells. Toxicity, cost and resistance remain major barriers to clinical use.
Emina Karahmet Sher, Amina Džidić‐Krivić, Emma Pinjic, Nejra Selak, Kanita Omerbasic, Alexandar Chupin, Andrej Belancic, Almir Fajkic   +7 more
wiley   +1 more source

Unsupervised clustering of electroclinical features in temporal lobe epilepsy: A data‐driven approach

Epileptic Disorders, EarlyView.
Abstract Objective To identify clinically meaningful patterns in ictal electroclinical features of focal epilepsy using a data‐driven, unsupervised learning approach, and to assess whether such patterns can localize and lateralize the epileptogenic zone (EZ) more accurately than conventional electroclinical interpretation.
Maria Vlachou, Bernadett Molnár, Helene Kaas, Jesper Tveit, Harald Aurlien, Martin Ejler Fabricius, Guido Rubboli, Lars Hageman Pinborg, Sándor Beniczky   +8 more
wiley   +1 more source

A review on primary progressive aphasia

Neuropsychiatric Disease and Treatment, 2007
Gabriel C Léger1,2, Nancy Johnson31Neurology Service, Hôtel-Dieu du Centre Hospitalier de l’Univertité de Montréal, Montréal, Québec, Canada; 2Faculty of Medicine, Universit& ...
Gabriel C Léger, Nancy Johnson   +1 more
doaj