Introduction: Spinal muscular atrophy is an autosomal recessive disease in which the respiratory muscles are affected, with the most weakened intercostal muscles and a relatively preserved abdominal diaphragm. Purpose: We wanted to determine whether there is a difference in the values of the ten consecutive measurements of maximal inspiratory pressure between patients with spinal muscular atrophy and healthy subjects. In addition, we wanted to investigate whether there is a difference in the value of Fatigue Severity Scale between patients with spinal muscular atrophy and healthy subjects. We were interested in whether there is a correlation between the value of the motor functional scales and the absolute value of maximal inspiratory pressure in patients with spinal muscular atrophy. Methods: 20 adult patients with spinal muscular atrophy and 20 healthy subjects of similar age and gender were included in the study. Everyone performed ten consecutive measurements of maximum inspiratory pressure and filled out a fatigue questionnaire. From patients' documentation, we collected upper limb function and motor ability scores. Results: We found that there was no statistically significant difference in the absolute value of the individual executions of maximal inspiratory pressure between patients with spinal muscular atrophy and healthy subjects. Differences in the relative values of maximal inspiratory pressure between patients with spinal muscular atrophy and healthy subjects were expressed in the third, fifth, sixth, seventh, eighth, ninth and tenth executions, where patients had lower values. Patients with spinal muscular atrophy experienced greater fatigue than healthy subjects. In patients with spinal muscular atrophy, there was a statistically significant moderately high correlation between lower motor ability score and lower absolute value of the maximal inspiratory
pressure. Discussion and conclusion: Patients with spinal muscular atrophy achieved lower relative values of the maximal inspiratory pressure, meaning their respiratory muscles fatigued faster than in healthy subjects. Patients reported greater perceived fatigue in daily activities, which affected their quality of life.
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