739 results match your criteria Amyotrophic Lateral Sclerosis And Frontotemporal Degeneration[Journal]


Post-hoc analyses of the edaravone clinical trials Study 16 and Study 19: a step toward more efficient clinical trial designs in amyotrophic lateral sclerosis.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Apr 13:1-11. Epub 2019 Apr 13.

c Mitsubishi Tanabe Pharma America , Jersey City , NJ , USA , and.

Objectives: The edaravone development program established a study design in which a treatment effect slowing functional loss in amyotrophic lateral sclerosis (ALS) could be documented within a 24-week time frame. This report elucidates the strategic enrichment design utilized to create efficiency and precision in the development program.

Methods: Post-hoc analyses describe learning, sequential iteration, and evolution in study design. Read More

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http://dx.doi.org/10.1080/21678421.2019.1599955DOI Listing

Plasma creatinine and amyotrophic lateral sclerosis prognosis: a systematic review and meta-analysis.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Apr 9:1-8. Epub 2019 Apr 9.

a UMR 1253, Team 2, INSERM/University of Tours , Tours , France and.

Background: Plasma creatinine has been described as a prognostic biomarker for Amyotrophic Lateral Sclerosis (ALS), but with conflicting results in the literature. We performed a systematic review followed by a meta-analysis to address this question.

Methods: We performed a systematic review of Pubmed, Embase and Cochrane databases and retrieved 14 distinct cohorts (19 studies) reporting results regarding the relationship between plasma creatinine and a clinical marker for ALS progression, notably ALSFRS (ALS Functional Rating Scale) and survival. Read More

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http://dx.doi.org/10.1080/21678421.2019.1572192DOI Listing
April 2019
1 Read

Predicting the future of ALS: the impact of demographic change and potential new treatments on the prevalence of ALS in the United Kingdom, 2020-2116.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Apr 9:1-11. Epub 2019 Apr 9.

a Department of Basic and Clinical Neuroscience , King's College London, Maurice Wohl Clinical Neuroscience Institute , London , UK.

Objective: To model the effects of demographic change under various scenarios of possible future treatment developments in ALS.

Methods: Patients diagnosed with ALS at the King's College Hospital Motor Nerve Clinic between 2004 and 2017, and living within the London boroughs of Lambeth, Southwark, and Lewisham (LSL), were included as incident cases. We also ascertained incident cases from the Canterbury region over the same period. Read More

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http://dx.doi.org/10.1080/21678421.2019.1587629DOI Listing
April 2019
4 Reads

Evaluation of remote pulmonary function testing in motor neuron disease.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Apr 7:1-8. Epub 2019 Apr 7.

b Department of Neurology , Penn State College of Medicine , Hershey , PA , USA and.

Introduction: Motor neuron disease (MND) causes respiratory insufficiency, which is managed in part through use of noninvasive ventilation (NIV). Guidelines for the initiation of NIV are based on pulmonary function tests (PFTs), usually performed once every three months. In the setting of MND telemedicine, remote monitoring of respiratory health may permit earlier intervention, but proof of equivalence to conventional PFTs is lacking. Read More

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https://www.tandfonline.com/doi/full/10.1080/21678421.2019.1
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http://dx.doi.org/10.1080/21678421.2019.1587633DOI Listing
April 2019
4 Reads

ALSUntangled no. 49: resveratrol.

Authors:

Amyotroph Lateral Scler Frontotemporal Degener 2019 Apr 4:1-6. Epub 2019 Apr 4.

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http://dx.doi.org/10.1080/21678421.2019.1593596DOI Listing

Ferritin and LDL-cholesterol as biomarkers of fat-free mass loss in ALS.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Apr 3:1-4. Epub 2019 Apr 3.

c Laboratory of Biochemistry and Molecular Biology , University Hospital of Tours , Tours , France.

