The aim of this study is to assess the feasibility, reliability and validity of the Xsens 3D motion detector in patients with Leigh syndrome.
ID
Source
Brief title
Condition
- Other condition
Synonym
Health condition
mitochondriele aandoeningen
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
1. To determine the feasibility, reliability and validity of the Xsens 3D
motion detector for measuring daily activity in children with mitochondrial
disease and severe mental and / or physical disabilities.
a) Feasibility for measuring for 2 days
b) Reliability: for how many percent of the time does the Xsens 3D motion
detector obtain reliable data?
c) Validity:
o Agreement between Xsens data and the standardized
movements (if possible by means of the GMFM) for 30 minutes
o Correlation between Xsens data and activity reported by
the parents (reported on a scale of 0 to 10 every hour) for 1 weekend
Secondary outcome
1. Patterns in the frequency, duration, type and intensity of movements in
patients with Leigh syndrome
2. Patterns of movements in spasticity and various movement disorders
3. Testing the feasibility of the GMFM, Tardieu and MDRS
4. Get an indication of the frequency, duration, type and intensity of
movements related to how the patient feels.
Background summary
Mitochondrial disorders are the most common metabolic diseases that manifest
with disturbances in energy production. This leads to tiredness and exercise
intolerance in less affected patients and to a modified lifestyle with lots of
rest and low activity in more affected patients. Since more and more clinical
research is done in this disease, in particular in patients who are severely
affected, good outcome measures are of great importance. However, because many
of these patients are both physically and mentally severely disabled and
sometimes have problems with interaction, this is a challenge!
This pilot study explores a method to measure "exercise tolerance" (the ability
to maintain daily activities or the balance between rest and activity during
the day) in children who are severely mentally or physically limited.
We want to measure this with the Xsens 3D accelerometer. This is a
3-dimensional motion detector, which is able to measures the duration,
frequency, intensity and type of movement. The 3-dimensional motion detector
was able to estimate the level of activity in patients with Duchenne.
We expect that Xsens 3D motion detector will be able to measure physical
activity / fitness in patients with Leigh syndrome.
Study objective
The aim of this study is to assess the feasibility, reliability and validity of
the Xsens 3D motion detector in patients with Leigh syndrome.
Study design
This is an observational pilot study without invasive measurements.
Study burden and risks
We don't expect major risks in this pilot study. The burden is mainly a time
burden.
Geert Grooteplein-Zuid 10
Nijmegen 6525 GA
NL
Geert Grooteplein-Zuid 10
Nijmegen 6525 GA
NL
Listed location countries
Age
Inclusion criteria
Patients aged 0-18 years
- With on the MRI Leigh or Leigh-like syndrome
- And mitochondrial dysfunction in biochemical analysis of fresh muscle
- Who are regularly monitored in the Nijmegen Centre for Mitochondrial Disorders.
Exclusion criteria
It is expected that the trip to the hospital is too burdensome for the patient.
Fever
Epilepsia continua
Altered consiousness (according to parents/caretakers)
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 | NL42508.091.12 |