Research Topics
| Douglas M SprouleSummaryAffiliation: Columbia University Country: USA Publications
| Collaborators
|
Detail Information
Publications
Mitochondrial encephalopathy, lactic acidosis, and strokelike episodes: basic concepts, clinical phenotype, and therapeutic management of MELAS syndromeDouglas M Sproule
Columbia University, Pediatric Neurology, 180 Fort Washington Ave, Harkness Pavilion, 5th Floor, New York, NY 10032, USA
Ann N Y Acad Sci 1142:133-58. 2008..Although therapeutic options for MELAS and other mitochondrial diseases remain limited, and recent trials have been disappointing, we also consider current and potential therapeutic modalities...
Increased fat mass and high incidence of overweight despite low body mass index in patients with spinal muscular atrophyDouglas M Sproule
Division of Pediatric Neurosciences, Department of Neurology, SMA Clinical Research Center, Columbia University Medical Center, Harkness Pavilion, HP 514, 180 Fort Washington Avenue, New York, NY 10032 3791, USA
Neuromuscul Disord 19:391-6. 2009..Children with SMA have reduced lean and increased fat mass compared to healthy children. Obesity is a potentially important modifiable source of morbidity in SMA...
Bioelectrical impedance analysis can be a useful screen for excess adiposity in spinal muscular atrophyDouglas M Sproule
Division of Pediatric Neurosciences, Department of Neurology, SMA Clinical Research Center, Columbia University Medical Center, New York, New York 10032 3791, USA
J Child Neurol 25:1348-54. 2010..Although insufficiently accurate for use as a research tool, bioelectrical impedance can have application as a well-tolerated, noninvasive, easily used screening tool for excess adiposity in patients with spinal muscular atrophy...
Adiposity is increased among high-functioning, non-ambulatory patients with spinal muscular atrophyDouglas M Sproule
Division of Pediatric Neurosciences, Department of Neurology, SMA Clinical Research Center, Columbia University Medical Center, New York, NY 10032 3791, USA
Neuromuscul Disord 20:448-52. 2010....
Thigh muscle volume measured by magnetic resonance imaging is stable over a 6-month interval in spinal muscular atrophyDouglas M Sproule
Division of Pediatric Neurosciences, Department of Neurology, SMA Clinical Research Center, Columbia University Medical Center, New York, NY 10032 3791, USA
J Child Neurol 26:1252-9. 2011..Relative constancy of muscle volume estimation and correlation with established functional measures suggest a role for segmental magnetic resonance imaging as a biomarker of treatment effect in future therapeutic trials...
Muscle volume estimation by magnetic resonance imaging in spinal muscular atrophyDouglas M Sproule
Division of Pediatric Neurosciences, Department of Neurology, SMA Clinical Research Center, Columbia University Medical Center, New York, New York 10032 3791, USA
J Child Neurol 26:309-17. 2011..14) than type 3. Reproducibility, tolerability, and strong correlation with clinical measures make magnetic resonance imaging a candidate biomarker for clinical research...
Age at disease onset predicts likelihood and rapidity of growth failure among infants and young children with spinal muscular atrophy types 1 and 2Douglas M Sproule
Division of Pediatric Neurosciences, Department of Neurology, SMA Clinical Research Center, Columbia University Medical Center, New York, NY 10032 3791, USA
J Child Neurol 27:845-51. 2012..Most late onset (> 6 months) subjects avoided growth failure. Early clinical symptoms predict feeding dysfunction and growth failure. Immediate, proactive nutritional intervention is indicated for patients with early symptom onset...
Independent mobility after early introduction of a power wheelchair in spinal muscular atrophySally Dunaway
1Neurology Department, Columbia University Medical Center, New York, NY, USA
J Child Neurol 28:576-82. 2013..9 months (range: 73-458 days). Introduction of early power mobility is feasible in spinal muscular atrophy patients under age 2 years and should be introduced in late infancy when children typically acquire locomotor skills...
Prospective cohort study of spinal muscular atrophy types 2 and 3Petra Kaufmann
Department of Neurology, Columbia University, New York, USA
Neurology 79:1889-97. 2012..To characterize the natural history of spinal muscular atrophy type 2 and type 3 (SMA 2/3) beyond 1 year and to report data on clinical and biological outcomes for use in trial planning...
Spinal muscular atrophy type III: trying to understand subtle functional change over time--a case reportSally Dunaway
SMA Clinical Research Center, Columbia University Medical Center, New York, NY 10032, USA
J Child Neurol 27:779-85. 2012..However, she walked with assistance 2 years longer than expected. Our report highlights possible precipitating factors that could affect the natural history of spinal muscular atrophy type III...
Monitoring of cerebral vasodilatory capacity with transcranial Doppler carbon dioxide inhalation in patients with severe carotid artery diseaseRandolph S Marshall
The Neurological Institute, Columbia University, College of Physicians and Surgeons, New York, NY 10032, USA
Stroke 34:945-9. 2003....
Wolff-Parkinson-White syndrome in Patients With MELASDouglas M Sproule
Division of Pediatric Neurology, Department of Neurology, Columbia University, New York, NY, USA
Arch Neurol 64:1625-7. 2007..Tissues with high energy demands, such as the heart, are susceptible to the effects of mitochondrial DNA point mutations...
Therapeutic developments in spinal muscular atrophyDouglas M Sproule
Division of Pediatric Neurosciences, Department of Neurology, SMA Clinical Research Center, Columbia University Medical Center, Harkness Pavilion, HP 514, 180 Fort Washington Avenue, New York, NY 10032 3791, USA
Ther Adv Neurol Disord 3:173-85. 2010..The recent advances in preclinical research and the development of a wider range of animal models for SMA continue to provide cautious optimism that effective treatments for SMA will eventually emerge...
Scoliosis surgery in children with neuromuscular disease: findings from the US National Inpatient Sample, 1997 to 2003Alexandra I Barsdorf
Division of Pediatric Neurosciences, Department of Neurology, Spinal Muscular Atrophy Clinical Research Center, Columbia University Medical Center, 180 Fort Washington Ave, New York, NY 10032 3791, USA
Arch Neurol 67:231-5. 2010..Scoliosis is a frequent complication of pediatric neuromuscular disease (NMD). Scoliosis surgery in children with NMD is thought to carry greater morbidity and mortality...
