journal
https://read.qxmd.com/read/37738529/rapidly-progressive-dementia-due-to-intravascular-lymphoma-a-prion-disease-reference-center-experience
#1
JOURNAL ARTICLE
Giuseppe Mario Bentivenga, Simone Baiardi, Lorenzo Righini, Anna Ladogana, Sabina Capellari, Elena Sabattini, Piero Parchi
BACKGROUND: Intravascular large B-cell lymphoma (IVLBCL) is a rare extranodal lymphoma that is characterized by the selective growth of neoplastic cells in blood vessels, representing a potentially treatable cause of rapidly progressive dementia (RPD). Given its diverse clinical and instrumental presentation, it is often misdiagnosed with more common RPD causes, for example, Creutzfeldt-Jakob disease (CJD) or vascular dementia. METHODS: This study presents the clinical and histopathological characteristics of four IVLBCL cases that we diagnosed post-mortem over 20 years among over 600 brain samples received as suspected CJD cases at our prion disease reference center...
September 22, 2023: European Journal of Neurology
https://read.qxmd.com/read/37738526/treatment-patterns-in-essential-tremor-real-world-evidence-from-a-united-kingdom-and-france-primary-care-database
#2
JOURNAL ARTICLE
Ippazio Cosimo Antonazzo, Davide Rozza, Sara Conti, Carla Fornari, Paolo Angelo Cortesi, Caroline Eteve-Pitsaer, Claire Paris, Laurène Gantzer, Dennis Valentine, Lorenzo Giovanni Mantovani, Giampiero Mazzaglia
BACKGROUND AND PURPOSE: Essential tremor (ET) is one of the most common neurological disorders, but information on treatment pattern is still scant. The aim of this study was to describe the demographic and clinical characteristics, treatment patterns, and determinants of drug use in patients with newly diagnosed ET in France and the United Kingdom. METHODS: Incident cases of ET diagnosed between January 1, 2015 and December 31, 2018 with 2 years of follow-up were identified by using The Health Improvement Network (THIN®) general practice database...
September 22, 2023: European Journal of Neurology
https://read.qxmd.com/read/37738525/do-monosymptomatic-stroke-patients-with-dizziness-present-a-vestibular-syndrome-without-nystagmus-an-underestimated-entity
#3
JOURNAL ARTICLE
Florence Nikles, Hassen Kerkeni, Ewa Zamaro, Athanasia Korda, Franca Wagner, Thomas C Sauter, Roger Kalla, Miranda Morrison, Georgios Mantokoudis
BACKGROUND AND PURPOSE: Vestibular symptoms are common in emergency department (ED) patients and have various causes, including stroke. Accurate identification of stroke in patients with vestibular symptoms is crucial for timely management. We conducted a prospective cross-sectional study from 2015 to 2019 to determine stroke prevalence and associated symptoms in ED patients with vestibular symptoms, aiming to improve diagnosis and outcomes. METHODS: As part of the DETECT project, we screened 1647 ED patients with acute vestibular symptoms...
September 22, 2023: European Journal of Neurology
https://read.qxmd.com/read/37738517/the-prevalence-and-prognosis-of-asymptomatic-intracranial-atherosclerosis-in-a-community-based-population-results-based-on-high-resolution-magnetic-resonance-imaging
#4
JOURNAL ARTICLE
Shengde Li, Mingyu Tang, Dingding Zhang, Fei Han, Lixin Zhou, Ming Yao, Mingli Li, Liying Cui, Shuyang Zhang, Bin Peng, Zhengyu Jin, Yicheng Zhu, Jun Ni
BACKGROUND AND PURPOSE: Intracranial atherosclerotic disease (ICAD) is a major cause of ischemic stroke in China, but the prevalence and prognosis of asymptomatic ICAD detected using high-resolution magnetic resonance imaging (HR-MRI) is largely unknown. The aim of this study was to investigate the prevalence and prognosis in order to guide neurologists in interpreting ICAD detected on HR-MRI. METHODS: We included stroke-free participants from a community-based prospective cohort (Shunyi study participants) who underwent HR-MRI between July 2014 and April 2016...
