Metabolic state and survival in patients with amyotrophic lateral sclerosis

Metabolic state and survival in patients with amyotrophic lateral sclerosis - MEASURE

Status

Active, not recruiting

Phases

Unknown

Study type

Observational

Source

NL-OMON

Registry ID

NL-OMON50744

Enrollment

234

Registered

2016-04-13

Start date

2017-03-30

Completion date

Unknown

Last updated

2024-04-23

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

Conditions

amyotrophic lateral sclerosis (ALS) motor neuron disease (MND)

Interventions

None listed

Eligibility

Age

18 Years to 99 Years

Inclusion criteria

Inclusion criteria: For patients only (N = 78): meeting the El Escorial criteria for definite, probable, probable laboratory supported or possible ALS, For controls only (N = 78): diagnosis with neurological syndrome within the range of peripheral polyneuropathies with sensorimotor or motor fiber involvement. Or other motor neuron disease (primary lateral sclerosis, progressive muscular atrophy, progressive bulbar palsy or pseudobulbar palsy) , Asymptomatic carriers and family members (N = 2x39): absence of any neurological symptom associated with ALS., For family members, non-carriers and carriers, of any related gene to ALS: absence of any neurological symtpoms associated with ALS. , All subjects: >= 18 years of age

Exclusion criteria

Exclusion criteria: All subjects: 1.2. Participants

Design outcomes

Primary

Measure	Time frame
The main endpoint in the study will be disease progression, expressed as change from baseline on the revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R)	—

Secondary

Measure	Time frame
• Survival, defined as death of any cause and/or permanent respiratory support. • Lung function, analyzed as change from baseline and expressed as forced vital capacity (FVC). • The metabolic state will be evaluated as energy expenditure (expressed in VO2/kg consumption and estimated kilocalories/day) and respiratory exchange ratio (RER). • The body composition is evaluated by % fat mass, % fat free mass, body volume (liters) and body density (kg/L) • The dietary intake will be evaluated by average caloric intake/day. Subdivision of caloric intake in percentage protein, fat (saturated, unsaturated) and carbohydrates. Specific substrates will be registered and expressed as average daily intake in grams: Alpha-linolenic acid (ALA), eicosapentaenoic acid (EPA), docosahexaenoic acid (DHA), cholesterol, fibers, alcohol, calcium (mg), vitamin B2, C&E (mg), lycopene, flavonoids, glutamate and phytoestrogens	—

Measure

Time frame

• Survival, defined as death of any cause and/or permanent respiratory support. • Lung function, analyzed as change from baseline and expressed as forced vital capacity (FVC). • The metabolic state will be evaluated as energy expenditure (expressed in VO2/kg consumption and estimated kilocalories/day) and respiratory exchange ratio (RER). • The body composition is evaluated by % fat mass, % fat free mass, body volume (liters) and body density (kg/L) • The dietary intake will be evaluated by average caloric intake/day. Subdivision of caloric intake in percentage protein, fat (saturated, unsaturated) and carbohydrates. Specific substrates will be registered and expressed as average daily intake in grams: Alpha-linolenic acid (ALA), eicosapentaenoic acid (EPA), docosahexaenoic acid (DHA), cholesterol, fibers, alcohol, calcium (mg), vitamin B2, C&E (mg), lycopene, flavonoids, glutamate and phytoestrogens

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Countries

Netherlands

Outcome results