Positive airway pressure for sleep-disordered breathing in acute quadriplegia: a randomised controlled trial.

RATIONALE: Highly prevalent and severe sleep-disordered breathing caused by acute cervical spinal cord injury (quadriplegia) is associated with neurocognitive dysfunction and sleepiness and is likely to impair rehabilitation. OBJECTIVE: To determine whether 3 months of autotitrating CPAP would improve neurocognitive function, sleepiness, quality of life, anxiety and depression more than usual care in acute quadriplegia. METHODS AND MEASUREMENTS: Multinational, randomised controlled trial (11 centres) from July 2009 to October 2015. The primary outcome was neurocognitive (attention and information processing as measure with the Paced Auditory Serial Addition Task). Daytime sleepiness (Karolinska Sleepiness Scale) was a priori identified as the most important secondary outcome. MAIN RESULTS: 1810 incident cases were screened. 332 underwent full, portable polysomnography, 273 of whom had an apnoea hypopnoea index greater than 10. 160 tolerated at least 4 hours of CPAP during a 3-day run-in and were randomised. 149 participants (134 men, age 46±34 years, 81±57 days postinjury) completed the trial. CPAP use averaged 2.9±2.3 hours per night with 21% fully 'adherent' (at least 4 hours use on 5 days per week). Intention-to-treat analyses revealed no significant differences between groups in the Paced Auditory Serial Addition Task (mean improvement of 2.28, 95% CI -7.09 to 11.6; p=0.63). Controlling for premorbid intelligence, age and obstructive sleep apnoea severity (group effect -1.15, 95% CI -10 to 7.7) did not alter this finding. Sleepiness was significantly improved by CPAP on intention-to-treat analysis (mean difference -1.26, 95% CI -2.2 to -0.32; p=0.01). CONCLUSION: CPAP did not improve Paced Auditory Serial Addition Task scores but significantly reduced sleepiness after acute quadriplegia. TRIAL REGISTRATION NUMBER: ACTRN12605000799651.

Heart rate and perceived muscle pain responses to a functional walking test in McArdle disease.

The aim of this study was to assess a 12-min self-paced walking test in patients with McArdle disease. Twenty patients (44.7 ± 11 years; 11 female) performed the walking test where walking speed, distance walked, heart rate (HR) and perceived muscle pain (Borg CR10 scale) were measured. Median (interquartile range) distance walked was 890 m (470-935). From 1 to 6 min, median walking speed decreased (from 75.0 to 71.4 m∙min(-1)) while muscle pain and %HR reserve increased (from 0.3 to 3.0 and 37% to 48%, respectively). From 7 to 12 min, walking speed increased to 74.2 m∙min(-1), muscle pain decreased to 1.6 and %HR reserve remained between 45% and 48%. To make relative comparisons, HR and muscle pain were divided by walking speed and expressed as ratios. These ratios rose significantly between 1 and 6 min (HR:walking speed P = .001 and pain:walking speed P < .001) and similarly decreased between 6 and 11 min (P = .002 and P = .001, respectively). Peak ratios of HR:walking speed and pain:walking speed were inversely correlated to distance walked: rs (HR) = -.82 (P < .0001) and rs (pain) = -.55 (P = .012). Largest peak ratios were found in patients who walked < 650 m. A 12-min walking test can be used to assess exercise capacity and detect the second wind in McArdle disease.

The relation of thoracic and lumbar fracture configuration to the development of late deformity in childhood spinal cord injury.

STUDY DESIGN: A retrospective clinical observational study was conducted. OBJECTIVE: To assess the relation of spinal fracture type and its magnitude of distortion to subsequent long-term development of late spinal deformity in childhood onset spinal cord injury. SUMMARY OF BACKGROUND DATA: In this study, 76 adults who sustained spinal cord injury during childhood were examined clinically and radiographically alongside a retrospective review of case notes and radiographs. METHODS: The nature of the spinal injury and the progression of its displacement were defined from radiographs taken immediately after injury, then at 4 months and at 1 year. Eventual adult spinal deformity was defined from standardized erect long-plate radiographs. Scoliosis, kyphosis, and lordosis were measured using Cobb's method. RESULTS: There was no statistically significant difference in the severity of scoliosis, kyphosis, or lordosis between traumatic and nontraumatic injuries, nor between patients with and those without radiologically visible bony injury. Of the 14 patients with traumatic thoracic and lumbar injuries who had undergone no surgical intervention, 10 (71%) showed development of major scoliotic curves that did not include the fracture site. The patients with no angular displacement at the fracture site after 1 year went on to experience the development of more severe scoliosis (mean, 66 degrees) than those who had displaced fractures (mean, 38 degrees). In five, a low kyphotic curve and a compensatory lordosis above it developed. CONCLUSIONS: There is no evidence that the bony injury to the vertebral column itself in the child with spinal cord injury influences the development of late scoliosis or lordosis, but it may influence any eventual kyphosis.