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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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Plasma exchange is contraindicated in 10 to 20% of patients with Guillain-Barré syndrome (GBS). The optimal schedule for intravenous immune globulin (IVIg) therapy has not yet been established in these patients.The objective was to compare the eYcacy and safety of two IVIg treatment durations in patients with GBS with contraindications for plasma exchange.In this randomised, double blind, multicentre phase II trial conducted in seven French centres, patients with GBS with severe haemostasis, unstable haemodynamics, or uncontrolled sepsis were randomly assigned to 0.4 g/kg/day IVIg for 3 or 6 days. The primary outcome measure was the time needed to regain the ability to walk with assistance.Thirty nine patients were included from March 1994 to May 1997, 21 in the 3 day group and 18 in the 6 day group. Time to walking with assistance was nonsignificantly shorter in the 6 day group (84 (23-121) v 131 days (51-210), p=0.08); the diVerence was significant in ventilated patients (86 days (13-151) in the 6 day group v 152 days (54-332) in the 3 day group; p=0.04). The prevalence and severity of IVIg related adverse eVects were comparable between the two groups.In conclusion, in patients with GBS and contraindications for plasma exchange, especially those who need ventilatory assistance, IVIg (0.4 g/kg/day) may be more beneficial when given for 6 days rather than 3 days. (J Neurol Neurosurg Psychiatry 2001;71:235-238)