journal
https://read.qxmd.com/read/38648007/a-case-of-myotonic-dystrophy-type-1-with-severe-dilated-cardiomyopathy-an-unusual-presenting-manifestation-of-the-most-common-muscular-dystrophy-in-adults
#1
LETTER
Ritwik Ghosh, Moisés León-Ruiz, Abdus Samim Mondal, Souvik Dubey, Julián Benito-León
No abstract text is available yet for this article.
April 22, 2024: Neurological Sciences
https://read.qxmd.com/read/38644454/efgartigimod-as-a-novel-fcrn-inhibitor-for-autoimmune-disease
#2
REVIEW
Yun Yang, Zhengxuan Shen, Fan Shi, Fei Wang, Ning Wen
Immunoglobulin G (IgG) autoantibodies can lead to the formation of autoimmune diseases through Fab and/or Fc-mediated interactions with host molecules as well as activated T cells. The neonatal Fc receptor (FcRn) binds at acidic pH IgG and albumin, and the mechanism for prolonging serum IgG half-life is making IgG re-entry into circulation by prompting it not to be degraded by lysosomes and back to the cell surface. Given the FcRn receptor's essential role in IgG homeostasis, one of the strategies to promote the quick degradation of endogenous IgG is to suppress the function of FcRn, which is beneficial to the treatment of IgG-driven autoimmune disorders like myasthenia gravis (MG), chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), stiff person syndrome, and immune thrombocytopenia (ITP)...
April 22, 2024: Neurological Sciences
https://read.qxmd.com/read/38642323/rare-association-between-spinocerebellar-ataxia-and-amyotrophic-lateral-sclerosis-a-case-series
#3
JOURNAL ARTICLE
Valerio Ferrari, Matteo Conti, Roberta Bovenzi, Rocco Cerroni, Mariangela Pierantozzi, Nicola B Mercuri, Alessandro Stefani
INTRODUCTION: In this work, we describe a new case of association between SCA2 and MND. CASE REPORT: A 58-year-old man who was diagnosed with spinocerebellar ataxia type 2 presented dysphagia and a significant decline in his ability to walk, with a reduction in autonomy and the need to use a wheelchair. We performed electromyography and electroneurography of the four limbs and of the cranial district and motor-evoked potentials to study upper and lower motor neurons...
April 20, 2024: Neurological Sciences
https://read.qxmd.com/read/38642322/establishment-of-a-mild-cognitive-impairment-risk-model-in-middle-aged-and-older-adults-a-longitudinal-study
#4
JOURNAL ARTICLE
Xin Zhang, Hong Fan, Chengnan Guo, Yi Li, Xinyu Han, Yiyun Xu, Haili Wang, Tiejun Zhang
BACKGROUND: Early identification individuals at high risk of mild cognitive impairment (MCI) is essential for prevention and intervention strategies of dementia, such as Alzheimer's disease. MCI prediction considering the interdependence of predictors in longitudinal data needs to be further explored. We aimed to employ machine learning (ML) to develop and verify a prediction model of MCI. METHODS: In a longitudinal population-based cohort of China Health and Retirement Longitudinal Study (CHARLS), 8390 non-MCI participants were enrolled...
April 20, 2024: Neurological Sciences
https://read.qxmd.com/read/38642321/the-role-of-neuromodulation-in-the-management-of-drug-resistant-epilepsy
#5
REVIEW
HusamEddin Salama, Ahmed Salama, Logan Oscher, George I Jallo, Nir Shimony
Drug-resistant epilepsy (DRE) poses significant challenges in terms of effective management and seizure control. Neuromodulation techniques have emerged as promising solutions for individuals who are unresponsive to pharmacological treatments, especially for those who are not good surgical candidates for surgical resection or laser interstitial therapy (LiTT). Currently, there are three neuromodulation techniques that are FDA-approved for the management of DRE. These include vagus nerve stimulation (VNS), deep brain stimulation (DBS), and responsive neurostimulation (RNS)...
