Amyotrophic lateral sclerosis & frontotemporal degeneration
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Amyotroph Lateral Scler Frontotemporal Degener · Nov 2017
Review Meta AnalysisNocebo in motor neuron disease: systematic review and meta-analysis of placebo-controlled clinical trials.
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). ⋯ Our study indicates significant nocebo in trials for MND treatment, adversely affecting adherence and efficacy of current treatments in clinical practice, with additional implications for trial design.
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Amyotroph Lateral Scler Frontotemporal Degener · Nov 2017
Controlled Clinical TrialEffectiveness of assisted and unassisted cough capacity in amyotrophic lateral sclerosis patients.
Decreased cough capacity during a respiratory infection is one of the main causes of acute respiratory failure and hospitalisation in amyotrophic lateral sclerosis (ALS).
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Amyotroph Lateral Scler Frontotemporal Degener · Nov 2017
Comparative StudyNeuroimaging patterns along the ALS-FTD spectrum: a multiparametric imaging study.
Frontotemporal dementia is associated with considerable clinical, genetic and pathological heterogeneity. The objective of this study is to characterise the imaging signatures of the main FTD phenotypes along the ALS-FTD spectrum. A total of 100 participants underwent comprehensive multimodal neuroimaging, genetic testing and neuropsychological evaluation. ⋯ C9-ALS-FTD patients exhibited widespread extramotor pathology and significant precentral gyrus atrophy compared to ALSnci patients. ROI analyses confirmed focal grey matter alterations in Broca's and Wernicke's area in language variant FTD cohorts. Our findings confirm that the clinical manifestations of FTD are underpinned by phenotype-specific patterns of white and grey matter degeneration.
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Amyotroph Lateral Scler Frontotemporal Degener · Nov 2017
Incorporation of telehealth into a multidisciplinary ALS Clinic: feasibility and acceptability.
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. ⋯ Telehealth was generally viewed favourably by ALS patients, caregivers, and multidisciplinary team members. Improvements in technology and in methods to provide satisfactory remote care without person-to-person contact should be explored.
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Amyotroph Lateral Scler Frontotemporal Degener · Nov 2017
Ability of pulmonary function decline to predict death in amyotrophic lateral sclerosis patients.
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). ⋯ This study provides tools to calculate the increase in risk of death from a PFT decline. At an individual level, since each test showed some flaws, the use of a combination of PFTs for ALS respiratory monitoring is recommended.