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Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

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https://www.readbyqxmd.com/read/29160128/hospital-utilization-for-patients-with-amyotrophic-lateral-sclerosis-in-saskatoon-canada
#1
Adrianna Gunton, Gregory Hansen, Kerri Lynn Schellenberg
OBJECTIVE: This retrospective study reviewed hospital and intensive care unit (ICU) admissions for patients with amyotrophic lateral sclerosis (ALS) in Saskatoon, Canada, between 2005 and 2017. The purpose was to understand hospital utilization and admission patterns for patients with ALS in the absence of coordinated multidisciplinary care. METHODS: Hospital/ICU admissions were detected at two hospitals in Saskatoon using the International Classification of Diseases (ICD-10) coding for ALS...
November 21, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29092641/revisiting-the-pathoanatomy-of-pseudobulbar-affect-mechanisms-beyond-corticobulbar-dysfunction
#2
Peter Bede, Eoin Finegan
No abstract text is available yet for this article.
November 2, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29092645/usability-of-eyetracking-computer-systems-and-impact-on-psychological-wellbeing-in-patients-with-advanced-amyotrophic-lateral-sclerosis
#3
Katharina Linse, Wolfgang Rüger, Markus Joos, Henning Schmitz-Peiffer, Alexander Storch, Andreas Hermann
Restrictions in communicative abilities are well known in patients with amyotrophic lateral sclerosis (ALS), but only few approaches in terms of evaluation of supportive technologies have been made. We aimed to assess the use and perceived usability of eye-tracking computer devices (ETCS) of severely impacted patients with ALS in an independent, direct manner and relate it to psychological well-being. ETCS enable active communication and social participation in the quadriplegic and anarthric disease state. Therefore, ETCS-based versions of widely used psychosocial questionnaires (ADI-12, SeiQoL-DW, WHO-5) as well as structured questions on communicative functioning and ETCS usage were developed to assess ALS patients, their next of kin and professional caregivers...
November 1, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29063790/trauma-and-amyotrophic-lateral-sclerosis-a-european-population-based-case-control-study-from-the-eurals-consortium
#4
Elisabetta Pupillo, Marco Poloni, Elisa Bianchi, Giorgia Giussani, Giancarlo Logroscino, Stefano Zoccolella, Adriano Chiò, Andrea Calvo, Massimo Corbo, Christian Lunetta, Benoit Marin, Douglas Mitchell, Orla Hardiman, James Rooney, Zorica Stevic, Monica Bandettini di Poggio, Massimiliano Filosto, Maria Sofia Cotelli, Michele Perini, Nilo Riva, Lucio Tremolizzo, Eugenio Vitelli, Danira Damiani, Ettore Beghi
OBJECTIVES: To assess the association between amyotrophic lateral sclerosis (ALS) and previous traumatic events, age of trauma, and site of injury. METHODS: A population-based case-control study was performed in five European countries (Italy, Ireland, France, United Kingdom, Serbia). Newly diagnosed ALS patients and matched controls were interviewed to collect relevant demographic factors and exposures. Key clinical features at diagnosis were collected in ALS patients...
October 24, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29037069/factor-analysis-of-the-zarit-burden-interview-in-family-caregivers-of-patients-with-amyotrophic-lateral-sclerosis
#5
Juyeon Oh, Jung A Kim
OBJECTIVE: The Zarit Burden Interview has been used in many studies to assess caregiver burden in family caregivers of patients with amyotrophic lateral sclerosis, but the factor structure of the Zarit Burden Interview in the caregivers of amyotrophic lateral sclerosis patients is unknown. The aim of this study was to explore the factor structure of the Zarit Burden Interview in family caregivers of amyotrophic lateral sclerosis patients using exploratory factor analysis. METHODS: The exploratory factor analysis was performed using generalized least squares with oblique rotation in a sample of 202 family caregivers...