April 20, 2024: Neurological Sciences
https://read.qxmd.com/read/38639894/correlation-between-autistic-traits-and-brain-functional-connectivity-in-preschoolers-with-autism-spectrum-disorder-a-resting-state-meg-study
#6
JOURNAL ARTICLE
Matilde Taddei, Pablo Cuesta, Silvia Annunziata, Sara Bulgheroni, Silvia Esposito, Elisa Visani, Alice Granvillano, Sara Dotta, Davide Sebastiano Rossi, Ferruccio Panzica, Silvana Franceschetti, Giulia Varotto, Daria Riva
BACKGROUND: Neurophysiological studies recognized that Autism Spectrum Disorder (ASD) is associated with altered patterns of over- and under-connectivity. However, little is known about network organization in children with ASD in the early phases of development and its correlation with the severity of core autistic features. METHODS: The present study aimed at investigating the association between brain connectivity derived from MEG signals and severity of ASD traits measured with different diagnostic clinical scales, in a sample of 16 children with ASD aged 2 to 6 years...
April 19, 2024: Neurological Sciences
https://read.qxmd.com/read/38639893/longitudinal-change-of-energy-expenditure-body-composition-and-dietary-habits-in-progressive-supranuclear-palsy-patients
#7
JOURNAL ARTICLE
Maria Francesca Tepedino, Anna Rosa Avallone, Filomena Abate, Marina Serio, Miriam Caterino, Roberto Erro, Maria Teresa Pellecchia, Paolo Barone, Marina Picillo
INTRODUCTION: Alterations in metabolic status, body composition, and food intake are present in all neurodegenerative diseases. Aim of this study was to detect the progression of these changes in Progressive Supranuclear Palsy (PSP). METHODS: We conducted a longitudinal study of 15 patients with PSP. The assessments were performed at baseline (T0) and after 7(IQR = 5) months of follow-up (T1). We collected anthropometric measures including body weight, height, body mass index and waist circumference, metabolic parameters through indirect calorimeters, body composition using bioimpedance analysis, and dietary habits with a validated questionnaire...
April 19, 2024: Neurological Sciences
https://read.qxmd.com/read/38637341/exploring-the-intersection-of-tuberous-sclerosis-and-precocious-puberty-unveiled-by-hematocolpos
#8
JOURNAL ARTICLE
Ramandeep Singh, Sameer Peer, Arvinder Wander
We present the case of a 6-year-old girl who initially presented with acute pelvic pain, ultimately diagnosed with imperforate hymen leading to hematocolpos. Further investigation revealed additional clinical features including academic struggles, mood swings, and cutaneous findings, prompting consideration of a neurocutaneous syndrome. Magnetic Resonance Imaging (MRI) revealed features consistent with tuberous sclerosis complex (TSC), including radial migration lines in the subcortical white matter and an incidental arachnoid cyst...
April 19, 2024: Neurological Sciences
https://read.qxmd.com/read/38634957/the-imaging-findings-of-migraine-with-visual-aura-in-a-cadasil-patient
#9
JOURNAL ARTICLE
Ming Fang, Mingyue Wang
Head MRI images of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) patients during migraine attacks are rare.
April 18, 2024: Neurological Sciences
https://read.qxmd.com/read/38632177/a-systematic-review-about-the-importance-of-neuropsychological-features-in-heart-failure-is-at-heart-the-only-failure
#10
REVIEW
Luigi Maria Chiatto, Francesco Corallo, Rocco Salvatore Calabrò, Davide Cardile, Maria Pagano, Irene Cappadona
Heart failure can lead to cognitive impairment that is estimated to be present in over a quarter of patients. It is important to intervene at a cognitive level to promote brain plasticity through cognitive training programs. Interventions transformed by technology offer the promise of improved cognitive health for heart failure patients. This review was conducted on studies evaluating the role of cognitive rehabilitation in patients with heart failure. We examined clinical trials involving patients with heart failure...