The availability of longitudinal clinical and biological data led us to wonder whether these parameters could be used to predict disturbances in body composition during ALS progression. Bioelectrical impedance analysis (BIA), as well as clinical and biological parameters (blood lipids and ferritin), were collected one year after diagnosis in ALS patients. The correlations were evaluated by the Spearman test. Read More

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https://www.tandfonline.com/doi/full/10.1080/21678421.2019.1
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http://dx.doi.org/10.1080/21678421.2019.1597126DOI Listing
April 2019
2 Reads

Amyotrophic lateral sclerosis mortality rates among ethnic groups in a predominant admixed population in Latin America: a population-based study in Ecuador.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Apr 2:1-9. Epub 2019 Apr 2.

a INSERM, U1094, Tropical Neuroepidemiology , Limoges , France.

Current evidence suggests heterogeneity of amyotrophic lateral sclerosis (ALS) among geographic areas and populations. Lower mortality rates have been reported in admixed populations compared to European origin populations. We aimed to describe and compare ALS mortality rates among ethnic groups using a population-based approach in a multiethnic country. Read More

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http://dx.doi.org/10.1080/21678421.2019.1587632DOI Listing
April 2019
2 Reads

Telomere length is greater in ALS than in controls: a whole genome sequencing study.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Apr 1:1-6. Epub 2019 Apr 1.

a Department of Basic and Clinical Neuroscience , King's College London, Maurice Wohl Clinical Neuroscience Institute , London , UK.

Background: Amyotrophic lateral sclerosis is a neurodegenerative disease of motor neurons resulting in progressive paralysis and death, typically within 3-5 years. Although the heritability of ALS is about 60%, only about 11% is explained by common gene variants, suggesting that other forms of genetic variation are important. Telomeres maintain DNA integrity during cellular replication and shorten naturally with age. Read More

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http://dx.doi.org/10.1080/21678421.2019.1586951DOI Listing
April 2019
4 Reads

Systematic review of the prognostic role of body mass index in amyotrophic lateral sclerosis.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Apr 1:1-12. Epub 2019 Apr 1.

a Department of Neurology , West China Hospital, Sichuan University , Chengdu , Sichuan Province , P.R. China.

Studies have suggested that obesity is associated with better prognosis among individuals with various types of neurodegenerative diseases, and while some studies suggest that the same is true of amyotrophic lateral sclerosis (ALS), other works cast doubt on this conclusion. Therefore, we conducted a meta-analysis to systematically evaluate the role of body mass index in the prognosis of ALS. PubMed was systematically searched to identify eligible articles, and data on long-term survival were meta-analyzed in terms of hazard ratios (HR) with corresponding 95% confidence intervals (CI). Read More

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http://dx.doi.org/10.1080/21678421.2019.1587631DOI Listing
April 2019
2 Reads

Lack of consensus in ALS genetic testing practices and divergent views between ALS clinicians and patients.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Apr 1:1-6. Epub 2019 Apr 1.

a Division of Human Genetics , The Ohio State University Medical Center , Columbus , OH , USA.

Recent advances in ALS gene discovery have both empowered and challenged clinicians providing evaluation and care for persons with ALS, many of whom seek an answer as to the cause of their condition. In order to study clinician practices and attitudes towards genetic testing, we surveyed members of the Northeast ALS Consortium, an international group of specialist ALS clinicians; responses were received from 80 of 255 (response rate = 31.4%). Read More

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http://dx.doi.org/10.1080/21678421.2019.1582670DOI Listing
April 2019
1 Read

Incorporating self-reported questions for telemonitoring to optimize care of patients with MND on noninvasive ventilation (MND OptNIVent).

Amyotroph Lateral Scler Frontotemporal Degener 2019 Mar 26:1-12. Epub 2019 Mar 26.

e Chest Centre, Aintree University Hospitals NHS Foundation Trust , Liverpool , UK , and.

Objective: Previous studies suggest a positive impact of telehealth in the care of people with motor neuron disease/amyotrophic lateral sclerosis (MND/ALS). This study reports the development of self-reported questions for telemonitoring, using a tablet-based device Careportal, in the care of patients with MND on noninvasive ventilation (NIV) and its initial impact.

