Muscle & nerve
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Glial fibrillary acid protein (GFAP) is increased in serum and cerebrospinal fluid of patients with dementia, traumatic brain injury, stroke, and multiple sclerosis. To determine whether GFAP is increased in Guillain-Barré syndrome (GBS) we evaluated serum GFAP in 30 controls, 20 patients with acute inflammatory demyelinating neuropathy (AIDP), and 17 with primary axonal GBS. ⋯ Applying the cutoff value in serum of 0.63 to the diagnosis of axonal GBS, we obtained a sensitivity of 76.5% and a specificity of 86%. Thus, serum GFAP levels may be used in GBS as a diagnostic marker of the axonal variant and to predict outcome.
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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.
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Comparative Study Clinical Trial
Comparison of perception threshold testing and thermal-vibratory testing.
Current perception threshold testing (CPT) is thought to selectively activate and measure three types of afferent nerves. However, it has not been standardized or compared with better-studied methods of sensory testing. Our objectives were to determine the relationship between CPT (2000 Hz, 250 Hz, 5 Hz) and quantitative sensory testing (QST) using vibratory and heat thresholds, and to assess the test-retest reliability of both methods. ⋯ Thermal thresholds were moderately correlated with CPT at 5 Hz (rho = 0.49, P = 0.009), as were vibratory thresholds and CPT at 2000 Hz (rho = 0.5, P = 0.008). In contrast to CPT measurements, warm and vibratory and cold thresholds were correlated 1 week apart (rho = 0.73, P = 0.0001; rho = 0.83, P = 0.0001; and rho = 0.47, P = 0.0037, respectively). CPT testing and QST seem to be measuring similar afferent nerve-fiber populations, but QST has better test-retest reliability than CPT testing, justifying its role in clinical or research studies.