September 22, 2023: European Journal of Neurology
https://read.qxmd.com/read/37738515/frailty-is-associated-with-the-clinical-expression-of-neuropsychological-deficits-in-older-adults
#5
JOURNAL ARTICLE
Marco Canevelli, Lindsay M K Wallace, Giuseppe Bruno, Matteo Cesari, Kenneth Rockwood, David D Ward
BACKGROUND AND PURPOSE: The aim was to determine whether frailty is associated with the relationship between neuropsychological markers and global cognition in older adults. METHODS: Cross-sectional analyzes were conducted of baseline data from three large cohort studies: National Alzheimer's Coordinating Center (NACC), Rush Memory and Aging Project (MAP) and Alzheimer's Disease Neuroimaging Initiative (ADNI). Studies recruited North American participants along the spectrum of cognitive functioning (44% no cognitive impairment at baseline)...
September 22, 2023: European Journal of Neurology
https://read.qxmd.com/read/37725003/genetic-screening-for-hereditary-transthyretin-amyloidosis-with-polyneuropathy-in-western-sicily-two-years-of-experience-in-a-neurological-clinic
#6
JOURNAL ARTICLE
Vincenzo Di Stefano, Antonino Lupica, Paolo Alonge, Antonia Pignolo, Sofia Maria Augello, Francesca Gentile, Andrea Gagliardo, Francesca Giglia, Daniele Brinch, Maria Cappello, Daniela Di Lisi, Giuseppina Novo, Eugenia Borgione, Carmela Scuderi, Filippo Brighina
BACKGROUND AND PURPOSE: Hereditary transthyretin amyloidosis with polyneuropathy (ATTRv-PN) is caused by mutations in the TTR gene, leading to misfolded monomers that aggregate generating amyloid fibrils. METHODS: A prospective systematic genetic screening for ATTRv-PN was proposed in patients presenting with a sensory-motor idiopathic polyneuropathy and two or more "red flags" among the following: family history of polyneuropathy or cardiopathy, bilateral carpal tunnel syndrome, cardiac insufficiency, renal amyloidosis, lumbar tract stenosis, autonomic dysfunction, idiopathic gastrointestinal disease, amyloid deposits on biopsy, and vitreous opacities...
September 19, 2023: European Journal of Neurology
https://read.qxmd.com/read/37724995/serum-neurofilament-light-chain-a-promising-early-diagnostic-biomarker-for-hereditary-transthyretin-amyloidosis
#7
JOURNAL ARTICLE
Angela Romano, Guido Primiano, Giovanni Antonini, Marco Ceccanti, Silvia Fenu, Francesca Forcina, Luca Gentile, Maurizio Inghilleri, Luca Leonardi, Fiore Manganelli, Laura Obici, Andrea Sabino, Maria Ausilia Sciarrone, Stefano Tozza, Francesca Vitali, Marco Luigetti
BACKGROUND AND PURPOSE: Hereditary transthyretin amyloidosis (ATTRv) is a life-threatening disease caused by mutations in the gene encoding transthyretin (TTR). The recent therapeutic advances have underlined the importance of easily accessible, objective biomarkers of both disease onset and progression. Preliminary evidence suggests a potential role in this respect for neurofilament light chain (NfL). In this study, the aim was to determine serum NfL (sNfL) levels in a late-onset ATTRv population and evaluate whether it might represent a reliable biomarker of disease onset (i...