Methods: The study consisted of a question development phase and an evaluation phase of the use of Careportal. Read More

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http://dx.doi.org/10.1080/21678421.2019.1587630DOI Listing
March 2019
2 Reads

The C9orf72 hexanucleotide repeat expansion presents a challenge for testing laboratories and genetic counseling.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Mar 23:1-7. Epub 2019 Mar 23.

a Department of Clinical Medicine, Faculty of Medicine and Health Sciences , Macquarie University , Sydney , Australia.

C9orf72 hexanucleotide repeat expansions are the most common known cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Genetic testing for C9orf72 expansions in patients with ALS and/or FTD and their relatives has become increasingly available since hexanucleotide repeat expansions were first reported in 2011. The repeat number is highly variable and the threshold at which repeat size leads to neurodegeneration remains unknown. Read More

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http://dx.doi.org/10.1080/21678421.2019.1588904DOI Listing
March 2019
5 Reads

Amyotrophic lateral sclerosis among patients with a Medicare Advantage prescription drug plan; prevalence, survival and patient characteristics.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Mar 20:1-9. Epub 2019 Mar 20.

b Division of Toxicology and Human Health Sciences , Agency for Toxic Substances and Disease Registry/Centers for Disease Control and Prevention , Atlanta , GA , USA.

Objective: To estimate amyotrophic lateral sclerosis (ALS) prevalence, 5-year survival, and explore factors associated with survival in a Medicare population.

Methods: A validated administrative claims algorithm was used to classify individual's ages 18-89 years at index date (first claim with a diagnosis of motor neuron disease or ALS between 1 January 2007 and 31 December 2011) with Medicare Advantage prescription drug coverage into mutually exclusive categories: ALS, no ALS, and possible ALS. Crude prevalence and cumulative survival from index date to the date of death, disenrollment or end of the study were calculated. Read More

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https://www.tandfonline.com/doi/full/10.1080/21678421.2019.1
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http://dx.doi.org/10.1080/21678421.2019.1582674DOI Listing
March 2019
3 Reads

Defining pre-symptomatic amyotrophic lateral sclerosis.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Mar 20:1-7. Epub 2019 Mar 20.

a Department of Neurology , University of Miami , Miami , FL , USA and.

Successful treatment of neurodegenerative disease may hinge on early therapeutic intervention. This requires an understanding of early/pre-symptomatic disease, a need that is underscored by advances in antisense oligonucleotide, and viral-vector-based gene therapies. In amyotrophic lateral sclerosis (ALS), the study of pre-symptomatic disease requires a cohesive conceptual framework for describing this phase of disease. Read More

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http://dx.doi.org/10.1080/21678421.2019.1587634DOI Listing
March 2019
1 Read

Clinical audit research and evaluation of motor neuron disease (CARE-MND): a national electronic platform for prospective, longitudinal monitoring of MND in Scotland.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Mar 20:1-9. Epub 2019 Mar 20.

a Centre for Clinical Brain Sciences , University of Edinburgh , Edinburgh , UK.

Objectives: Launched in 1989, the Scottish Motor Neuron Disease Register (SMNDR) has provided a resource for prospective clinical data collection. However, in 2015 we aimed to evolve a system to allow: i) A patient-centered approach to care based on recognized standards, ii) Harmonized data sharing between Scottish health professionals in "real-time", iii) Regular audit of care to facilitate timely improvements in service delivery, and iv) Patient participation in a diverse range of observational and interventional research studies including clinical trials.

Methods: We developed a standardized national electronic data platform-Clinical Audit Research and Evaluation of MND (CARE-MND) which integrates clinical audit and research data fields. Read More

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http://dx.doi.org/10.1080/21678421.2019.1582673DOI Listing

Facial onset sensory and motor neuronopathy: a motor neuron disease with an oligogenic origin?

Amyotroph Lateral Scler Frontotemporal Degener 2019 Mar 20:1-4. Epub 2019 Mar 20.

a Instituto de Investigación Sanitaria la Fe (IIS La Fe) , Neuromuscular Research Unit , Valencia , Spain.

