Lou Gehrig's disease Motor neuron disease
Conditions
Interventions
None listed
Sponsors
Universitair Medisch Centrum Utrecht
Eligibility
Age
18 Years to 99 Years
Inclusion criteria
Inclusion criteria: 1. Age >= 18 years at the time of screening 2. capable of providing informed consent and complying with the study procedures; 3. Belong to one of the following population bases: a) Diagnosis of ALS according to the revised El Escorial criteria (possible, probable-laboratory supported, probable or definite), PMA or PLS b) Healthy controls with: no history of nerve entrapment syndromes (such as carpal tunnel syndrome); no active neurological, neuropsychiatric or neuromuscular conditions. c) Family members from patients with a history of ALS in the family (regardless of the presence of gene mutation status or knowledge thereof), with: no history of nerve entrapment syndromes (such as carpal tunnel syndrome); no active neurological, neuropsychiatric or neuromuscular conditions.
Exclusion criteria
Exclusion criteria: 1. Pregnancy 2. Alcohol dependence syndrome, current regular use of neuroleptic or psychoactive medication 3. The presence of frontotemporal dementia (FTD) with a degree of severity that could potentially hamper compliance with the study protocol. Judgment is up to the investigators. Further exclusion criteria for patients: patients with frontotemporal dementia may initially be able to comply with the study procedures. These participants may be excluded from follow-up recordings, if they lose the ability to adequately follow and adhere to the examiner*s instructions. Further exclusion criteria for participants of cortical excitability studies: although cortical excitability testing is not incorporated in the two base sequences A-B (see section 5.3), these may be added later. For these specific tests we will follow the expert guidelines for safety and recommendations for TMS, excluding individuals with (a history of) epilepsy or metallic implants (e.g. pacemakers or deep-brain stimulators).
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Primary endpoints: • Measures derived directly from CMAP-scans pertaining to motor neuron loss (MUNE, D50, CMAP-max, etc.) • Measures derived directly from peripheral excitability tests (SDTC, superexcitability, etc.) | — |
Secondary
| Measure | Time frame |
|---|---|
| Secondary endpoints: measures derived directly from cortical excitability tests and RNS tests, clinical parameters of functional state (ALSFRS-R questionnaire, neurological examination), genetic data, survival and/or time to assisted ventilation | — |
Countries
Netherlands
Outcome results
None listed