Muscle & nerve
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Comparative Study
ALSFRS and appel ALS scores: discordance with disease progression.
Progression of disease and effectiveness of therapy in patients with amyotrophic lateral sclerosis (ALS) are determined by both questionnaire- and examination-based measures. To determine whether both types of measurement tools are equally predictive at all stages of disease, we compared questionnaire-based ALS Functional Rating Scale (ALSFRS) scores to the examination-based Appel ALS (AALS) scores at different stages of disease. Same-day scores were obtained during 174 visits in 62 patients with definite or probable ALS. ⋯ Predictions of ALSFRS based on AALS scores were less than half as precise in the later stages of disease. Both scales showed significant change with disease progression, but ALSFRS consistently underestimated disease severity defined by AALS (P < 0.001). Questionnaire-based measurements should be compared against objective scales at all stages of disease severity before they are accepted as primary endpoint measures.
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Ultrasound allows for a non-invasive structural assessment of nerves, muscles, and surrounding tissues, and therefore it is increasingly being used as a supplement to traditional electrodiagnostic studies. As investigators have begun to use ultrasound to explore peripheral nerves, it has become clear that conditions such as entrapment, hereditary neuropathies, acquired neuropathies, trauma, and nerve tumors result in an increase in nerve cross-sectional area. ⋯ Mean cross-sectional area, as well as side-to-side differences, are reported for each site, and qualitative data are provided to guide imaging at each site. The information provided in this study should serve as the starting point for quantitatively evaluating these nerve sites with ultrasound.