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Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Rubika Balendra, Ahmad Al Khleifat, Ton Fang, Ammar Al-Chalabi
OBJECTIVE: Clinical stages in amyotrophic lateral sclerosis (ALS) can be measured using a simple system based on the number of CNS regions involved and requirement for gastrostomy or noninvasive ventilation (NIV). We aimed to design a standard operating procedure (SOP) to define the standardized use and application of the King's staging system. METHODS: We designed a SOP for the King's staging system. We wrote case vignettes representative of ALS patients at different disease stages...
February 18, 2019: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
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No abstract text is available yet for this article.
January 29, 2019: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Richard Bedlack
No abstract text is available yet for this article.
January 28, 2019: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Leif Simmatis, Ghada Atallah, Stephen H Scott, Sean Taylor
OBJECTIVE: We used the KINARM robot to quantify impairments in cognitive and upper-limb sensorimotor performance in a cohort of people with amyotrophic lateral sclerosis (ALS). We sought to study the feasibility of using this technology for ALS research, to quantify patterns of impairments in individuals living with ALS, and elucidate correlations between robotic and traditional clinical behavioral measures. METHODS: Participants completed robot-based behavioral tasks testing sensorimotor, cognitive, and proprioceptive performance...
January 27, 2019: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
JosÉ E de la Rubia, Eraci Drehmer, JosÉ L Platero, MarÍa Benlloch, Jordi Caplliure-Llopis, Carlos Villaron-Casales, Nieves de Bernardo, Jorge AlarcÓn, Cristian Fuente, Sandra Carrera, David Sancho, Pilar GarcÍa-Pardo, Raquel Pascual, Marta JuÁrez, María Cuerda-Ballester, Alfonso Forner, Sandra Sancho-Castillo, Carlos Barrios, Elena Obrador, Patricia Marchio, Rosario Salvador, Holly E Holmes, Ryan W Dellinger, Leonard Guarente, José M Estrela
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease, characterized by progressive loss of spinal and cortical motor neurons, leading to muscular atrophy, respiratory failure, and ultimately death. There is no known cure, and the clinical benefit of the two drugs approved to treat ALS remains unclear. Novel disease-modifying therapeutics that are able to modulate the disease course are desperately needed. Our objective was to evaluate the efficacy and tolerability of Elysium Health's candidate drug EH301 in people with ALS (PALS)...
January 22, 2019: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Giulia Vinceti, Nicholas Olney, Maria Luisa Mandelli, Salvatore Spina, H Isabel Hubbard, Miguel A Santos-Santos, Christa Watson, Zachary A Miller, Catherine Lomen-Hoerth, Paolo Nichelli, Bruce L Miller, Lea T Grinberg, William W Seeley, Maria Luisa Gorno-Tempini
OBJECTIVE: Behavioral variant frontotemporal dementia (bvFTD), is commonly considered the cognitive presentation of the frontotemporal dementia-motor neuron disease (FTD-MND) spectrum disorder. We evaluated the prevalence of primary progressive aphasia in a series of pathologically confirmed cases of FTD-MND spectrum. METHODS: Pathologically confirmed cases of frontotemporal lobar degeneration-motor neuron disease (FTLD-MND) were obtained from the UCSF brain bank...
January 22, 2019: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
R S Bedlack, Paul Wicks, Timothy Vaughan, Alicia Opie, Rebecca Blum, Amanda Dios, Ghazaleh Sadri-Vakili
OBJECTIVE: Lunasin, a soy peptide that reportedly alters histone acetylation in vitro, was associated with a single ALS reversal in the media. Following an ALSUntangled report, we sought to determine whether Lunasin altered histone acetylation and improved progression in people with ALS, and whether patient-centric trial design features might improve enrollment and retention. METHODS: This single-center, year-long trial (NCT02709330) featured broad inclusion criteria, historical controls, primarily virtual data collection, and real-time results...
January 21, 2019: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Sicong Tu, Ricarda A L Menke, Kevin Talbot, Matthew C Kiernan, Martin R Turner
The cerebellum shows neuropathological change in a number of neurodegenerative conditions where clinical involvement is not the primary feature, including amyotrophic lateral sclerosis (ALS). Whether these changes are associated with disruption to the direct cerebellar tract pathways to the motor cortex and spinal cord in ALS is uncertain. Diffusion tensor imaging was used to examine the integrity of two primary cerebellar pathways, the dentato-rubro-thalamo-cortical (DRTC) and spino-cerebellar (SC) tracts...
