Vagus Nerve Stimulation in children: A focus on intellectual disability

Vagus Nerve Stimulation (VNS) can be an efficacious add-on treatment in patients with drug-resistant epilepsy, who are not eligible for surgery. Evidence of VNS efficacy in children with intellectual disability (ID) is scarce.publisher: Elsevier articletitle: Vagus Nerve Stimulation in children: A focus on intellectual disability journaltitle: European Journal of Paediatric Neurology articlelink: http://dx.doi.org/10.1016/j.ejpn.2017.01.011 content_type: article copyright: © 2017 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.status: publishe

Muscle ultrasonography to predict survival in amyotrophic lateral sclerosis

International audienceWe assessed the prognostic value of muscle ultrasonography in 31 patients with amyotrophic lateral sclerosis (ALS) and compared it with accepted prognostic variables like functional capacity (measured with the ALSFRS-R rating scale) and muscle strength. Each patient was examined once. The following ultrasonography parameters were determined: muscle thickness, echo intensity (EI) and the presence of fasciculations. Correlations between baseline measurements, preslope values and survival were calculated. EI, disease duration, muscle strength preslope and ALSFRS-R preslope correlated with survival. Using a stepwise multivariate analysis, the combination of EI preslope and ALSFRS-R preslope was shown to have the best predictive value for survival

Perampanel bij patiënten met een verstandelijke beperking

De initiële registratiestudies van perampanel zijn inmiddels opgevolgd door ‘klinische’ studies die in essentie de registratiestudies lijken te bevestigen. Het aantal studies bij patiënten met een verstandelijke beperking is beperkt. In deze studie wordt het effect van perampanel bij patiënten met epilepsie en een verstandelijke beperking onderzocht, waarbij ook aandacht is voor het optreden van somatische en gedragsmatige bijwerkingen

TDP-43 accumulation is common in myopathies with rimmed vacuoles.

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Lifetime physical activity and the risk of amyotrophic lateral sclerosis

It has been hypothesised that physical activity is a risk factor for developing amyotrophic lateral sclerosis (ALS), fuelled by observations that professional soccer players and Gulf War veterans are at increased risk. In a population based study, we determined the relation between physical activity and risk of sporadic ALS, using an objective approach for assessing physical activity. 636 sporadic ALS patients and 2166 controls, both population based, completed a semistructured questionnaire on lifetime history of occupations, sports and hobbies. To objectively compare the energy cost of a lifetime history of occupational and leisure time physical activities and to reduce recall bias, metabolic equivalent scores were assigned to each activity based on the Compendium of Physical Activities. ALS patients had significantly higher levels of leisure time physical activity compared with controls (OR 1.08, 95% CI 1.02 to 1.14, p=0.008). No significant difference was found between patients and controls in the level of vigorous physical activities, including marathons and triathlons, or in occupational activity. Cumulative measures of physical activity in quartiles did not show a dose-response relationship. An increased risk of ALS with higher levels of leisure time physical activity was found in the present study. The lack of association with occupational physical activity and the absence of a dose-response relationship strengthen the hypothesis that not increased physical activity per se but rather a genetic profile or lifestyle promoting physical fitness increases ALS susceptibilit