October 16, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29037065/patterns-of-symptom-development-in-patients-with-motor-neuron-disease
#6
Renée Walhout, Esther Verstraete, Martijn P Van Den Heuvel, Jan H Veldink, Leonard H Van Den Berg
OBJECTIVE: To investigate whether symptom development in motor neuron disease (MND) is a random or organized process. METHODS: Six hundred patients with amyotrophic lateral sclerosis (ALS), upper motor neuron (UMN) or lower motor neuron (LMN) phenotypes were invited for a questionnaire concerning symptom development. A binomial test was used to examine distribution of symptoms from site of onset. Development of symptoms over time was evaluated by Kaplan-Meier analysis...
October 16, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29034720/investigating-the-neuroanatomical-substrate-of-pathological-laughing-and-crying-in-amyotrophic-lateral-sclerosis-with-multimodal-neuroimaging-techniques
#7
Foteini Christidi, Efstratios Karavasilis, Panagiotis Ferentinos, Sophia Xirou, Georgios Velonakis, Michalis Rentzos, Vasiliki Zouvelou, Ioannis Zalonis, Efstathios Efstathopoulos, Nikolaos Kelekis, Ioannis Evdokimidis
OBJECTIVE: Pathological laughing and crying (PLC) is common in several neurological and psychiatric diseases and is associated with a distributed network involving the frontal cortex, the brainstem and cortico-pontine-cerebellar circuits. By applying multimodal neuroimaging approach, we examined the neuroanatomical substrate of PLC in a sample of patients with amyotrophic lateral sclerosis (ALS). METHODS: We studied 56 non-demented ALS patients and 25 healthy controls (HC)...
October 15, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29020837/evaluating-the-completeness-of-the-national-als-registry-united-states
#8
Wendy E Kaye, Laurie Wagner, Ruoming Wu, Paul Mehta
Our objective was to evaluate the completeness of the United States National ALS Registry (Registry). We compared persons with ALS who were passively identified by the Registry with those actively identified in the State and Metropolitan Area ALS Surveillance project. Cases in the two projects were matched using a combination of identifiers, including, partial social security number, name, date of birth, and sex. The distributions of cases from the two projects that matched/did not match were compared and Chi-square tests conducted to determine statistical significance...
October 11, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28990425/mendelian-forms-of-disease-and-age-at-onset-affect-survival-in-frontotemporal-dementia
#9
Maura Cosseddu, Alberto Benussi, Stefano Gazzina, Rosanna Turrone, Silvana Archetti, Elisa Bonomi, Giorgio Biasiotto, Isabella Zanella, Raffaele Ferrari, Maria S Cotelli, Antonella Alberici, Alessandro Padovani, Barbara Borroni
OBJECTIVE: Frontotemporal dementia (FTD) is a common cause of young onset dementia. Very few reports on disease duration are currently available and predictors of survival are still undefined. The aim of the present study was to assess the natural history of FTD and to define predictors of survival. METHODS: Four hundred amd eleven FTD patients, including 294 with behavioural variant FTD, 77 with agrammatic variant primary progressive aphasia (PPA) and 40 with semantic variant PPA, were consecutively enrolled and demographic and clinical variables carefully recorded...
October 8, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28980493/the-use-of-a-hand-held-ventilator-to-supplement-niv-for-patients-with-als-mnd-with-respiratory-insufficiency
#10
David Oliver, Sandip Banerjee, Lisa Vincent-Smith, Jane Kindred, Katharine Martin
No abstract text is available yet for this article.
October 5, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28980485/sympathetic-vascular-response-to-facial-cooling-is-increased-in-flail-phenotypes-of-amyotrophic-lateral-sclerosis
#11
Marcin Tutaj, MaŁgorzata Miller, Barbara Tomik, Aleksandra Golenia, Agnieszka Stanuszek, Katarzyna BŁońska, Agnieszka SŁowik
OBJECTIVE: To assess cardiovascular responses to cold face test (CFT) in patients with classic-onset ALS (bulbar or limb onset, ALS-C) and in patients with flail arm and flail leg phenotypes (FA/FL). METHODS: In 18 ALS-C, eight FA/FL patients and 10 age-matched controls we continuously monitored heart rate (HR), systolic (SBP), diastolic (DBP) and mean blood pressure (MBP) during two-minute baseline and one-minute cold stimulus application. HR and BP responses to CFT were calculated as differences between the peak responses and baseline values (dHR, dSBP, dDBP, dMBP), as percent changes from baseline (dHR%, dSBP%, dDBP%, dMBP%), and also latencies and durations of HR and BP responses were assessed (LatHR, tHR, LatBP, tBP)...
