S Sl le ee ep p--r re el la at te ed d r re es sp pi ir ra at to or ry y d di is st tu ur rb ba an nc ce es s i in n p pa at ti ie en nt ts s w wi it th h D Du uc ch he en nn ne e m mu us sc cu ul la ar r d dy ys st tr ro op ph hy y Four patients (67%) showed symptoms that suggest sleep-related respiratory disturbances. At night, the apnoea-hypopnoea index (AHI) was 11±6. The patients with more symptoms during the daytime had the highest AHI scores. Most of the apnoeas (85%) were central, particularly during rapid eye movement (REM) sleep. Sleep architecture was well-preserved. Arterial desaturation (>5% below baseline) occurred during 25±23% of total time. AHI correlated with daytime PaO 2 , and AHI in REM sleep correlated with age. A stepwise multivariate analysis showed that PaO 2 and, to some extent, the degree of airflow obstruction were significantly correlated with AHI.We conclude that sleep-related respiratory disturbances are frequently present in patients with Duchenne muscular dystrophy. Therefore, physicians should look for symptoms related to sleep-related respiratory disturbances in these patients. Furthermore, sleep-related respiratory disturbances should be strongly suspected in older Duchenne muscular dystrophy patients, particularly if diurnal arterial hypoxaemia is concurrently present.

Twenty consecutive patients (16 women and 4 men), with a mean age of 40 years, who were diagnosed and treated for myasthenia gravis were enrolled in a prospective investigation aimed at determining the amount of respiratory disturbance occurring during sleep while they received treatment. Patients were clinically evaluated to determine body mass index, presence of upper airway anatomical abnormalities, level of functional capacity and activity scored from 1 to 5, and presence of sleep-related complaints. They underwent daytime pulmonary function tests, determination of maximal static inspiratory pressure, measurement of transdiaphragmatic pressure, and measurement of arterial blood gas levels. Polygraphic monitoring during sleep, evaluating respiration and oxygen saturation, was also performed. Results indicated that in the studied population, all subjects had evidence of daytime diaphragmatic weakness as demonstrated by transdiaphragmatic pressure measurements, independent of the degree of autonomy and functional capacity and activity level reached. Older patients with moderately increased body mass index, abnormal total lung capacity, and abnormal daytime blood gas concentrations were the primary candidates for development of diaphragmatic sleep apneas and hypopneas, and oxygen desaturation of less than 90% during sleep. However, these clear indicators were not found in all subjects with sleep-related disordered breathing. Rapid-eye-movement sleep was the time of highest breathing vulnerability during sleep. Sleep-related complaints may also help identify subjects at risk for abnormal breathing during sleep, even when daytime functional activity is judged normal.

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To determine whether electrophysiological features predict endotracheal mechanical ventilation (ETMV) in Guillain-Barré syndrome (GBS). Non-ventilated GBS patients admitted to an ICU underwent standard electrophysiological testing. Endotracheal mechanical ventilation was decided by physicians who were unaware of electrophysiological results. Sixty consecutive patients underwent electrophysiological testing within 17 days of GBS onset; based on Hadden's criteria, 37 (62%) had primary demyelinating, 18 (30%) equivocal and five (8%) axonal disease. Time at electrophysiological testing and proportions of patients treated by plasma exchange and intravenous immunoglobulins were similar in the three groups, whereas primary demyelinating patients had worse results for disability grade and arm grade. The ETMV was required within 20 days of electrophysiological testing in 20 patients, 17 (46%) in the primary demyelinating group, three (17%) in equivocal group and none in the axonal group (P = 0.02). This prospective study suggests that electrophysiological demyelination may predict a need for ETMV in GBS.

Clinical improvement has been observed in myasthenia gravis patients treated by intravenous immunoglobulin (IVIg). In order to investigate the mechanism of action of these IVIg, we looked for an in vitro interaction between IVIg and the anti-acetylcholine receptor autoantibodies. Significant inhibition by IVIg of anti-acetylcholine receptor autoantibody activity from 30 MG sera was observed and binding of anti-acetylcholine receptor autoantibodies on IVIg was found for four of five myasthenia gravis sera. These observations suggest that IVIg contains Ig directly binding to and inhibiting pathogenic autoantibodies.