September 19, 2023: European Journal of Neurology
https://read.qxmd.com/read/37724992/levodopa-induced-orthostatic-hypotension-in-parkinsonism-a-red-flag-of-autonomic-failure
#8
JOURNAL ARTICLE
Ilaria Cani, Pietro Guaraldi, Giulia Giannini, Luisa Sambati, Giorgio Barletta, Pietro Cortelli, Giovanna Calandra-Buonaura
BACKGROUND AND PURPOSE: Levodopa (LD) is the main treatment for parkinsonism, but its use may be limited by a potential hypotensive effect. METHODS: We evaluated the cardiovascular effect of LD performing head-up tilt test (HUTT) before and 60 min after 100/25 mg LD/dopa-decarboxylase inhibitor (pre-LD vs. post-LD HUTT) in 164 patients with parkinsonism on chronic LD treatment. Features predictive of LD-induced orthostatic hypotension (OH) were assessed by logistic regression analysis...
September 19, 2023: European Journal of Neurology
https://read.qxmd.com/read/37724989/reverse-lasagna-s-law-the-ethics-and-undesirable-consequences-of-overbooking-patients-for-trials
#9
EDITORIAL
Thomas C van den Hoek, Michel D Ferrari
No abstract text is available yet for this article.
September 19, 2023: European Journal of Neurology
https://read.qxmd.com/read/37724986/adults-living-with-duchenne-muscular-dystrophy-old-and-new-challenges-in-a-cohort-of-19-patients-in-their-third-to-fifth-decade
#10
JOURNAL ARTICLE
Giulio Gadaleta, Guido Urbano, Chiara Brusa, Rossella D'Alessandro, Enrica Rolle, Ilaria Cavallina, Alessio Mattei, Fulvia Ribolla, Claudia Raineri, Stefano Pidello, Liliana Vercelli, Federica S Ricci, Tiziana E Mongini
BACKGROUND AND PURPOSE: Advances in multidisciplinary care are extending overall survival in Duchenne muscular dystrophy (DMD) patients. Our research objective was to delineate the clinical characteristics of this particular cohort and identify novel challenges associated with the disease. METHODS: Nineteen individuals aged 25-48 years (median 34 years) with a confirmed diagnosis of out-of-frame DMD gene mutation were selected. RESULTS: All patients were mechanically ventilated (5/19 via tracheostomy), with different patterns of cardiomyopathy...
September 19, 2023: European Journal of Neurology
https://read.qxmd.com/read/37707348/rates-of-john-cunningham-virus-seroconversion-greatly-reduced-in-natalizumab-treated-patients-during-covid-19-related-lockdowns
#11
JOURNAL ARTICLE
Chris Dwyer, Sifat Sharmin, Tomas Kalincik
BACKGROUND AND PURPOSE: This study was undertaken to retrospectively compare rates of John Cunningham virus (JCV) seroconversion in natalizumab-treated patients before and during COVID-19-related community restrictions. Natalizumab is highly effective therapy for relapsing-remitting multiple sclerosis. Prolonged exposure to natalizumab in JCV-positive patients can cause progressive multifocal leukoencephalopathy, a potentially fatal brain infection. Serial assessment of JCV status is required for patients receiving natalizumab...
September 14, 2023: European Journal of Neurology
https://read.qxmd.com/read/37697728/healthcare-employment-as-a-risk-factor-for-functional-neurological-disorder-a-case-control-study
#12
JOURNAL ARTICLE
Giorgio Vanini, Janine Bühler, Samantha Weber, Manuela Steinauer, Selma Aybek
BACKGROUND AND PURPOSE: Female gender, younger age and stressful life events are known predisposing factors for functional neurological disorders (FNDs). Employment in a healthcare profession has also been suggested to be a predisposing factor. We set out to conduct a large-scale case-control study to estimate the rate employment in a healthcare profession among people with FND. METHODS: We included 200 consecutive patients with a confirmed diagnosis of FND, referred to our clinic at University Hospital Bern Switzerland between October 1, 2016, and August 1, 2019...