October 5, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28938856/intrafamilial-phenotypic-variations-in-familial-cases-of-cervical-flexion-induced-myelopathy-hirayama-disease
#12
Kiran Polavarapu, Veeramani Preethish-Kumar, Saraswati Nashi, Seena Vengalil, Chandrajit Prasad, Kajari Bhattacharya, Abha Verma, Nupur Pruthi, Dhananjay I Bhat, Atchayaram Nalini
Hirayama disease is generally considered to be a sporadic disorder, except for a few reports of familial occurrence. In this study, we describe eight patients from four families with cervical flexion induced myelopathy (CFIM)/Hirayama disease (HD) and intra-familial phenotypic variations. All underwent clinical and electrophysiological evaluation, while seven of them had contrast MR imaging of cervical spine in flexion. There was significant intra-familial variability: distal bimelic form in four patients, classical monomelic form in three and proximo-distal form in one...
September 22, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28931329/cramps-and-vinpocetine-in-als
#13
Mamede de Carvalho
No abstract text is available yet for this article.
September 21, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28920459/alsuntangled-42-elysium-health-s-basis
#14
Richard Bedlack
No abstract text is available yet for this article.
September 18, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28891333/profile-of-medical-care-costs-in-patients-with-amyotrophic-lateral-sclerosis-in-the-medicare-programme-and-under-commercial-insurance
#15
Lisa Meng, Amy Bian, Scott Jordan, Andrew Wolff, Jeremy M Shefner, Jinsy Andrews
OBJECTIVE: To determine amyotrophic lateral sclerosis (ALS)-associated costs incurred by patients covered by Medicare and/or commercial insurance before, during and after diagnosis and provide cost details. METHODS: Costs were calculated from the Medicare Standard Analytical File 5% sample claims data from Parts A and B from 2009, 2010 and 2011 for ALS Medicare patients aged ≥70 years (monthly costs) and ≥65 years (costs associated with disability milestones)...
September 11, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28869393/ethics-of-clinical-research-in-patients-with-als-is-there-a-risk-of-exploitation
#16
Carmel Armon
Amyotrophic lateral sclerosis is the most common degenerative disease of the motor neuron network. Due to its inevitably fatal outcome, numerous attempts have been made to ameliorate its course. The emergence of every new technology has spurred research to see if it might benefit patients with ALS (PALS). This paper suggests a detailed road map to be followed when performing clinical research in PALS to satisfy the ethics requirements of the Declaration of Helsinki, expressed using the terminology of Beauchamp and Childress...
September 4, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28859526/increase-in-dna-methylation-in-patients-with-amyotrophic-lateral-sclerosis-carriers-of-not-fully-penetrant-sod1-mutations
#17
Fabio Coppedè, Andrea Stoccoro, Lorena Mosca, Roberta Gallo, Claudia Tarlarini, Christian Lunetta, Alessandro Marocchi, Lucia Migliore, Silvana Penco
OBJECTIVE: More than 180 different superoxide dismutase 1 (SOD1) mutations have been described to date in amyotrophic lateral sclerosis (ALS) patients, including not completely penetrant ones leading to phenotypic heterogeneity among carriers. We collected DNA samples from five ALS families with not fully penetrant SOD1 mutations (p.Asn65Ser, p.Gly72Ser, p.Gly93Asp, and p.Gly130_Glu133del) searching for epigenetic differences among ALS patients, asymptomatic/paucisymptomatic carriers and non-carrier family members...
September 1, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29113492/theme-7-electrophysiology
#18
(no author information available yet)
No abstract text is available yet for this article.
November 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29113491/poster-contents-page
#19
(no author information available yet)
No abstract text is available yet for this article.
November 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29113490/theme-13-respiratory-and-nutritional-management
#20
(no author information available yet)
No abstract text is available yet for this article.
November 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
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