title: Strength: a relevant link to functional performance in the neurodegenerative disease of adrenomyeloneuropathy.
abstract: BACKGROUND
  With progressive abnormalities in leg strength, tone, and sensation, adrenomyeloneuropathy (AMN) is a differential diagnosis for multiple sclerosis and hereditary spastic paraparesis. AMN pathology has been linked to weakness, making it a relevant model to evaluate the relationship between neurodegeneration and disability. Quantifying symptom severity in AMN is essential for treatment development in rehabilitative management.
  
  
  OBJECTIVE
  To identify deficits in body functions, activity, and participation of people with AMN and provide a practical framework for evaluating the severity of disability.
  
  
  METHODS
  Cohort analysis of 142 participants with AMN.
  
  
  METHODS
  of body functions (leg strength, vibration sensation, range of motion, and spasticity), activity (walk velocity, standing balance, Timed Up and Go, and Sit-to-Stand Time), and participation (6-Minute Walk) are evaluated. Regression analyses identify relationships between the measures. A staging framework (mild, moderate, and severe) reflects the continuum of disability. Finally, an analysis of variance/Kruskal-Wallis was used for between-stage and sex differences among the variables.
  
  
  RESULTS
  Strength is the strongest correlate for the 5 measures of activity and participation. Staging based on weakness distinguishes 3 levels of severity along a continuum of disability. Differences between the sexes are more prevalent earlier in the continuum but show equally severe deficits in the last stage.
  
  
  CONCLUSIONS
  In AMN, staging based on degrees of weakness provides a practical means to characterize the severity of common deficits in body functions as well as activity and participation at each stage, to direct the evaluation. Such information could help clinicians develop more effective rehabilitative techniques.
authors.affiliation: Kennedy Krieger Institute, Baltimore, MD, USA.
authors.forename: Jennifer L
authors.initials: JL
authors.lastname: Keller
authors.forename: Joseph I
authors.initials: JI
authors.lastname: Wang
authors.forename: Jonathan Y
authors.initials: JY
authors.lastname: Kang
authors.forename: Joseph A
authors.initials: JA
authors.lastname: Hanson
authors.forename: Priya
authors.initials: P
authors.lastname: Kamath
authors.forename: Jennifer O
authors.initials: JO
authors.lastname: Swain
authors.forename: Gerald V
authors.initials: GV
authors.lastname: Raymond
authors.forename: Kathleen M
authors.initials: KM
authors.lastname: Zackowski
mesh_terms: D000326:Adrenoleukodystrophy / Q000150:complications / Q000503:physiopathology* / Q000534:rehabilitation*; D000328:Adult; D000842:Ankle / Q000502:physiology; D003430:Cross-Sectional Studies; D003627:Data Interpretation, Statistical; D004185:Disability Evaluation; D004311:Double-Blind Method; D004338:Drug Combinations; D004883:Erucic Acids / Q000627:therapeutic use; D005260:Female; D006225:Hand / Q000502:physiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009119:Muscle Contraction / Q000502:physiology; D053580:Muscle Strength / Q000502:physiology*; D018908:Muscle Weakness / Q000209:etiology / Q000503:physiopathology*; D019636:Neurodegenerative Diseases / Q000150:complications / Q000503:physiopathology* / Q000534:rehabilitation*; D012044:Regression Analysis; D012684:Sensory Thresholds / Q000502:physiology; D014110:Touch / Q000502:physiology; D016896:Treatment Outcome; D014304:Triolein / Q000627:therapeutic use
chemical_list: D004338:Drug Combinations; D004883:Erucic Acids; C079420:Lorenzo's oil; D014304:Triolein