Amyotrophic lateral sclerosis: considerations about the diagnosis criterial

BV UNIFESP: Teses e dissertaçõe

Restless leg syndrome exacerbated by amytriptiline in a patient with Duchenne Muscular Dystrophy

We report an unusual case of a Duchenne Muscular Dystrophy(DMD) patient who initiated a restless leg syndrome after the use of amytriptiline. The prescription and use of this medication for patients with persistent neuropathic pain is relatively common, especially for patients with DMD. Normally, this medication is well tolerated, however, we now report the occurrence of an induction or intensification of a restless leg syndrome case in a young patient with DMD, treated with amytriptiline for his chronic pain

MUNIX: Reproducibility and clinical correlations in Amyotrophic Lateral Sclerosis

Objective: To study the reproducibility, diagnostic yield to detect denervation, and clinical correlations of the Motor Unit Number Index (MUNIX) in subjects with Amyotrophic Lateral Sclerosis (ALS). Methods: MUNIX evaluation was performed in three muscles twice on the same day to assess reproducibility. Cut-off values for the MUNIX were based on data from 51 healthy subjects (controls) to evaluate the sensitivity of the technique to detect denervation in 30 subjects with ALS. Results: The method had good reproducibility. The variability was greater in the ALS group. In 23 ALS subjects (77%), low MUNIX values were detected. Most of the muscles with low MUNIX had also low compound muscle action potential (CMAP) and strength, but these parameters were normal in 9% of muscles. According to ROC curve analysis, MUNIX was generally accurate (AUC = 0.9504) for discriminating between healthy individuals and subjects with at least one denervated muscle. Conclusions: MUNIX variability was higher in the ALS group. The method showed good diagnostic performance for the detection of denervation in a sample of patients with ALS. Significance: This study demonstrated that in addition to being a quantitative tool MUNIX can detect denervation in subjects with ALS. (C) 2016 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.Univ Fed Sao Paulo, Dept Neurol, Sao Paulo, SP, BrazilUniv Sao Paulo, Sch Med FMUSP, Div Mol Med, Dept Med, BR-05508 Sao Paulo, BrazilUniv Fed Sao Paulo, Dept Neurol, Sao Paulo, SP, BrazilWeb of Scienc