September 11, 2023: European Journal of Neurology
https://read.qxmd.com/read/37697714/anti-amyloid-antibody-treatments-for-alzheimer-s-disease
#13
REVIEW
Robert Perneczky, Geert Dom, Andrew Chan, Peter Falkai, Claudio Bassetti
Our aim is to review the most recent evidence on novel antibody therapies for Alzheimer's disease directed against amyloid-β. This is a joint statement of the European Association of Neurology and the European Psychiatric Association. After numerous unsuccessful endeavors to create a disease-modifying therapy for Alzheimer's disease, substantial and consistent evidence supporting the clinical effectiveness of monoclonal antibodies aimed at amyloid-β is finally emerging. The latest trials not only achieved their primary objective of slowing the progression of the disease over several months but also demonstrated positive secondary clinical outcomes and a decrease in amyloid-β levels as observed through positron emission tomography scans...
September 11, 2023: European Journal of Neurology
https://read.qxmd.com/read/37694681/systematic-review-of-drug-therapy-for-chorea-in-nxk2-1-related-disorders-efficacy-and-safety-evidence-from-case-studies-and-series
#14
REVIEW
Laia Nou-Fontanet, Carmen Martín-Gómez, Rebeca Isabel-Gómez, Anne-Catherine Bachoud-Lévi, Giovanna Zorzi, Alessandro Capuano, Juan Antonio Blasco-Amaro, Juan Darío Ortigoza-Escobar
BACKGROUND: The NKX2-1-related disorders (NKX2-1-RD) is a rare disorder characterized by choreiform movements along with respiratory and endocrine abnormalities. The European Reference Network of Rare Neurological Disorders funded by the European Commission conducted a systematic review to assess drug treatment of chorea in NKX2-1-RD, aiming to provide clinical recommendations for its management. METHODS: A systematic pairwise review using various databases, including MEDLINE, Embase, Cochrane, CINAHL, and PsycInfo, was conducted...
September 11, 2023: European Journal of Neurology
https://read.qxmd.com/read/37691341/home-based-exergaming-to-treat-gait-and-balance-disorders-in-patients-with-parkinson-s-disease-a-phase-ii-randomized-controlled-trial
#15
JOURNAL ARTICLE
Dijana Nuic, Sjors van de Weijer, Saoussen Cherif, Anna Skrzatek, Eline Zeeboer, Claire Olivier, Jean-Christophe Corvol, Pierre Foulon, Jénica Z Pastor, Gregoire Mercier, Brian Lau, Bastiaan R Bloem, Nienke M De Vries, Marie-Laure Welter
BACKGROUND: Exergaming has been proposed to improve gait and balance disorders in Parkinson's disease (PD) patients. We aimed to assess the efficacy of a home-based, tailored, exergaming training system designed for PD patients with dopa-resistant gait and/or balance disorders in a controlled randomized trial. METHODS: We recruited PD patients with dopa-resistant gait and/or balance disorders. Patients were randomly assigned (1:1 ratio) to receive 18 training sessions at home by playing a tailored exergame with full-body movements using a motion capture system (Active group), or by playing the same game with the computer's keyboard (Control group)...
September 10, 2023: European Journal of Neurology
https://read.qxmd.com/read/37688443/theory-of-mind-and-executive-dysfunction-in-chronic-inflammatory-demyelinating-polyneuropathy
#16
JOURNAL ARTICLE
Hannah Lochmann, Michelle Wyrobnik, Christin Kupper, Charlotte Rewitzer, Fabian Klostermann
BACKGROUND AND PURPOSE: Although chronic inflammatory demyelinating polyneuropathy (CIDP) is understood as a disease affecting the peripheral nervous system, mild cognitive dysfunction, particularly in the executive domain, has been described to form part of the condition. Here our interest lay in CIDP-related theory of mind (ToM) capacities as an aspect of social cognition relevant for many aspects of everyday life. METHODS: Twenty-nine patients with CIDP and 23 healthy controls participated in this study...
