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Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

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https://www.readbyqxmd.com/read/28799809/nutrition-management-methods-effective-in-increasing-weight-survival-time-and-functional-status-in-als-patients-a-systematic-review
#1
Jaylin Kellogg, Lindsey Bottman, Erin J Arra, Stephen M Selkirk, Frances Kozlowski
Poor prognosis and decreased survival time correlate with the nutritional status of patients with amyotrophic lateral sclerosis (ALS). Various studies were reviewed which assessed weight, body mass index (BMI), survival time and ALS functional rating scale revised (ALSFRS-R) in order to determine the best nutrition management methods for this patient population. A systematic review was conducted using CINAHL, Medline, and PubMed, and various search terms in order to determine the most recent clinical trials and observational studies that have been conducted concerning nutrition and ALS...
August 11, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28799808/decision-making-among-patients-and-their-family-in-als-care-a-review
#2
Geraldine Foley, Geralyn Hynes
OBJECTIVES: Practice guidelines in ALS care emphasise the role of the patient and their family in the decision-making process. We aimed to examine the ALS patient/family relationship in the decision-making process and to ascertain how patients and their family can shape one another's decisions pertaining to care. METHODS: We conducted a review of peer-reviewed empirical research, published in full and in English between January 2007 and January 2017, relating to care decision-making among ALS patients and their family...
August 11, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28795874/lack-of-association-between-the-p413l-variant-of-chromogranin-b-and-als-risk-or-age-at-onset-a-meta-analysis
#3
Xinglong Yang, Shimei Li, Dongmei Xing, Peiyun Li, Ci Li, Ling Qi, Yanming Xu, Hui Ren
BACKGROUND: Amyotrophic lateral sclerosis (ALS), the most common motor neuron disease, is thought to result from interaction of genetic and environmental risk factors. Whether the potentially functional exonic P413L variant in the chromogranin B gene influences ALS risk and age at onset is controversial. METHOD: We meta-analysed or other studies assessing the association between the P413L variant and ALS risk or age at ALS onset indexed in Web of Science, PubMed, Embase, Chinese National Knowledge Infrastructure, Wanfang, and SinoMed databases...
August 10, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28766957/immunohistochemical-detection-of-c9orf72-protein-in-frontotemporal-lobar-degeneration-and-motor-neurone-disease-patterns-of-immunostaining-and-an-evaluation-of-commercial-antibodies
#4
Yvonne S Davidson, Andrew C Robinson, Sara Rollinson, Stuart Pickering-Brown, Shangxi Xiao, Janice Robertson, David M A Mann
We have employed as 'gold standards' two in-house, well-characterised and validated polyclonal antibodies, C9-L and C9-S, which detect the longer and shorter forms of C9orf72, and have compared seven other commercially available antibodies with these in order to evaluate the utility of the latter as credible tools for the demonstration of C9orf72. C9-L and C9-S antibodies immunostained cytoplasmic 'speckles', and the nuclear membrane, respectively, in cerebellar Purkinje cells of the cerebellum in patients with behavioural variant frontotemporal dementia (bvFTD) with amyotrophic lateral sclerosis (ALS), and in patients with ALS alone...
August 2, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28762856/exploring-the-diagnosis-delay-and-als-functional-impairment-at-diagnosis-as-relevant-criteria-for-clinical-trial-enrolment
#5
Bello Hamidou, Benoit Marin, Geraldine Lautrette, Marie Nicol, William Camu, Philippe Corcia, Marie-Christine Arnes-Bes, Christine Tranchant, Pierre Clavelou, Didier Hannequin, Giroud Maurice, Katell Beauvais, Jean-Christophe Antoine, Véronique Danel-Brunaud, Fausto Viader, Pierre-Marie Preux, Philippe Couratier
Objectives were: i) to describe the phenotypic heterogeneity of incident amyotrophic lateral sclerosis (ALS) patients diagnosed in 2012 in French ALS centres; ii) to look at the associations between ALSFRS-R score and ALSFRS-R slope (ΔFS) at time of diagnosis with diagnosis delay, ALS phenotypes and Airlie House diagnosis criteria (AHDC); iii) to describe the rate of progression on ΔFS, according to diagnosis delay. METHODS: Incident ALS cases diagnosed in French ALS centres were included...
August 1, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28745069/a-case-series-of-pls-patients-with-frontotemporal-dementia-and-overview-of-the-literature
#6
Bálint S de Vries, Laura M M Rustemeijer, Anneke J van der Kooi, Joost Raaphorst, Carin D Schröder, Tanja C W Nijboer, Jeroen Hendrikse, Jan H Veldink, Leonard H van den Berg, Michael A van Es
OBJECTIVE: Primary lateral sclerosis (PLS) is a rare form of motor neuron disease characterised by UMN degeneration leading to slowly progressive spasticity. Whether it is a separate disease or a subtype of ALS has been debated. In ALS comorbid frontotemporal dementia (FTD) is frequently seen (±15%). However, cognitive and behavioural changes are generally not considered to be a part of PLS. METHODS: To report the clinical findings and frequency of PLS patients that developed FTD in a referral-based cohort and provide an overview of the literature...
