Imaging in motor neuron diseases; biomarker research with MRI

Withdrawn

Conditions: motor neuron disease (MND)
muscle disease
10029317

Registration Number: NL-OMON35176

Lead Sponsor: niversitair Medisch Centrum Utrecht

Brief Summary: Not available

Detailed Description: Not available

Recruitment & Eligibility

Status: Withdrawn

Sex: Not specified

Target Recruitment: 900

Inclusion Criteria

1. Patients: i) For ALS patients: definite, probable, probable-laboratory supported or possible ALS according to the revised El Escorial criteria (Brooks 2000); familial ALS is defined only if there is a family history of ALS. ii) For progressive spinal muscular atrophy (PSMA) or primary lateral sclerosis (PLS): patients with clinical diagnosis of PSMA or PLS, after excluding other diseases. iii) Patients with *ALS mimic syndromes*: patients suspected of mimic disorders (e.g. multifocal motor neuropathy, inclusion body myositis, cervical myeloradiculopathy, myasthenia gravis,
Kennedy*s disease). 2. Age 18 - 80 years (inclusive). 3. 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; 2 ) Any history or presence of brain injury, epilepsy, psychiatric illness and other cerebral disease; 3) Any intoxication or medication known to have an association with motor neuron dysfunction, which might confound or obscure the diagnosis of motor neuron disease; 4) Presence of pronounced swallowing disorders (which make it dangerous to lie supine in the MRI scanner); 5) Contra-indication for 3 or 7Tesla MRI imaging (as established by the department); 6) Pregnancy

Study & Design

Study Type: Observational invasive

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
<p>1) Cortical thickness, measured in T1 weighted images. The imaging data will be<br /><br>compared with controls and ALS mimic syndromes and to non-affected cortical<br /><br>regions in the same subjects (3Tesla and/or 7Tesla); 2).Structural connectivity<br /><br>of motor pathways with DTI and deterministic fiber tracking. (3Tesla); 3) Brain<br /><br>functional connectivity with resting-state fMRI (3Tesla and/or 7Tesla); 4)<br /><br>Glutamate/GABA intensity with MR spectroscopy (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>