Abstract:The frequency of motor vehicle and working accidents was analyzed by means of a strictly anomymous questionnaire in 156 patients with sleep apnea syndrome (SAS) and in 160 age-gender matched controls. In the SAS group 12.4% of all drivers had motor vehicle accidents as compared to 2.9% in the control group (p<0.005). The motor vehicle accident rate was 13.0 per million km in patients with more severe SAS (AHI > 34/h, n=78) as compared to 1.1 in patients with milder SAS (AHI 10-34/h, n=78) (p<0.05), and 0.78 in control group (p<0.005), respectively. The accident rates in both patients and the control group were also greater than the rate of 0.02 "accidents due to sleepiness" per one million km in the Swiss driving population as reported by official statistics. During treatment with nasal continuous airway pressure (nCPAP) in 85 SAS patients, the motor vehicle accident rate dropped from 10.6 to 2.7 per million km (p<0.05). We conclude that patients with moderate to severe SAS have an up to fifteen-fold risk increase of motor vehicle accidents that constitutes a serious and often underestimated hazard on the roads, which can be reduced by adequate treatment.

Clinical, neurophysiological, morphological and biochemical investigations were performed in 2 patients with the adult form of glycogenosis II and related to the findings of 58 well-documented cases published in the literature. According to these findings three types can be distinguished from each other. The first one is characterized by an involvement of the limb-girdle muscles only. The second type shows the same pattern with additional progressive insufficiency of the respiratory muscles. The third type presents with weakness of the respiratory muscles without any other severe muscle involvement. Our case 1 can be related to the first, our case 2 to the second type. EMG-studies in case 1 showed myopathic changes and myotonic discharges without clinical signs of myotonia. A myotonic pattern was described in one third of the published cases. In case 2 neurogenic changes as well as in 4 cases in the literature were found. The muscle biopsy is the diagnostic clue in the differential diagnosis of progressive myopathy in the adult. Patients with glycogenosis II show glycogen storage specially in type I-fibres. The enzyme defect can be confirmed biochemically in muscle tissue or cultured fibroblasts. Various therapeutic concepts have been tried in patients with glycogenosis II but most of them remain disappointing. A diet with a low carbohydrate and a high protein proportion was observed to be of some benefit. In patients with respiratory muscle involvement artificial ventilation support showed a positive effect on the general condition for some time.