Aerobic Training in Patients With Spinal Muscular Atrophy Type III
Condition: Spinal Muscular Atrophy
MeSH terms: Muscular Atrophy; Muscular Atrophy, Spinal; Atrophy
Study type: Interventional

Summary: 
      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.
    

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.
    

Intervention name(s): Aerobic conditioning
Intervention type(s): Behavioral
Intervention description(s): 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.
Intervention model: Single Group Assignment
Masking: None (Open Label)
Primary purpose: Treatment