1) Identify and further explore the structural and functional brain alterations in ALS patients, PSMA patients annd PLS patients in comparison to healthy controls and ALS mimic sydromes, both cross-sectional and longitudinal; 2) Explore anatomical…
ID
Source
Brief title
Condition
- Neuromuscular disorders
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
1) Cortical thickness, measured in T1 weighted images. The imaging data will be
compared with controls and ALS mimic syndromes and to non-affected cortical
regions in the same subjects (3Tesla and/or 7Tesla); 2).Structural connectivity
of motor pathways with DTI and deterministic fiber tracking. (3Tesla); 3) Brain
functional connectivity with resting-state fMRI (3Tesla and/or 7Tesla); 4)
Glutamate/GABA intensity with MR spectroscopy (7Tesla).
Secondary outcome
The structural changes will be regarded in relation to the clinical condition,
duration of illness and clinical parameters of motor function.
Background summary
Neuropathological as well as radiological studies have demonstrated structural
changes in brain and spinal cord of patients with ALS, for example white matter
changes and cortical atrophy especially in the motor cortex.
Study objective
1) Identify and further explore the structural and functional brain alterations
in ALS patients, PSMA patients annd PLS patients in comparison to healthy
controls and ALS mimic sydromes, both cross-sectional and longitudinal; 2)
Explore anatomical changes in asymptomatic family members of patients with
familial ALS. 3) Investigate the correlations of anatomical changes with
clinical variables.
Study design
Observational cross sectional and longitudinal study
Study burden and risks
The participants will undergo clinical assessment, 3Tesla and 7Tesla MRI in the
UMC Utrecht. For the individual participant there are no direct benefits. The
information acquired by this research project may provide new insights in
diagnosing, measuring disease progression and pathogenesis of ALS/MND.
Heidelberglaan 100
3584 CX Utrecht
Nederland
Heidelberglaan 100
3584 CX Utrecht
Nederland
Listed location countries
Age
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
Design
Recruitment
Followed up by the following (possibly more current) registration
No registrations found.
Other (possibly less up-to-date) registrations in this register
No registrations found.
In other registers
| Register | ID |
|---|---|
| CCMO | NL38399.041.11 |