Objective: To describe a patient with facial onset sensory and motor neuronopathy (FOSMN) carrying heterozygous mutations in both TARDBP and SQSTM1 genes.

Methods: The patient underwent neurological, neuropsychological, and neurophysiological examinations. Brain magnetic resonance imaging (MRI) and extensive genetic analysis were also performed. Read More

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http://dx.doi.org/10.1080/21678421.2019.1582671DOI Listing
March 2019
4 Reads

Identification and functional analysis of novel mutations in the SOD1 gene in Chinese patients with amyotrophic lateral sclerosis.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Mar 19:1-7. Epub 2019 Mar 19.

a Department of Neurology and Institute of Neurology , First Affiliated Hospital Fujian Medical University , Fuzhou , China.

Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease characterized by selective involvement of motor neurons in the central nervous system (CNS). The most common causative gene of ALS in the Chinese population is the Cu/Zn superoxide dismutase 1 (SOD1) gene, which accounts for 20-42.9% of familial ALS (FALS) and 1-2% of sporadic ALS (SALS) cases. Read More

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http://dx.doi.org/10.1080/21678421.2019.1582668DOI Listing
March 2019
1 Read

FUS-ALS presenting with myoclonic jerks in a 17-year-old man.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Mar 18:1-3. Epub 2019 Mar 18.

a Department of Neurology , Greater Manchester Neurosciences Centre, Salford Royal Foundation Trust , Salford , UK.

Fused in sarcoma-related amyotrophic lateral sclerosis (FUS-ALS) accounts for 4% of all familial motor neurone disease, but has a much higher incidence in juvenile ALS. We present a case of a 17-year-old British man with rapidly progressive bulbar and respiratory failure. On examination he had weak periocular muscles, neck flexion weakness, and a wasted, fasciculating and weak tongue. Read More

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http://dx.doi.org/10.1080/21678421.2019.1582665DOI Listing

ALSgeneScanner: a pipeline for the analysis and interpretation of DNA sequencing data of ALS patients.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Mar 5:1-9. Epub 2019 Mar 5.

c UK Dementia Research Institute, King's College London , London , UK.

Amyotrophic lateral sclerosis (ALS, MND) is a neurodegenerative disease of upper and lower motor neurons resulting in death from neuromuscular respiratory failure, typically within two years of first symptoms. Genetic factors are an important cause of ALS, with variants in more than 25 genes having strong evidence, and weaker evidence available for variants in more than 120 genes. With the increasing availability of next-generation sequencing data, non-specialists, including health care professionals and patients, are obtaining their genomic information without a corresponding ability to analyze and interpret it. Read More

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http://dx.doi.org/10.1080/21678421.2018.1562553DOI Listing
March 2019
4 Reads
2.591 Impact Factor

Improved survival and 30-day mortality after gastrostomy in Scottish motor neurone disease patients: evidence from a national retrospective cohort study using STROBE criteria.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Mar 5:1-7. Epub 2019 Mar 5.

e 5 Department of Neurology , Ealing Hospital , Southall, UK.

Objectives: Defining historical changes and outcomes in the use of gastrostomy in the management of Scottish MND patients.

Methods: The 1989-1998 and 2015-2016 Scottish national MND cohorts were used to examine the frequency, timing, and survival related to gastrostomy. The cohorts were censored for survival analysis. Read More

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http://dx.doi.org/10.1080/21678421.2019.1570271DOI Listing
March 2019
2 Reads

Excitability in somatosensory cortex correlates with motoric impairment in amyotrophic lateral sclerosis.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Feb 23:1-7. Epub 2019 Feb 23.

a Department of Neurology, Medical Faculty , BG University Hospital Bergmannsheil, Ruhr University Bochum , Bochum , Germany and.

Objective: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative motoneuron disease. As previous studies reported alterations in motor cortex excitability, we evaluate excitability changes in somatosensory system.

