Postural breathing pattern changes in patients with myotonic dystrophy

We recorded by pneumotachography the breathing in nine patients with myotonic dystrophy (MD), both seated and supine and with eyes open in both positions. Irregular breathing (coefficient of variation > 20% for VT and TTOT) was observed in six of the patients, two of whom showed irregularity in both positions whilst the remaining four had irregular breathing only when supine. In addition, in this latter group, irregularities first appeared in VT and only after a few minutes in TTOT. Whereas in the group exhibiting irregular breathing in both seated and supine positions, irregularities were observed throughout the recording. However, no significant difference in any ventilatory variable was observed as between the two postures. Rib cage (RC) and abdomen (AB) motions were recorded by uncalibrated respiratory inductance plethysmography. Although for MD patients the mean values of the RC/AB ratio lay within the normal range the relative decrease in value as between seated (0.78 +/- 0.52) and supine (0.31 +/- 0.13) position was less than in healthy subjects. These observations suggest that MD may cause deficiencies in several mechanisms. Analyses of the respiratory pattern in each patient may provide information leading to the identification of the impaired respiratory mechanisms. (C) 2000 Elsevier Science B.V. All rights reserved.