88 results
Primary:The primary objectives of the study are to estimate and rank-order the longitudinal standardized mean changes over 6 months and over 12 months, for a set of novel outcome measures administered to subjects with ALS, in order to identify…
The main objective of this prospective population-based study of patients with proximal SMA in the Netherlands is to register SMA patients in the Netherlands in a socalled SMA-database to study prevalence and severity of SMA types 1-4, to perform…
To evaluate whether neuromuscular ultrasound discriminates between patients with and without ICU-AW.To detect differences between patients that do and do not have ICU-AW in order to determine relevant cut-offs which can be used in future diagnostic…
The frontotemporal syndrome in amyotrophic lateral sclerosis: screen, impact, imaging and pathology.
The main objectives are: 1. to develop a diagnostic tool for the assessment of the frontotemporal syndrome. 2. To investigate the structural and functional brain networks of ALS patients with and without a frontotemporal syndrome, in order to gain…
The primary objectives of this study are (a) to evaluate reliability and precision of an instrumented spasticity assessment device (ISA) in children with cerebral palsy and adults with spastic paresis (MS and stroke patients) and (b) to validate ISA…
To study the role of brain metabolites and macromolecules in relationship to pathogenesis, structural brain changes and clinical phenotype of ALS. This will reveal underlying molecular mechanisms of pathogenesis, structural brain changes and…
Main objective: - Obtain long-term clinical follow-up data in PLS patients to investigate the reliability of the diagnosis PLS. - Obtain data on cognitive functioning to investigate the possible relation between PLS and FTD.Secondary objectives: -…
To determine the reproducibility and validity of specific outcome measures for skeletal and respiratory fatigability in subjects with SMA.
To investigate motor connectivity in vivo in patients with SMA using MR- techniques for brain imaging.We will investigate connectivity of upper motor neurons in patients with SMA compared to disease controls (patients with myopathic disorders) and…
Primary:- To identify causal genetic variants and proteomes involved in achalasia and to study their relevanceSecundary:- To build-up a large scale harmonized biobank of achalasia patients, based on combining existing and new data from the European…
- To study the clinical characteristics and disease course of patients with LEMS with or without SCLC- To identify the characteristics of the humoral or cellular immune response that are associated with the prolonged survival of patients with SCLC…
To obtain normal values for neuromuscular ultrasound parameters in adults.
In this pilot study we aim to investigate the additional value of sEMG signals of respiratory muscles during ICU admission in adults.
To optimize ventilator support in critically ill patients admitted to the intensive care unit by monitoring electromyography of the respiratory muscles.
The main objective of this prospective cohort study of patients with proximal SMA in the Netherlands is to investigate the function of the neuromuscular junction by means of repetitive nerve stimulation (RNS).
To develop a screening test to predict ICU-AW at an early stage in patients admitted to the ICU. Also by prospectively following the patient group in time, this study aims to describe the natural history and functional limitations in patients…
1. To investigate the possible relationship between amount of dystrophin expression and clinical severity of BMD2. To investigate the relationship between specific BMD-gene mutations and clinical severity of BMD3. To investigate possible other…
The primary objective of this proof of principle study is to determine the effect of levosimendan on the strength and endurance of the diaphragm in healthy subjects. The secondary objective is to gain insight in the neural activation and efficiency…
The primary objective is to determine whether the diaphragm develops contractile fatigue during a weaning trial and if this is associated with weaning failure. Furthermore, the hypothesis whether diaphragm efficiency (Effdi = electrical activity of…
The purpose of this study is: 1) to develop a method to quantify muscular fat content in brachial plexus injury patients;2) to investigate whether muscular fat content is more in the affected arm compared to the contralateral arm in BPI patients;3)…