motor neuron disease muscle disease
Conditions
Interventions
None listed
Sponsors
Universitair Medisch Centrum Utrecht
Eligibility
Age
18 Years to 99 Years
Inclusion criteria
Inclusion criteria: 1. Definite, probable, probable-laboratory supported or possible ALS according to the revised El Escorial World Federation of Neurology criteria, primary lateral sclerosis or progressive spinal muscular atrophy. 2. Disease duration (at inclusion) more than 6 months and less than 36 months (disease onset is defined as the date of first symptoms excluding muscle cramps and fasciculations). 3. Vital capacity (VC%) >= 70 % of normal value (slow expiration, best of a minimum of three and a maximum of five measurements, with a respiratory function validly assessable and spontaneous, non-assisted ventilation). 4. Age 18 - 80 years (inclusive) 5. Right-handedness 6. Capable of thoroughly understanding the study information given; has signed the informed consent.
Exclusion criteria
Exclusion criteria: 1. Tracheostomy, tracheostomal ventilation of any type, non-invasive ventilation more than 16 hours/ day, or supplemental oxygen during the last three months prior to inclusion. 2. Any medical condition or intoxication known to have an association with motor neuron dysfunction, which might confound or obscure the diagnosis of ALS. 3. Presence of any other systemic or cerebral disease: diabetes, hypertension, dyslipidaemia and a history of stroke. 4. Presence of pronounced swallowing disorders (which make it dangerous to lie supine in the MRI scanner) 5. Contra-indication for 7Tesla MRI imaging (as established by the department) 6. Left-handedness and ambidextrous 7. Pregnancy
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| 1. Cortical thickness will be measured and compared with controls and non-affected cortex in the same subjects (3 and 7Tesla); 2. Motor pathways with DTI and probabilistic fibertracking (3Tesla); 3. Functional connectivity in motor pathways, with resting-state fMRI and probabilistic independent component analysis (3Tesla and 7Tesla). | — |
Secondary
| Measure | Time frame |
|---|---|
| The structural changes will be regarded in relation to the clinical condition, duration of illness and clinical parameters of motor function | — |
Countries
Netherlands
Outcome results
None listed