Methods: We examined 15 ALS patients and 15 healthy controls. Read More

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https://www.tandfonline.com/doi/full/10.1080/21678421.2019.1
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http://dx.doi.org/10.1080/21678421.2019.1570270DOI Listing
February 2019
5 Reads

ALSUntangled 48: Perampanel (Fycompa).

Authors:
Rick Bedlack

Amyotroph Lateral Scler Frontotemporal Degener 2019 Feb 20:1-4. Epub 2019 Feb 20.

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http://dx.doi.org/10.1080/21678421.2019.1573850DOI Listing
February 2019

Early post-marketing experience with edaravone in an unselected group of patients with ALS.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Feb 20:1-4. Epub 2019 Feb 20.

a Department of Neurology , Neuromuscular Diseases Unit, Tel Aviv Sourasky Medical Center , Tel Aviv , Israel.

Introduction: Treatment with edaravone has shown efficacy in a subgroup of patients with amyotrophic lateral sclerosis (ALS). However, it has been estimated that <7% of ALS patients fulfill the stringent inclusion criteria of the trial. In the current study, we aimed to explore retrospectively the efficacy of edaravone in unselected ALS patients. Read More

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https://www.tandfonline.com/doi/full/10.1080/21678421.2019.1
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http://dx.doi.org/10.1080/21678421.2019.1572191DOI Listing
February 2019
10 Reads

Clinical disease stage related changes of serological factors in amyotrophic lateral sclerosis.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Feb 20:1-8. Epub 2019 Feb 20.

a Department of Neurology , West China Hospital, Sichuan University , Chengdu , Sichuan , China.

Objective: Little is known whether disease clinical stage would influence the serological values in Amyotrophic lateral sclerosis (ALS). We aimed to explore the association between the levels of serological factors with clinical progression determined by the King's College staging system.

Methods: ALS Patients were registered from May 2008 to December 2016. Read More

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http://dx.doi.org/10.1080/21678421.2018.1550516DOI Listing
February 2019
1 Read

A standard operating procedure for King's ALS clinical staging.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Feb 18:1-6. Epub 2019 Feb 18.

c Department of Basic and Clinical Neuroscience , Maurice Wohl Clinical Neuroscience Institute, King's College London , London , UK.

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. Read More

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http://dx.doi.org/10.1080/21678421.2018.1556696DOI Listing
February 2019
3 Reads

Correction.

Authors:

Amyotroph Lateral Scler Frontotemporal Degener 2019 Jan 29. Epub 2019 Jan 29.

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http://dx.doi.org/10.1080/21678421.2018.1540461DOI Listing
January 2019

ALSUntangled No. 47: RT001.

Authors:
Richard Bedlack

Amyotroph Lateral Scler Frontotemporal Degener 2019 Jan 28:1-4. Epub 2019 Jan 28.

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http://dx.doi.org/10.1080/21678421.2018.1549531DOI Listing
January 2019

The feasibility of using robotic technology to quantify sensory, motor, and cognitive impairments associated with ALS.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Jan 27:1-10. Epub 2019 Jan 27.

a Centre for Neuroscience Studies, Queen's University , Kingston , Canada.

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. Read More

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https://www.tandfonline.com/doi/full/10.1080/21678421.2018.1
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http://dx.doi.org/10.1080/21678421.2018.1550515DOI Listing
January 2019
12 Reads

Efficacy and tolerability of EH301 for amyotrophic lateral sclerosis: a randomized, double-blind, placebo-controlled human pilot study.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Jan 22:1-8. Epub 2019 Jan 22.

j Department of Physiology , University of Valencia , Valencia , Spain.

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. Read More

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https://www.tandfonline.com/doi/full/10.1080/21678421.2018.1
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http://dx.doi.org/10.1080/21678421.2018.1536152DOI Listing
January 2019
31 Reads

Primary progressive aphasia and the FTD-MND spectrum disorders: clinical, pathological, and neuroimaging correlates.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Jan 22:1-13. Epub 2019 Jan 22.

a Department of Neurology, Memory and Aging Center , University of California , San Francisco , CA , USA.

