Intramuscular pattern of fat infiltration in FSHD measured by MRI: towards identification of disease initiation and progression and improved diagnosis of disease severity

Intramuscular pattern of fat infiltration in FSHD measured by MRI: towards identification of disease initiation and progression and improved diagnosis of disease severity - Intramuscular fat infiltration pattern and progression in FSHD using MRI

Status

Active, not recruiting

Phases

Unknown

Study type

Observational

Source

NL-OMON

Registry ID

NL-OMON46124

Enrollment

Registered

2018-07-23

Start date

2018-09-18

Completion date

Unknown

Last updated

2024-04-15

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

fascioscapulohumeral muscular dystrophy muscle disease FSHD

Interventions

None listed

Eligibility

Age

18 Years to 99 Years

Inclusion criteria

Inclusion criteria: Genetically confirmed FSHD Adult (> 18 years old) Already underwent a MRI scan of the whole lower extremity ± 3 years ago

Exclusion criteria

Exclusion criteria: contra-indications for undergoing a MRI scan (e.g. because of pacemaker, claustrophobia, metal implants, metal splinters in the eye) inability to lie supine for 90 minutes.

Design outcomes

Primary

Measure	Time frame
The change in fat fraction and muscle volume over 3-4 years in the lower extremity muscles of FSHD patients	—

Secondary

Measure	Time frame
1) The effect of fat infiltration and STIR/TIRM hyper intensity at the baseline scan on the change in fat fraction and muscle volume over 3-4 years. To evaluate this we will first determine the percentage of muscles that were * Intermediately fat infiltrated at baseline that became fully fat infiltrated in 3-4 years * Non-fat infiltrated at baseline that became intermediately fat infiltrated in 3-4 years * Non-fat infiltrated at baseline that remained non-fat infiltrated after 3-4 years * STIR/TIRM hyper intensity at baseline and showed increased fat fraction in 3-4 years Second, we will apply a linear mixed model, see statistics paragraph. 2) Outcome measures for evaluating the pattern of infiltration in the baseline MRI scan are: * Fit quality (r2) of each model for every muscle. * The percentage of muscles where a) model A is the best fit b) model B is the best, c) model C is the best fit	—

Measure

Time frame

1) The effect of fat infiltration and STIR/TIRM hyper intensity at the baseline scan on the change in fat fraction and muscle volume over 3-4 years. To evaluate this we will first determine the percentage of muscles that were * Intermediately fat infiltrated at baseline that became fully fat infiltrated in 3-4 years * Non-fat infiltrated at baseline that became intermediately fat infiltrated in 3-4 years * Non-fat infiltrated at baseline that remained non-fat infiltrated after 3-4 years * STIR/TIRM hyper intensity at baseline and showed increased fat fraction in 3-4 years Second, we will apply a linear mixed model, see statistics paragraph. 2) Outcome measures for evaluating the pattern of infiltration in the baseline MRI scan are: * Fit quality (r2) of each model for every muscle. * The percentage of muscles where a) model A is the best fit b) model B is the best, c) model C is the best fit

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Countries

Netherlands

Outcome results