Amyotrophic lateral sclerosis and other motor neuron disorders : official publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases
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The ALSFRS-R is an attractive primary outcome measure in clinical trials of ALS because it is validated, easy to administer, minimizes dropout, reduces cost, and correlates with survival. Unlike the other standard outcome measures currently employed, the ALSFRS-R is also a measure of global function.
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Amyotroph. Lateral Scler. Other Motor Neuron Disord. · Sep 2004
Comparative StudyReduced NAA in motor and non-motor brain regions in amyotrophic lateral sclerosis: a cross-sectional and longitudinal study.
After replication of previous findings we aimed to: 1) determine if previously reported (1)H MRSI differences between ALS patients and control subjects are limited to the motor cortex; and 2) determine the longitudinal metabolic changes corresponding to varying levels of diagnostic certainty. ⋯ NAA ratios are reduced in the motor cortex and outside the motor cortex in ALS, suggesting widespread neuronal injury. Longitudinal changes of NAA are not reliable, suggesting that NAA may not be a useful surrogate marker for treatment trials.
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Amyotroph. Lateral Scler. Other Motor Neuron Disord. · Sep 2004
Comparative StudyOropharyngeal dysphagia in amyotrophic lateral sclerosis: neurological and dysphagia specific rating scales.
Oropharyngeal dysphagia is highly prevalent in amyotrophic lateral sclerosis (ALS). Patients with dysphagia and weight loss are frequently offered gastrostomy. Although the neurological basis of dysphagia in ALS is complex, there are currently no specifically validated scales for dysphagia in ALS, and the reliability of existing generic scales has not been assessed. ⋯ Generic dysphagia scales are reliable indicators of dysphagia in ALS. The bulbar components of the ALS specific scales is sensitive to dysphagia. The bulbar section of the Norris scale can be utilised as an independent and reliable indicator of the severity of dysphagia in ALS. In the absence of availability of detailed swallowing assessment using videofluoroscopy, these scales, i.e., the Norris and to a lesser degree the ALS FRS-R bulbar sections, are adequate to diagnose and follow clinically significant dysphagia in ALS, and can be used as an indicator for dysphagia treatment initiation.