April 17, 2024: Neurological Sciences
https://read.qxmd.com/read/38632176/research-progress-on-intestinal-microbiota-regulating-cognitive-function-through-the-gut-brain-axis
#11
REVIEW
Kaijie Lin, Feng Peng, Kunyang He, Zhengyu Qian, Xuan Mei, Zhikun Su, Yilihamujiang Wujimaiti, Xun Xia, Tianyao Zhang
The intestinal microbiota community is a fundamental component of the human body and plays a significant regulatory role in maintaining overall health and in the management disease states.The intestinal microbiota-gut-brain axis represents a vital connection in the cognitive regulation of the central nervous system by the intestinal microbiota.The impact of intestinal microbiota on cognitive function is hypothesized to manifest through both the nervous system and circulatory system. Imbalances in intestinal microbiota during the perioperative period could potentially contribute to perioperative neurocognitive dysfunction...
April 17, 2024: Neurological Sciences
https://read.qxmd.com/read/38627298/safety-of-masitinib-in-patients-with-neurodegenerative-diseases-a-meta-analysis-of-randomized-controlled-trials
#12
JOURNAL ARTICLE
Abdullah Ashraf Hamad, Basma Ehab Amer
OBJECTIVES: This meta-analysis aimed to examine the safety of masitinib in patients with neurodegenerative diseases. METHODS: We considered randomized controlled trials (RCTs) comparing different doses of masitinib versus placebo. We performed our analysis using the R (v.4.3.0) programming language and the incidence of adverse events was pooled using risk ratio (RR) and 95% confidence interval (CI). RESULTS: We included five RCTs, focusing on multiple sclerosis (MS), Alzheimer's disease (AD), and amyotrophic lateral sclerosis...
April 17, 2024: Neurological Sciences
https://read.qxmd.com/read/38625608/efficacy-of-rtms-combined-with-cognitive-training-in-tbi-with-cognition-disorder-a-systematic-review-and-meta-analysis
#13
REVIEW
Jia Chen, Yuanwei Dong, Hong Guo, Tianyu Zhao, Di Zhang, Song Jin
Post-traumatic brain injury cognitive disorder(PTBICD) is one of the common symptoms of TBI survivors, severely limiting their life and rehabilitation progress. Repetitive transcranial magnetic stimulation (rTMS) has been shown to modulate cognition in a non-invasive manner while there are inconsistencies in previous studies. A comprehensive systematic review of rTMS treatment in patients with PTBICD is warranted. To evaluate the efficacy and safety of rTMS + cognitive training(CT) in enhancing cognitive function among PTBICD patients...
April 16, 2024: Neurological Sciences
https://read.qxmd.com/read/38622454/investigation-of-total-cerebellar-and-flocculonodular-lobe-volume-in-parkinson-s-disease-and-healthy-individuals-a-brain-segmentation-study
#14
JOURNAL ARTICLE
Merve Nur Ozgen, Necati Emre Sahin, Nurcan Ertan, Bunyamin Sahin
BACKGROUND: Parkinson's disease (PD) is a neurodegenerative disorder with an unexplored link to the cerebellum. In the pathophysiology of balance disorders in PD, the role of the flocculonodular lobe (FL) is linked to the impairment of the dopaminergic system. Dopamine deficiency can also lead to changes in cerebellum functions, disrupting balance control. This study compares cerebellar and FL volumes between healthy controls (HC) and PD patients, analyzing their correlation with clinical outcomes...
April 16, 2024: Neurological Sciences
https://read.qxmd.com/read/38622453/long-term-treatment-of-hereditary-transthyretin-amyloidosis-with-patisiran-multicentre-real-world-experience-in-italy
#15
JOURNAL ARTICLE
Luca Gentile, Anna Mazzeo, Chiara Briani, Silvia Casagrande, Marcella De Luca, Gian Maria Fabrizi, Christian Gagliardi, Chiara Gemelli, Francesca Forcina, Marina Grandis, Valeria Guglielmino, Giacomo Iabichella, Luca Leonardi, Alessandro Lozza, Fiore Manganelli, Roberta Mussinelli, Filomena My, Giuseppe Occhipinti, Silvia Fenu, Massimo Russo, Angela Romano, Alessandro Salvalaggio, Matteo Tagliapietra, Stefano Tozza, Giovanni Palladini, Laura Obici, Marco Luigetti
BACKGROUND: Hereditary transthyretin (ATTRv, v for variant) amyloidosis with polyneuropathy is a rare disease caused by mutations in the transthyretin gene. In ATTRv amyloidosis, multisystem extracellular deposits of amyloid cause tissue and organ dysfunction. Patisiran is a small interfering RNA molecule drug that reduces circulating levels of mutant and wild-type TTR proteins. Prior to its regulatory approval, patisiran was available in Italy through a compassionate use programme (CUP)...
