Publications by authors named "Jeanine Schierbecker"
Introduction: Outcomes sensitive to change over time in non-ambulatory boys/men with Duchenne muscular dystrophy (DMD) are not well-established.
Methods: Subjects (n = 91; 16.8 ± 4.
Introduction: Therapeutic trials in Duchenne muscular dystrophy (DMD) often exclude non-ambulatory individuals. Here we establish optimal and reliable assessments in a multicenter trial.
Methods: Non-ambulatory boys/men with DMD (N = 91; 16.
Article Synopsis
- Traditional outcome measures in DMD trials exclude young boys due to reliance on cooperation, but alternative assessments like the Bayley III, HFMSE, and NSAA show promise.
- A study assessed 24 infants and boys with DMD, revealing significantly low scores in motor, cognitive, and language development compared to typical children, indicating developmental delays.
- Results suggest that outcome assessments for young boys with DMD are feasible, with the Bayley III emerging as the most effective measure in this multicenter study.
Introduction: The Accurate Test of Limb Isometric Strength (ATLIS) device can reliably measure the strength of 12 muscle groups using a fixed load cell. The purpose of this study was to analyze ATLIS data from healthy adults to calculate an individual's predicted strength scores.
Methods: ATLIS data were collected from 432 healthy adults.
We describe a large family with amyotrophic lateral sclerosis (ALS) caused by an I113T mutation in superoxide dismuatse type 1 (SOD1). The proband developed symptoms typical for ALS at age 39 years and is still walking five years later. Marked phenotypic variability is manifested by her mother with onset of gait difficulty and decision-making problems at age 67 years and a five-year course marked by progressive mild upper motor neuron weakness, frontotemporal dementia and chorea.
View Article and Find Full Text PDFDaily prednisone improves strength in boys with Duchenne muscular dystrophy, but side effects are almost universal. We used a different dosing regimen of prednisone to determine if benefit to boys with Duchenne muscular dystrophy might be maintained with fewer side effects. Twice weekly oral prednisone was given each Friday and Saturday (5mg/kg/dose).
View Article and Find Full Text PDF