July 26, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28741375/comparison-of-slow-and-forced-vital-capacities-on-ability-to-predict-survival-in-als
#7
Susana Pinto, Mamede de Carvalho
INTRODUCTION: Slow (SVC) and forced (FVC) vital capacities are the most used pulmonary function tests in amyotrophic lateral sclerosis (ALS). It is unknown if they equally predict survival in ALS. The aim of the present study was to compare both measures in predicting survival in this disease. METHODS: Consecutive definite/probable ALS patients (2000-2014) in whom respiratory tests were performed at baseline and four months later were included. All patients were evaluated with the revised ALS functional rating scale (ALSFRS-R), respiratory (RofALSFRS-R), bulbar (ALSFRSb), upper and lower limb subscores, SVC, FVC, maximal inspiratory (MIP) and expiratory (MEP) pressures...
July 25, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28728441/ability-of-pulmonary-function-decline-to-predict-death-in-amyotrophic-lateral-sclerosis-patients
#8
Irina Enache, Cristina Pistea, Marie Fleury, Mickael Schaeffer, Monique Oswald-Mammosser, Andoni Echaniz-Laguna, Christine Tranchant, Nicolas Meyer, Anne Charloux
OBJECTIVES: Objectives were to evaluate the relative risk of death associated with lung function decline in patients with amyotrophic lateral sclerosis (ALS), and to examine the ability of ALS patients to perform volitional pulmonary function tests (PFTs). METHODS: The PFTs of 256 consecutive patients referred to the Strasbourg University Hospital ALS Centre over an eight-year period were reviewed. Slow vital capacity (VC), maximal inspiratory and expiratory pressures (MIP, MEP), sniff nasal inspiratory pressure (SNIP), and peak cough flow (PCF) were performed at diagnosis and then every four months...
July 20, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28720005/alsuntangled-41-eric-is-winning
#9
(no author information available yet)
No abstract text is available yet for this article.
July 18, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28719997/the-benefit-of-evolving-multidisciplinary-care-in-als-a-diagnostic-cohort-survival-comparison
#10
Sarah Martin, Emma Trevor-Jones, Sabyha Khan, Keelan Shaw, Deepti Marchment, Anna Kulka, Catherine E Ellis, Rachel Burman, Martin R Turner, Liam Carroll, Leah Mursaleen, P Nigel Leigh, Christopher E Shaw, Neil Pearce, Daniel Stahl, Ammar Al-Chalabi
BACKGROUND: Care for people with amyotrophic lateral sclerosis (ALS) has altered at King's College Hospital over the last 20 years. The clinic has been a multidisciplinary, specialist, tertiary referral centre since 1995 with a large team with integrated palliative and respiratory care since 2006. We hypothesised that these changes would improve survival. METHODS: In this retrospective observational study, patients diagnosed with El Escorial definite, probable and possible ALS between 1995-1998 and 2008-2011 were followed up...
July 18, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28705085/international-survey-of-als-experts-about-critical-questions-for-assessing-patients-with-als
#11
Mamede De Carvalho, Adam Ryczkowski, Peter Andersen, Marta Gromicho, Julian Grosskreutz, Magdalena Kuźma-Kozakiewicz, Susanne Petri, Maria Piotrkiewicz, Gabriel Miltenberger Miltenyi
OBJECTIVE: To define an applicable dataset for ALS patient registries we weighted specific clinical items as scored by worldwide ALS experts. METHODS: Sixty participants were invited based on relevant clinical work, publications and personal acquaintance. They rated 160 clinical items consensually agreed by the members of our project, incorporating specialists from five European Centres. Scoring scheme was defined as: 1 - essential; 2 - important; 3 - not very important...
July 13, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28705014/mutation-in-the-rrm2-domain-of-tdp-43-in-amyotrophic-lateral-sclerosis-with-rapid-progression-associated-with-ubiquitin-positive-aggregates-in-cultured-motor-neurons
#12
Cindy Maurel, Blandine Madji-Hounoum, Rose-Anne Thepault, Sylviane Marouillat, Céline Brulard, Véronique Danel-Brunaud, Jean-Philippe Camdessanche, Helene Blasco, Philippe Corcia, Christian R Andres, Patrick Vourc'h
Mutations in the TAR-DNA Binding Protein-43 (TDP-43) encoding the TARDBP gene are present in amyotrophic lateral sclerosis (ALS). TDP-43 is the major component of ubiquitin-positive inclusions in motor neurons in ALS patients. We report here a novel heterozygous missense mutation in TARDBP in an ALS patient presenting a rapid form of ALS. This mutation p.N259S is located within the RNA recognition motif 2 (RRM2) in very close proximity with nucleotides in RNA. It is the first time a mutation was reported in this RRM2 domain of TDP-43...