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. Read More

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http://dx.doi.org/10.1080/21678421.2018.1556695DOI Listing
January 2019
24 Reads

Lunasin does not slow ALS progression: results of an open-label, single-center, hybrid-virtual 12-month trial.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Jan 21:1-9. Epub 2019 Jan 21.

c NeuroEpigenetics Laboratory , MassGeneral Institute for Neurodegenerative Disease , Charlestown , MA , USA.

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. Read More

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https://www.tandfonline.com/doi/full/10.1080/21678421.2018.1
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http://dx.doi.org/10.1080/21678421.2018.1556698DOI Listing
January 2019
11 Reads

Cerebellar tract alterations in PLS and ALS.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Jan 21:1-4. Epub 2019 Jan 21.

b Wellcome Centre for Integrative Neuroimaging , University of Oxford , Oxford , UK.

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. Read More

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https://www.tandfonline.com/doi/full/10.1080/21678421.2018.1
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http://dx.doi.org/10.1080/21678421.2018.1562554DOI Listing
January 2019
12 Reads

Typical bulbar ALS can be linked to GARS mutation.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Jan 20:1-3. Epub 2019 Jan 20.

c Inserm Unit U1253, Tours University , Inserm , Tours , France and.

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). Read More

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https://www.tandfonline.com/doi/full/10.1080/21678421.2018.1
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http://dx.doi.org/10.1080/21678421.2018.1556699DOI Listing
January 2019
7 Reads

Predictors of diagnostic delay in amyotrophic lateral sclerosis: a cohort study based on administrative and electronic medical records data.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Jan 18:1-10. Epub 2019 Jan 18.

e Leibniz Institute for Prevention Research and Epidemiology-BIPS , Bremen , Germany.

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). Read More

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https://www.tandfonline.com/doi/full/10.1080/21678421.2018.1
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http://dx.doi.org/10.1080/21678421.2018.1550517DOI Listing
January 2019
10 Reads

Primary lateral sclerosis: a distinct entity or part of the ALS spectrum?

Amyotroph Lateral Scler Frontotemporal Degener 2019 Jan 18:1-13. Epub 2019 Jan 18.

a Computational Neuroimaging Group, Academic Unit of Neurology , Biomedical Sciences Institute, Trinity College , Dublin , Ireland.

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. Read More

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https://www.tandfonline.com/doi/full/10.1080/21678421.2018.1
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http://dx.doi.org/10.1080/21678421.2018.1550518DOI Listing
January 2019
13 Reads

Psychological distress and coping styles of caregivers of patients with amyotrophic lateral sclerosis: a longitudinal study.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Jan 18:1-7. Epub 2019 Jan 18.

a Center of Excellence for Rehabilitation Medicine , Brain Center Rudolf Magnus, University Medical Center Utrecht, Utrecht University and De Hoogstraat Rehabilitation , Utrecht , The Netherlands.

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. Read More

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https://www.tandfonline.com/doi/full/10.1080/21678421.2018.1
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http://dx.doi.org/10.1080/21678421.2018.1534976DOI Listing
January 2019
9 Reads

Elevated serum ferritin level as a predictor of reduced survival in patients with sporadic amyotrophic lateral sclerosis in China: a retrospective study.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Jan 17:1-6. Epub 2019 Jan 17.

a College of Medicine , Nankai University , Tianjin , China.

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. Read More

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https://www.tandfonline.com/doi/full/10.1080/21678421.2018.1
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http://dx.doi.org/10.1080/21678421.2018.1555599DOI Listing
January 2019
9 Reads
2.591 Impact Factor

Characteristics of impaired voluntary cough function in individuals with amyotrophic lateral sclerosis.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Jan 17:1-6. Epub 2019 Jan 17.

b Swallowing Systems Core , University of Florida , Gainesville , FL , USA.

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. Read More

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https://www.tandfonline.com/doi/full/10.1080/21678421.2018.1
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http://dx.doi.org/10.1080/21678421.2018.1510011DOI Listing
January 2019
7 Reads

Does executive functioning contribute to locomotion in amyotrophic lateral sclerosis patients?