April 16, 2024: Neurological Sciences
https://read.qxmd.com/read/38622452/acute-ataxic-neuropathy-due-to-bartonella-infection-a-rare-variant-for-a-rare-association
#16
LETTER
Giorgia Sciacca, Angelo Battiato, Mario Zappia
No abstract text is available yet for this article.
April 15, 2024: Neurological Sciences
https://read.qxmd.com/read/38622451/large-vessel-occlusion-detection-by-non-contrast-ct-using-artificial-%C3%A4-ntelligence
#17
JOURNAL ARTICLE
Emrah Aytaç, Murat Gönen, Sinan Tatli, Ferhat Balgetir, Sengul Dogan, Turker Tuncer
INTRODUCTION: Computer vision models have been used to diagnose some disorders using computer tomography (CT) and magnetic resonance (MR) images. In this work, our objective is to detect large and small brain vessel occlusion using a deep feature engineering model in acute of ischemic stroke. METHODS: We use our dataset. which contains 324 patient's CT images with two classes; these classes are large and small brain vessel occlusion. We divided the collected image into horizontal and vertical patches...
April 15, 2024: Neurological Sciences
https://read.qxmd.com/read/38622450/correlation-between-early-computed-tomography-findings-and-neurological-outcome-in-pediatric-traumatic-brain-injury-patients
#18
JOURNAL ARTICLE
Süleyman Şahin, Edin Botan, Emrah Gün, Merve Feyza Yüksel, Nurşah Yeniay Süt, Ayşe Tuğba Kartal, Anar Gurbanov, Fevzi Kahveci, Hasan Özen, Merve Havan, Miraç Yıldırım, Seda Kaynak Şahap, Ömer Bektaş, Serap Teber, Suat Fitoz, Tanıl Kendirli
Traumatic brain injury (TBI) is a leading cause of morbidity and mortality in children. Head computed tomography (CT) is frequently utilized for evaluating trauma-related characteristics, selecting treatment options, and monitoring complications in the early stages. This study assessed the relationship between cranial CT findings and early and late neurological outcomes in pediatric TBI patients admitted to the pediatric intensive care unit (PICU). The study included children aged 1 month to 18 years who were admitted to the PICU due to TBI between 2014 and 2020...
April 15, 2024: Neurological Sciences
https://read.qxmd.com/read/38613590/myotonic-dystrophy-type-1-in-south-korea-a-comprehensive-analysis-of-cancer-and-comorbidity-risks
#19
JOURNAL ARTICLE
Incheol Seo, Jin-Mo Park
BACKGROUND AND PURPOSE: Myotonic dystrophy type 1 (DM1) is an inherited neuromuscular disorder characterized by myotonia and progressive muscle weakness. Beyond the primary symptoms, there is growing concern regarding a higher incidence of certain comorbidities in DM1 patients, including cancer, diabetes, thyroid dysfunction, and cataracts. This study was designed to examine the occurrence of these conditions among patients diagnosed with DM1 in South Korea, using data from the National Health Insurance Service database...
April 13, 2024: Neurological Sciences
https://read.qxmd.com/read/38607534/cognitive-assessment-during-the-phases-of-a-spontaneous-migraine-a-prospective-cohort-study
#20
JOURNAL ARTICLE
Jason C Ray, David Darby, Helmut Butzkueven, Manjit S Matharu, Elspeth J Hutton
INTRODUCTION: Cognitive symptoms are reported commonly throughout all phases of a migraine; however, there is a paucity of objective cognitive profiling. Previous studies have been limited by practice effect, and variable populations. METHODS: Participants completed 1 month of daily testing with a computerised cognitive battery involving a simple reaction (SRT), choice reaction (CRT) and a working memory test (WM). Results were correlated with their diary to identify interictal scores, and scores during each phase of a migraine, and non-migraine headache days...
April 12, 2024: Neurological Sciences
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