Amyotrophic lateral sclerosis & frontotemporal degeneration
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Amyotroph Lateral Scler Frontotemporal Degener · Jun 2014
Multicenter StudyHealth utility decreases with increasing clinical stage in amyotrophic lateral sclerosis.
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease typically causing death within three years. Understanding the impact of disease on patients using health utility at different stages of ALS would allow meaningful cost-benefit analysis of new potential therapies. A common health-related quality of life measurement, developed and validated for the UK, is the EQ-5D. ⋯ Age of onset, disease onset, gender and treatment group were not predictors of EQ-5D, depression or anxiety. In conclusion, increasing severity of King's ALS Clinical Stage is associated with a progressive decrease in EQ-5D health utility. This is useful for cost-benefit analysis of new therapies and validates this ALS clinical staging system.
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Amyotroph Lateral Scler Frontotemporal Degener · Jun 2014
Estimating clinical stage of amyotrophic lateral sclerosis from the ALS Functional Rating Scale.
ALS is a progressive neurodegenerative disease. The stage of disease reached can be described using a simple system based on the number of central nervous system regions involved. Historically, datasets have not attempted to record clinical stage, but being able to re-analyse the data by stage would have several advantages. ⋯ Results showed that the agreement between staging by the two methods was excellent with an intraclass correlation coefficient of 0.92 (95% confidence interval 0.88-0.94). There was no systematic bias towards over-staging or under-staging using the algorithm. In conclusion, we have shown that clinical stage in ALS can be reliably estimated using the ALSFRS-R in historical data and in current data where stage has not been recorded.
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Amyotroph Lateral Scler Frontotemporal Degener · Jun 2014
A visual MRI atrophy rating scale for the amyotrophic lateral sclerosis-frontotemporal dementia continuum.
Our objective was to distinguish ALS, ALS-FTD and bvFTD via a novel visual MRI cortical atrophy scale that can be employed in a clinical setting. MRI images of 100 participants (33 ALS, 11 ALS-FTD, 22 bvFTD and 34 controls) were rated in four brain areas: orbitofrontal cortex, anterior temporal pole, anterior cingulate, and motor cortex. Areas were rated on a 5- point Likert scale by two raters blinded to the diagnosis. ⋯ Our study demonstrates that a simple visual MRI rating scale can reliably distinguish ALS, ALS-FTD and bvFTD atrophy patterns in a clinical setting. Motor cortex, anterior cingulate and anterior temporal atrophy emerged as good diagnostic markers for ALS-FTD. Employment of this MRI rating scale can complement clinical diagnostics of patients in the ALS-FTD continuum.
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Amyotroph Lateral Scler Frontotemporal Degener · Jun 2014
Distinct patterns of cortical atrophy in ALS patients with or without dementia: an MRI VBM study.
Voxel based morphometry (VBM) allows objective and automated detection of structural changes in brains of patients with amyotrophic lateral sclerosis (ALS). We investigated whether VBM could identify cortical atrophy from T1-weighted images obtained during routine 1.5T studies of ALS patients with various clinically defined phenotypes. For this purpose T1-weighted brain MRI was obtained at 1.5T during routine clinical study in neurologic disease controls (n = 15) and ALS patients (n = 88) categorized into four subgroups based on their clinical phenotypes: predominant upper motor neuron (UMN) dysfunction with or without corticospinal tract (CST) hyperintensity (ALS-CST+/-), combined UMN and prominent lower motor neuron (LMN) dysfunction (classic ALS), and frontotemporal dementia (ALS-FTD). ⋯ Results demonstrated that clinically obtained brain MRI at 1.5T revealed significantly reduced GM volume in brains of only ALS-FTD patients and not of those with predominant UMN dysfunction or classic ALS, compared to neurologic disease controls. In conclusion, GM volume loss in motor and extramotor regions of only ALS patients with FTD and not of ALS patients without FTD suggests distinct sites of predominant pathology and possibly of disease onset. Brain volumetric measures supplemented by histopathological correlations and other neuroimaging techniques, such as diffusion tensor imaging, may provide insight into ALS pathophysiology.
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Amyotroph Lateral Scler Frontotemporal Degener · Jun 2014
Improved survival with an ambulatory model of non-invasive ventilation implementation in motor neuron disease.
Non-invasive ventilation (NIV) increases survival and quality of life in motor neuron disease (MND). NIV implementation historically occurred during a multi-day inpatient admission at this institution; however, increased demand led to prolonged waiting times. The aim of this study was to evaluate the introduction of an ambulatory model of NIV implementation. ⋯ Survival was also prolonged (median (IQR) 278 (51-512) days pre- vs 580 (306-1355) days post-introduction of the Day Admission model; hazard ratio 0.41, p = 0.04). Daytime PaCO2 was no different. In conclusion, reduced waiting time to commence ventilation and improved survival were observed following introduction of an ambulatory model of NIV implementation in people with MND, with no change in the effectiveness of ventilation.