Fasciculations are a nearly universal feature in people with amyotrophic lateral sclerosis (ALS). The prognostic value of fasciculations remains uncertain. Twenty-four patients with ALS were evaluated for the effects of atrophy, limb weakness, disease duration, and physical activity on fasciculation frequency (as measured by surface electromyography and clinical counting). Variables were compared by multiple linear regression. As strength of the limb deteriorated, the number of fasciculations in the same limb increased, as long as physical activity was maintained or increased. Fasciculation frequency was not associated with the duration of ALS (r = 0.22; p = 0.30) and was independent of the degree of limb weakness (p>0.05) and limb atrophy (p>0.05). No prediction of disease duration could be made based on fasciculation frequency alone. Fasciculations therefore appear to have diagnostic, but not prognostic, utility in the care of people with ALS.
"Both BCIs, the P300 and the SMR neurofeedback paradigm, do require a substantial amount of selective and sustained attention which may be impaired in neurological patients (Hanagasi et al., 2002; Pinkhardt et al., 2008). Other general factors that may influence BCI performance are daytime sleepiness (Buhr-Schinner et al., 1995; Barthlen and Lange, 2000; Mochty et al., 2005) and noisy data due to fasciculation (Mateen et al., 2008). Additionally, BCI trials have to be interrupted more often, because ALS patients cannot suppress coughing, swallowing saliva or yawning, as a consequence of the disease, which leads to larger EMG artifacts than in healthy controls. "
[Show abstract][Hide abstract] ABSTRACT: The current study investigated the effects of psychological well-being measured as quality of life (QoL), depression, current mood and motivation on brain-computer interface (BCI) performance in amyotrophic lateral sclerosis (ALS). Six participants with most advanced ALS were trained either for a block of 20 sessions with a BCI based on sensorimotor rhythms (SMR) or a block of 10 sessions with a BCI based on event-related potentials, or both. Questionnaires assessed QoL and severity of depressive symptoms before each training block and mood and motivation before each training session. The SMR-BCI required more training than the P300-BCI. The information transfer rate was higher with the P300-BCI (3.25 bits/min) than with the SMR-BCI (1.16 bits/min). Mood and motivation were related to the number of BCI sessions. Motivational factors, specifically challenge and mastery confidence, were positively related to BCI performance (controlled for the number of sessions) in tow participants, while incompetence fear was negatively related with performance in one participant. BCI performance was not related to motivational factors in three other participants nor to mood in any of the six participants. We conclude that motivational factors may be related to BCI performance in individual subjects and suggest that motivational factors and well-being should be assessed in standard BCI protocols. We also recommend using P300-based BCI as first choice in severely paralyzed patients who present with a P300 evoked potential.
Frontiers in Neuroscience 07/2010; 4. DOI:10.3389/fnins.2010.00055 · 3.66 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Our objective was to determine the prevalence and distribution of fasciculations in healthy adults and to assess the effect of age, caffeine and exercise. Fasciculations were studied with ultrasonography in 58 healthy adults in various age categories. Questionnaires were used to determine effect of caffeine and regular exercise on the presence of fasciculations. Finally, we tested the effect of strenuous exercise on fasciculations in 10 healthy adults. Twenty-five subjects (43%) showed fasciculations on ultrasonography, mostly in the abductor hallucis longus muscle. Fasciculations were only sporadically encountered in muscle groups above the knee. Subjects with fasciculations were significantly older than those without. Caffeine and regular physical exercise did not influence the prevalence of fasciculations. However, strenuous physical exercise caused a temporary increase in fasciculations, but only in lower leg muscles. Fasciculations above the knee should raise suspicion and may warrant further investigation.
[Show abstract][Hide abstract] ABSTRACT: Respiratory muscle involvement is one of the main prognostic factors in amyotrophic lateral sclerosis (ALS). Acute respiratory failure is sometimes the first manifestation of the disease, although onset can be more insidious. In the present retrospective study, it was proposed to review the clinical features and to assess the effects of non-invasive ventilation (NIV) on the prognosis of patients with respiratory onset, which was taken to be present when the first symptoms of muscular weakness were dyspnoea at exertion, dyspnoea at rest, or orthopnoea. Respiratory onset ALS is uncommon, since it accounts for less than 3% of ALS cases. ALS with respiratory onset has some common clinical features: male predominance, frequent camptocormia or dropped head, frequent widespread fasciculations, limb mobility fairly well preserved and significant weight loss in the early stages. ALS patients with respiratory onset still have a poor prognosis compared with those with bulbar or spinal forms. NIV should be proposed promptly because it improves the symptoms, general state of health and survival time. Efforts should be made to inform general practitioners and chest physicians and remind them that neuromuscular respiratory insufficiency may be attributable to this particular form of ALS.
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