September 9, 2023: European Journal of Neurology
https://read.qxmd.com/read/37679868/serpina1-levels-in-amyotrophic-lateral-sclerosis-patients-an-exploratory-study
#17
JOURNAL ARTICLE
Ilaria Martinelli, Elisabetta Zucchi, Cecilia Simonini, Giulia Gianferrari, Roberta Bedin, Chiara Biral, Andrea Ghezzi, Nicola Fini, Serena Carra, Jessica Mandrioli
BACKGROUND: SerpinA1, a serine protease inhibitor, is involved in the modulation of microglial-mediated inflammation in neurodegenerative diseases. We explored SerpinA1 levels in cerebrospinal fluid (CSF) and serum of amyotrophic lateral sclerosis (ALS) patients to understand its potential role in the pathogenesis of the disease. METHODS: SerpinA1, neurofilament light (NfL) and heavy (NfH) chain, and chitinase-3-like protein-1 (CHI3L1) were determined in CSF and serum of ALS patients (n = 110) and healthy controls (n = 10) (automated next-generation ELISA), and correlated with clinical parameters, after identifying three classes of progressors (fast, intermediate, slow)...
September 7, 2023: European Journal of Neurology
https://read.qxmd.com/read/37658515/associations-of-postprandial-ghrelin-liver-expressed-antimicrobial-peptide-2-and-leptin-levels-with-body-composition-disease-progression-and-survival-in-patients-with-amyotrophic-lateral-sclerosis
#18
JOURNAL ARTICLE
Stephanie L Howe, Cory J Holdom, Pamela A McCombe, Robert D Henderson, Jeffrey M Zigman, Shyuan T Ngo, Frederik J Steyn
BACKGROUND AND PURPOSE: Loss of appetite contributes to weight loss and faster disease progression in amyotrophic lateral sclerosis (ALS). Impairment of appetite control in ALS may include altered production or action of orexigenic (i.e., ghrelin) and anorexigenic (i.e., liver-expressed antimicrobial peptide 2 [LEAP2] and leptin) hormones. We aimed to determine if postprandial circulating ghrelin levels, LEAP2 levels, LEAP2:ghrelin molar ratio and leptin levels differ in ALS patients compared to non-neurodegenerative disease controls, and whether they are associated with disease progression and body composition...
September 1, 2023: European Journal of Neurology
https://read.qxmd.com/read/37650365/cognitive-effect-of-antiseizure-medications-in-medial-temporal-lobe-epilepsy
#19
JOURNAL ARTICLE
Cedric Dusanter, Marion Houot, Marie Mere, Marisa Denos, Séverine Samson, Bastien Herlin, Vincent Navarro, Sophie Dupont
BACKGROUND AND PURPOSE: The specific effects of antiseizure medications (ASMs) on cognition are a rich field of study, with many ongoing questions. The aim of this study was to evaluate these effects in a homogeneous group of patients with epilepsy to guide clinicians to choose the most appropriate medications. METHODS: We retrospectively identified 287 refractory patients with medial temporal lobe epilepsy associated with hippocampal sclerosis. Scores measuring general cognition (global, verbal and performance IQ), working memory, episodic memory, executive functions, and language abilities were correlated with ASM type, number, dosage and generation (old vs...
August 31, 2023: European Journal of Neurology
https://read.qxmd.com/read/37650337/clinical-evidence-in-ischemic-stroke-where-we-have-gone-so-far-and-hopes-for-the-future
#20
JOURNAL ARTICLE
Tianhua Li, Chengyu Song, David S Liebeskind, Adam A Dmytriw, Ran Xu, Xue Wang, Jie Wang, Hengxiao Zhao, Wenbo Cao, Haozhi Gong, Chao Zhang, Xuesong Bai, Liqun Jiao
OBJECTIVE: Ischemic stroke is a significant cause of disability and death worldwide. Randomized clinical trials (RCTs) are important in changing guidelines and treatment strategies. This study aimed to analyze the progress of RCTs in ischemic stroke and to guide future research directions. METHODS: Ischemic stroke-related RCT articles were identified in six high-impact medical journals using the Web of Science Core Collection database. Google Scholar was used to check whether relevant articles were included in the guidelines...
August 31, 2023: European Journal of Neurology
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