January 21, 2019: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
P Corcia, C Brulard, S Beltran, S Marouillat, S E Bakkouche, C R Andres, H Blasco, P Vourc'h
BACKGROUND: Amyotrophic lateral sclerosis is the most frequent motor neuron disorders (MND) in adults. The role of genetic factors is worldwide accepted, and currently, more than 30 genes have been linked to this disease. Genetics was also the matter of numerous studies in distal hereditary motor neuropathies (dHMN). GARS is classically linked to a predominant dHMN and, until now, no mutation has been described in GARS in other MND. CASE REPORT: We report the case of a 70-year-old woman who developed a classical bulbar ALS phenotype...
January 20, 2019: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Francesca Palese, Arianna Sartori, Giancarlo Logroscino, Federica Edith Pisa
OBJECTIVES: To characterize the timing and pathway of amyotrophic lateral sclerosis (ALS) diagnosis and to identify predictors of delayed diagnosis in a retrospective cohort. METHODS: The cohort included all patients with incident ALS between 2010 and 2014 in Friuli-Venezia Giulia (FVG) region, Italy, admitted to two University Hospitals. Information on demographics, clinical presentation, and healthcare use was obtained from health databases and electronic medical records (EMRs)...
January 18, 2019: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Eoin Finegan, Rangariroyashe H Chipika, Stacey Li Hi Shing, Orla Hardiman, Peter Bede
Primary lateral sclerosis (PLS) has been traditionally viewed as a distinct upper motor neuron condition (UMN) but is increasingly regarded as a sub-phenotype within the amyotrophic lateral sclerosis (ALS) spectrum. Despite established diagnostic criteria, formal diagnosis can be challenging and the protracted diagnostic journey and uncertainty about longer-term prognosis cause considerable distress to patients and caregivers. PLS patients are invariably excluded from ALS clinical trials, while PLS pharmacological trials are lacking...
January 18, 2019: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Jessica de Wit, Leonhard A Bakker, Annerieke C van Groenestijn, Joost F Baardman, Leonard H van den Berg, Johanna M A Visser-Meily, Carin D SchrÖder
OBJECTIVES: To assess psychological distress levels over time in caregivers of patients in the early stage of amyotrophic lateral sclerosis (ALS) and to investigate the association between caregivers' coping styles and psychological distress over time. METHODS: Fifty-four caregivers were followed in a RCT of patients in the early stage of ALS. Questionnaires were administered at baseline, 4, 7, and 10 months. Psychological distress was assessed using the Hospital Anxiety and Depression Scale (HADS) and task-, emotion-, and avoidance-oriented coping styles were identified with the short version of the Coping Inventory for Stressful Situations (CISS-21)...
January 18, 2019: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Qionghua Sun, Fei Yang, Hongfen Wang, Fang Cui, Yanran Li, Siyuan Li, Yuting Ren, Wenjie Lan, Mao Li, Wenjia Zhu, Xiaosun Sun, Zhengqing He, Xue Zhao, Li Yang, Xusheng Huang
OBJECTIVE: The objective of this study was to compare iron metabolic variables in the serum and cerebrospinal fluid (CSF) of patients with sporadic amyotrophic lateral sclerosis (sALS) with those of patients with multiple system atrophy (MSA) and control subjects. We also assessed the correlations of these variables with sALS progression and severity and estimated their roles in predicting prognosis. METHODS: We retrospectively collected iron metabolic parameters, including serum levels of iron, ferritin, transferrin levels and total iron binding capacity and the CSF level of ferritin, from 435 sALS patients, 176 MSA patients and 431 control subjects...
January 17, 2019: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Lauren C Tabor-Gray, Alessandra Gallestagui, Terrie Vasilopoulos, Emily K Plowman
OBJECTIVE: Although cough impairment (dystussia) is common in individuals with amyotrophic lateral sclerosis (ALS) and contributes to a reduced physiologic capacity to defend the airway, characteristics of dystussia have not yet been delineated. Therefore, we aimed to compare voluntary cough spirometry airflow patterns between individuals with ALS and healthy age and gender-matched controls. METHODS: Thirty-two individuals with a diagnosis of probable-definite ALS (El-Escorial Criterion) and 29 healthy age and gender-matched controls underwent voluntary cough spirometry testing...
