[Indicators for an impending need of artificial respiration in patients with muscular dystrophy]

In 1990 we examined 25 patients with Duchenne Muscular Dystrophy (DMD) or Spinal Muscular Atrophy type II (SMA-II). The purpose was to elucidate their general physical capability and find indicators for future need of artificial ventilation. We used interviews and measurements. The interviews were based on classifications described as 1) EAMDA, 2) VIGNOS, 3) BROOKE for use with DMD patients. Our own classification (called EK) is described and used. Moreover, we measured five parameters for the mechanical lung function, the strength of eight great muscle groups, and anthropometrical data. In January 1992, between two and 18 months after our examination, seven of the patients (all DMD) required artificial ventilation. Among the patients who needed artificial ventilation, we found the DMDs to have the lowest physical capability, as measured by the interview (EK and EAMDA) and the smallest values for forced vital capacity (FVC) and peak expiratory flow rate (PEF). All 11 patients with SMA-II could administer their lives without ventilator. Comparing their values with the DMD group, we would expect that several would need artificial ventilation. The reason why the SMA-II patients can manage without artificial ventilation is not clarified. Combining our classification (EK) and the value for FVC, we can predict the need for artificial ventilation among the participating DMD patients.