Aerobic Training in Patients With Spinal Muscular Atrophy Type III

This study has been completed.
Sponsor:
Information provided by (Responsible Party):
Karen Lindhardt Madsen, Rigshospitalet, Denmark
ClinicalTrials.gov Identifier:
NCT02003937
First received: August 22, 2013
Last updated: December 2, 2013
Last verified: December 2013
  Purpose

Spinal muscular atrophy type III, (SMAIII) is a disease in the nerve cells in the spinal cord which leads to to progressive muscle weakness and atrophy. No effective treatment is available for SMA. We have previously shown that patients with muscular dystrophies improve oxidative capacity (VO2max), muscle strength and daily function by aerobic conditioning. Patients with SMAIII share many clinical features with these conditions, although the mechanism of muscle weakness is different. In this study, we investigated how patients with SMAIII respond to aerobic training.

6 patients and 9 healthy age- and sex-matched controls completed a 12 weeks training program. Subjects performed a total of 42 training session of 30 min on a stationary cycle ergometer at home. The work intensity was moderate and set to match a target heart rate.

Training induced an increase without inducing muscle damage. However, training-induced fatigue was a major complaint in all patients, and caused one patient to drop out, increased the need for sleep in three patients and two had to modify the training program.

The fatigue limits the use of this therapy. The training-induced fatigue, which is not encountered in muscle diseases, warrants investigations into alternative training methods to improve quality of life in patients with SMAIII.


Condition Intervention
Spinal Muscular Atrophy
Behavioral: Aerobic conditioning

Study Type: Interventional
Study Design: Intervention Model: Single Group Assignment
Masking: Open Label
Primary Purpose: Treatment
Official Title: Aerobic Training Improves Oxidative Capacity, But Not Function in Spinal Muscular Atrophy III

Resource links provided by NLM:


Further study details as provided by Rigshospitalet, Denmark:

Primary Outcome Measures:
  • Changes in oxidative capacity with aerobic conditioning in SMAIII patients [ Time Frame: After 12 weeks of aerobic training ] [ Designated as safety issue: No ]

Secondary Outcome Measures:
  • Changes in maximal workload capacity with aerobic conditioning in SMAIII patients [ Time Frame: After 12 weeks of aerobic conditioning ] [ Designated as safety issue: No ]
    The maximal workload capacity was defined as: The maximal resistance the subject was able to work at, during an incremental load exercise test, performed on a cycle ergometer.

  • Changes in isometric muscle strength with aerobic conditioning in SMAIII patients [ Time Frame: After 12 weeks of aerobic conditioning ] [ Designated as safety issue: No ]
    Isometric muscle strength was measured using a hand held dynamometer testing the strengths in the gastrocnemius and the quadriceps muscles of the legs. Strengths in the biceps and deltoid muscles were used as controls.

  • Changes in daily function with aerobic conditioning in SMAIII patients [ Time Frame: After 12 weeks of aerobic training ] [ Designated as safety issue: No ]
    Changes in daily function was measured as changes in activities af daily living (ADL) reported in a ADL-questionnaire


Enrollment: 15
Study Start Date: September 2009
Study Completion Date: September 2010
Primary Completion Date: September 2010 (Final data collection date for primary outcome measure)
Arms Assigned Interventions
Experimental: 12 weeks of aerobic conditioning
12 weeks of aerobic conditioning, a total 42 sessions of 30min exercise on a stationary cycle ergometer
Behavioral: Aerobic conditioning
Subjects performed 12 weeks of aerobic training on a stationary cycle ergometer. Subjects compleeted a total of 42 training sessions of 30min. exercise at an individually adjusted moderate workload.

Detailed Description:

Spinal muscular atrophy type III, (SMAIII) is a recessively inherited disease in the lower motor neuron in the anterior horn of spinal cord leading to to progressive muscle weakness and atrophy. Currently there is no effective treatment available for SMA. We have previously shown that patients with muscular dystrophies improve oxidative capacity (VO2max), muscle strength and daily function by aerobic conditioning. Patients with SMAIII share many clinical features with these conditions, although the mechanism of muscle weakness is different. In this study, we investigated how patients with SMAIII respond to aerobic training.

6 patients and 9 healthy age- and sex-matched controls completed a 12 weeks training program. Subjects performed a total of 42 training session of 30 min on a stationary cycle ergometer at home. The work intensity was moderate and set to match a target heart rate.

VO2max was measured during a incremental exercise test using indirect calorimetry before and after the training period. Functional tests adressing patients walking and stair climbing abilities, were performed before and after the training period. Changes in activities of daily living was adressed in a standardized questionnaire after the training period.

Training induced an increase without inducing muscle damage. There were no changes in patients' functional capacities. However, training-induced fatigue was a major complaint in all patients, and caused one patient to drop out, increased the need for sleep in three patients and two had to modify the training program.

The fatigue limits the use of this therapy. The training-induced fatigue, which is not encountered in muscle diseases, warrants investigations into alternative training methods to improve quality of life in patients with SMAIII.

  Eligibility

Ages Eligible for Study:   18 Years to 65 Years
Genders Eligible for Study:   Both
Accepts Healthy Volunteers:   Yes
Criteria

Inclusion Criteria:

  • Genetically verified SMAIII

Exclusion Criteria:

  • other serious medical conditions that could confound the interpretation of results and
  • regular exercise more than one hour weekly
  Contacts and Locations
Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below. For general information, see Learn About Clinical Studies.

Please refer to this study by its ClinicalTrials.gov identifier: NCT02003937

Locations
Denmark
Rigshospitalet, Neuromuscular Research Unit, 3342
Copenhagen E, Denmark, 2100
Sponsors and Collaborators
Rigshospitalet, Denmark
Investigators
Principal Investigator: Karen L Madsen, B.Sc Rigshospitalet, Denmark
  More Information

No publications provided

Responsible Party: Karen Lindhardt Madsen, B.Sc, Rigshospitalet, Denmark
ClinicalTrials.gov Identifier: NCT02003937     History of Changes
Other Study ID Numbers: H-KF297836
Study First Received: August 22, 2013
Last Updated: December 2, 2013
Health Authority: Denmark: Ethics Committee

Additional relevant MeSH terms:
Muscular Atrophy
Muscular Atrophy, Spinal
Spinal Muscular Atrophies of Childhood
Atrophy
Spinal Cord Diseases
Neuromuscular Manifestations
Neurologic Manifestations
Nervous System Diseases
Pathological Conditions, Anatomical
Signs and Symptoms
Central Nervous System Diseases
Motor Neuron Disease
Neurodegenerative Diseases
Neuromuscular Diseases
Heredodegenerative Disorders, Nervous System
Genetic Diseases, Inborn

ClinicalTrials.gov processed this record on July 22, 2014