Imaging in motor neuron diseases; biomarker research with MRI

Recruiting

Conditions: ALS
muscle disease
10029317

Registration Number: NL-OMON55454

Lead Sponsor: niversitair Medisch Centrum Utrecht

Brief Summary: Not available

Detailed Description: Not available

Recruitment & Eligibility

Status: Recruiting

Sex: Not specified

Target Recruitment: 2203

Inclusion Criteria

1a) 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 /with
mimic disorders (e.g. multifocal motor neuropathy, inclusion body myositis,
cervical myeloradiculopathy, myasthenia gravis, Kennedy*s disease, spinal
muscular atrophy).
1b) Healthy controls without manifest diagnosis of motor neuron disease or ALS
mimic
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 3Tesla MRI imaging (as established by the
radiology 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 in patients (ALS, MND, ALS mimics) and controls (3Tesla);<br /><br>2).Structural connectivity of motor pathways with DTI and deterministic fiber<br /><br>tracking (3Tesla); 3) Brain functional connectivity with resting-state fMRI<br /><br>(3Tesla).</p><br>

Secondary Outcome Measures