January 17, 2019: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
EglĖ SukockienĖ, Gilles Allali, Jean-Paul Janssens, Ruxandra Iancu Ferfoglia
OBJECTIVE: Amyotrophic lateral sclerosis (ALS) is associated with co-existing motor and cognitive impairment in almost half of the patients; however, the relationship between cognitive and motor functioning has rarely been studied in ALS. We hypothesized that impaired executive functioning would be linked to poor mobility in ALS patients. METHODS: A total of 49 non-demented ambulant ALS patients (mean age: 68.4 ± 12.6 years; 53% female), were evaluated in the Centre for ALS and Related Disorders of Geneva University Hospitals...
January 17, 2019: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Natalie Simon, Laura H Goldstein
OBJECTIVES: Cognitive and behavioral change in Amyotrophic Lateral Sclerosis (ALS) is well-accepted. Several screening tools have been developed to detect such changes. Further guidance on their use may come from a consideration of the rigor with which they were validated. This systematic review set out to critically appraise and present published data pertaining to the validation of six screening tools used to diagnose cognitive and/or behavioral change in patients with ALS. METHODS: The screening tools considered in this search included: The Edinburgh Cognitive and Behavioural ALS Screen (ECAS), The ALS Cognitive Behavioural Screen (ALS-CBS), The Motor Neuron Disease Behavioural Scale (MiND-B), The Frontal Behavioural Inventory ALS Version, The ALS Frontotemporal Dementia Questionnaire (ALS-FTD-Q), and The Beaumont Behavioural Inventory (BBI)...
December 26, 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Elisa Semler, Lukas Petersdorff, Sarah Anderl-Straub, Sarah Böhm, Dorothée Lulé, Heiner Fangerau, Albert C Ludolph, Markus Otto, Ingo Uttner
OBJECTIVE: To investigate moral judgment competence in patients with the behavioral variant frontotemporal dementia (bvFTD) compared to amyotrophic lateral sclerosis (ALS) and controls. METHODS: N = 12 bvFTD, N = 22 ALS patients and N = 19 neurological unimpaired controls were examined. In the 'Moral Competence Test' (MCT), participants had to evaluate two moral dilemmas by predefined arguments to measure the consistency of their moral judgments. The 'Ethics Position Questionnaire' (EPQ) addresses whether individuals prefer binding ethical standards, the Idler Index of Religiosity (IIR) measures the level of religiosity...
December 4, 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Seward B Rutkove, Kristin Qi, Kerisa Shelton, Julie Liss, Visar Berisha, Jeremy M Shefner
OBJECTIVE: To design an ALS clinical study in which patients are remotely recruited, screened, enrolled and then assessed via daily data collection at home by themselves or caregivers. METHODS: This observational, natural-history study included two academic medical centers, one providing overall clinical management and the other overseeing computing and web-services design and management. Both healthy and ALS subjects were recruited on the Internet via advertisement on governmental and foundation websites as well as through Facebook and Google paid advertisements...
November 28, 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Shan Ye, Angela Rosenbohm, Sarah Böhm, Ingo Uttner, Ying Ji, Albert C Ludolph, Dorothée Lulé, Dongsheng Fan
This study aimed to clarify whether differences between German and Chinese studies using the standardized Edinburgh Cognitive and Behavioural ALS Screen (ECAS), might be explained by differences in translated versions of ECAS, by patient demographic or clinical characteristics or by population-specific factors. Comparisons were performed on data from two previous studies in Germany and China. We found except for spelling task (p = 0.05), no differences between control groups of two countries were detected after adjusting for demographics...
November 26, 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Nilda Turgut, Ozlem Birgili, Gamze Varol SaraÇoglu, Selahattin Kat, Kemal Balci, BabÜrhan GÜldiken, Levent Kabayel
OBJECTIVE: The aim of the study was to investigate the incidence and prevalence of amyotrophic lateral sclerosis (ALS) in Thrace, Turkey in a five-year time period (2006-2010). METHODS: Study population included residents of three provinces (Edirne, Tekirdag, Kırklareli) in the Thrace region. Cases were ascertained from all of the neurologic centers and hospitals of these provinces. Demographic and clinical information was collected for each patient. Newly diagnosed ALS patients who are fulfilling the El Escorial revised diagnostic criteria were enrolled into the study...
November 23, 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
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