Respiratory Aspects of Pseudohypertrophic Muscular Dystrophy

Respiratory problems in children with neurological impairment

“…In the different neuromuscular syndromes, although the rate of progression varies, the sequence is similar. Intercostals are affected relatively early, 11 causing paradoxical breathing (chest wall sucked in as abdomen goes out) and a bell shaped chest. Diaphragm weakness occurs later and heralds the onset of respiratory failure.…”

Cardiopulmonary Response to Exercise in Patients with Neuromuscular Diseases

“…(2) Is there evidence for an abnormal cardiorespiratory response to exercise in such patients? (3) Are there reasons to anticipate that training of the respiratory muscles will possibly have a negative effect on such pa tients' condition.…”

“…Most of the reported data are interpreted as consequences of an insufficient cough ing function due to muscular weakness and restrictive impairment with no indica tion of obstructive elements [1][2][3][4]. Con tradictory ventilatory responses to CO2 have been reported [5,6], The type of re striction is not fully clarified although a reduction in total lung capacity and an in crease in residual volume associated with a loss in muscle strength has been found [6] but with no increase in airway resist ance (Raw).…”

Correlations of Egen Klassifikation and Barthel Index scores with pulmonary function parameters in Duchenne muscular dystrophy

“…Previous studies investigated the presence of correlations between some functional assessment scales, such as the scale of Vignos and pulmonary function parameters. [9][10][11] Although statistical analysis was poor or absent, these investigations strongly suggested a relationship between worse functional classes and pulmonary function deterioration. Clear, statistically significant correlations between worse respiratory parameters and a general functional scale have been described only for the EK scale in a small group of patients with DMD and spinal muscular atrophy.…”

“…8 Although these instruments were designed to evaluate functional impairment caused by peripheral muscle dysfunction, a correlation between worse functional classes and reduced forced vital capacity (FVC) and total lung capacity has been suggested. [9][10][11] More recently, an additional instrument was developed to evaluate the functional status of patients with advanced DMD and spinal muscular atrophy. 12,13 The Egen Klassifikation (EK) scale (Egen Klassifikation translation from Danish: "our own classification") was designed to reflect the progressive loss of physical ability for 10 main tasks: (1) control an electric chair, (2) transfer from the wheelchair, (3) stand, (4) sit up, (5) use the arms, (6) use the arms for eating, (7) turn in bed, (8) cough, (9) talk, and (10) general well-being.…”