Amyotroph Lateral Scler Frontotemporal Degener 2019 Jan 17:1-3. Epub 2019 Jan 17.

a Center for ALS and Related Disorders, Division of Neurology, Department of Clinical Neurosciences , Geneva University Hospitals , Geneva , Switzerland.

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. Read More

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https://www.tandfonline.com/doi/full/10.1080/21678421.2018.1
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http://dx.doi.org/10.1080/21678421.2018.1542536DOI Listing
January 2019
11 Reads

Screening for cognitive and behavioral change in amyotrophic lateral sclerosis/motor neuron disease: a systematic review of validated screening methods.

Amyotroph Lateral Scler Frontotemporal Degener 2018 Dec 26:1-11. Epub 2018 Dec 26.

b Department of Psychology, Institute of Psychiatry, Psychology and Neuroscience, King's College London , London , UK.

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. Read More

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http://dx.doi.org/10.1080/21678421.2018.1530264DOI Listing
December 2018
3 Reads

Moral judgment in patients with behavioral variant of frontotemporal dementia and amyotrophic lateral sclerosis: no impairment of the moral position, but rather its execution.

Amyotroph Lateral Scler Frontotemporal Degener 2018 Dec 4:1-7. Epub 2018 Dec 4.

a Department of Neurology , University of Ulm , Ulm , Germany and.

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. Read More

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https://www.tandfonline.com/doi/full/10.1080/21678421.2018.1
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http://dx.doi.org/10.1080/21678421.2018.1534972DOI Listing
December 2018
4 Reads

ALS longitudinal studies with frequent data collection at home: study design and baseline data.

Amyotroph Lateral Scler Frontotemporal Degener 2018 Nov 28:1-7. Epub 2018 Nov 28.

b Department of Neurology , Barrow Neurological Institute , Phoenix , AZ , USA.

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. Read More

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http://dx.doi.org/10.1080/21678421.2018.1541095DOI Listing
November 2018

Cognitive and behavioral impairments in German and Chinese ALS populations - a post-hoc comparison of national study data.

Amyotroph Lateral Scler Frontotemporal Degener 2018 Nov 26:1-9. Epub 2018 Nov 26.

a Department of Neurology , Peking University Third Hospital , Beijing , China.

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. Read More

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http://dx.doi.org/10.1080/21678421.2018.1542535DOI Listing
November 2018
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An epidemiologic investigation of amyotrophic lateral sclerosis in Thrace, Turkey, 2006-2010.

Amyotroph Lateral Scler Frontotemporal Degener 2018 Nov 23:1-7. Epub 2018 Nov 23.

f Neurology Department , Ekol Hospital , Edirne , Turkey.

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. Read More

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http://dx.doi.org/10.1080/21678421.2018.1525403DOI Listing
November 2018
2 Reads

Session Author Index.

Authors:

Amyotroph Lateral Scler Frontotemporal Degener 2018 ;19(sup1):85-87

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http://dx.doi.org/10.1080/21678421.2018.1510208DOI Listing
January 2018

Session Subject Index.

Authors:

Amyotroph Lateral Scler Frontotemporal Degener 2018 ;19(sup1):88-89

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http://dx.doi.org/10.1080/21678421.2018.1510209DOI Listing
January 2018

Theme 7 Pre-clinical therapeutic strategies.

Authors:

Amyotroph Lateral Scler Frontotemporal Degener 2018 ;19(sup1):217-239

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http://dx.doi.org/10.1080/21678421.2018.1510574DOI Listing
January 2018

Poster Subject Index.

Authors:

Amyotroph Lateral Scler Frontotemporal Degener 2018 ;19(sup1):398-401

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http://dx.doi.org/10.1080/21678421.2018.1530873DOI Listing
January 2018

Theme 8 Clinical imaging and electrophysiology.

Authors:

Amyotroph Lateral Scler Frontotemporal Degener 2018 ;19(sup1):240-263

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http://dx.doi.org/10.1080/21678421.2018.1510575DOI Listing
January 2018