July 13, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28678542/incorporation-of-telehealth-into-a-multidisciplinary-als-clinic-feasibility-and-acceptability
#13
Andrew Geronimo, Courtney Wright, Anne Morris, Susan Walsh, Bethany Snyder, Zachary Simmons
OBJECTIVE: The practice of telehealth in the care of patients with ALS has received little attention, but has the potential to change the multidisciplinary care model. This study was carried out to assess the feasibility and acceptability of telehealth for ALS care via real-time videoconferencing from the clinic to patients' homes. METHODS: Patients and caregivers engaged in live telehealth videoconferencing from their homes with members of a multidisciplinary ALS care team who were located in an ALS clinic, in place of their usual in-person visit to the clinic...
July 5, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28664756/late-onset-bipolar-disorder-and-frontotemporal-dementia-with-mutation-in-progranulin-gene-a-case-report
#14
Elisa Rubino, Alessandro Vacca, Salvatore Gallone, Flora Govone, Milena Zucca, Annalisa Gai, Patrizia Ferrero, Pierpaola Fenoglio, Maria Teresa Giordana, Innocenzo Rainero
Bipolar disorder is a chronic psychiatric illness characterised by fluctuation in mood state, with a relapsing and remitting course. Frontotemporal dementia (FTD) is a clinically and genetically heterogeneous syndrome, with the most frequent phenotype being behavioural variant frontotemporal dementia (bvFTD). Here, we report the case of an Italian male presenting with late-onset bipolar disorder that developed into bvFTD over time, carrying a mutation in the GRN gene. Interestingly, the patient carried the c...
June 30, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28651439/erratum
#15
(no author information available yet)
No abstract text is available yet for this article.
June 26, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28618842/alsuntangled-40-ayahuasca
#16
(no author information available yet)
No abstract text is available yet for this article.
June 15, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28616937/influence-of-arterial-hypertension-type-2-diabetes-and-cardiovascular-risk-factors-on-als-outcome-a-population-based-study
#17
Cristina Moglia, Andrea Calvo, Antonio Canosa, Davide Bertuzzo, Paolo Cugnasco, Luca Solero, Maurizio Grassano, Enrica Bersano, Stefania Cammarosano, Umberto Manera, Fabrizio Pisano, Letizia Mazzini, Laura A Dalla Vecchia, Gabriele Mora, Adriano Chiò
OBJECTIVE: To assess the prognostic influence of pre-morbid type 2 diabetes mellitus, arterial hypertension and cardiovascular (CV) risk profile on ALS phenotype and outcome in a population-based cohort of Italian patients. METHODS: A total of 650 ALS patients from the Piemonte/Valle d'Aosta Register for ALS, incident in the 2007-2011 period, were recruited. Information about premorbid presence of type 2 diabetes mellitus, arterial hypertension was collected at the time of diagnosis...
June 15, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28616933/split-hand-index-and-ulnar-to-median-ratio-in-hirayama-disease-and-amyotrophic-lateral-sclerosis
#18
Jayantee Kalita, Surendra Kumar, Usha K Misra, Zafar Neyaz
OBJECTIVE: We report the relative usefulness of split hand index (SHI) and ulnar to median (UM) ratio in Hirayama disease (HD) and amyotrophic lateral sclerosis (ALS). METHODS: The patients with HD, ALS and matched controls were subjected to evaluation of UM ratio and SHI. Compound muscle action potentials (CMAPs) were recorded from abductor digit minimi (ADM) and first dorsal interosseous (FDI) by stimulating ulnar nerve at the wrist, and abductor pollicis brevis (APB) by stimulating median nerve at the wrist...
June 15, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28597697/effectiveness-of-assisted-and-unassisted-cough-capacity-in-amyotrophic-lateral-sclerosis-patients
#19
Jesús Sancho, Emilio Servera, Pilar Bañuls, Julio Marín
Decreased cough capacity during a respiratory infection is one of the main causes of acute respiratory failure and hospitalisation in amyotrophic lateral sclerosis (ALS). OBJECTIVE: To determine whether a respiratory measurement could identify the effectiveness of cough capacity in ALS during a respiratory infection. METHODS: This was a prospective study of all ALS patients who were treated at a respiratory care unit due to a respiratory infection from 2012 to 2016...
June 9, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28593798/nocebo-in-motor-neuron-disease-systematic-review-and-meta-analysis-of-placebo-controlled-clinical-trials
#20
Faiza Shafiq, Dimos-Dimitrios Mitsikostas, Panagiotis Zis
INTRODUCTION: Nocebo is very prevalent among neurological disorders, resulting in low adherence and treatment outcome. We sought to examine the AEs following placebo administration in placebo-controlled randomised clinical trials (RCTs) for motor neuron disease (MND). METHODS: After a systematic literature search for RCTs for MND pharmacotherapy treatments, we assessed the number of discontinuations because of placebo intolerance. RESULTS: Data were extracted from 12 RCTs fulfilling our search criteria...
June 8, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
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