Imaging in amyotrophic lateral sclerosis; novel biomarker research with 7Tesla MRI

Completed

Conditions: 10029317
motor neuron disease
muscle disease

Registration Number: NL-OMON34004

Lead Sponsor: niversitair Medisch Centrum Utrecht

Brief Summary: Not available

Detailed Description: Not available

Recruitment & Eligibility

Status: Completed

Sex: Not specified

Target Recruitment: 140

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

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

Study & Design

Study Type: Observational invasive

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
<p>1. Cortical thickness will be measured and compared with controls and<br /><br>non-affected cortex in the same subjects (3 and 7Tesla); 2. Motor pathways with<br /><br>DTI and probabilistic fibertracking (3Tesla); 3. Functional connectivity in<br /><br>motor pathways, with resting-state fMRI and probabilistic independent component<br /><br>analysis (3Tesla and 7Tesla). </p><br>

Secondary Outcome Measures

Name	Time	Method
<p>The structural changes will be regarded in relation to the clinical condition,<br /><br>duration of illness and clinical parameters of motor function </p><br>