Strength: A relevant link to functional performance in the neurodegenerative disease of adrenomyeloneuropathy

Jennifer L. Keller, Joseph I. Wang, Jonathan Y. Kang, Joseph A. Hanson, Priya Kamath, Jennifer O. Swain, Gerald Raymond, Kathleen M. Zackowski

Research output: Contribution to journalArticle

5 Citations (Scopus)

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. Measures 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.

Original languageEnglish (US)
Pages (from-to)1080-1088
Number of pages9
JournalNeurorehabilitation and Neural Repair
Volume26
Issue number9
DOIs
StatePublished - Nov 1 2012

Fingerprint

Adrenoleukodystrophy
Neurodegenerative Diseases
Sex Characteristics
Leg
Spastic Paraparesis
Body Weights and Measures
Articular Range of Motion
Vibration
Multiple Sclerosis
Analysis of Variance
Differential Diagnosis
Cohort Studies
Regression Analysis
Pathology

All Science Journal Classification (ASJC) codes

  • Rehabilitation
  • Neurology
  • Clinical Neurology

Cite this

Keller, J. L., Wang, J. I., Kang, J. Y., Hanson, J. A., Kamath, P., Swain, J. O., ... Zackowski, K. M. (2012). Strength: A relevant link to functional performance in the neurodegenerative disease of adrenomyeloneuropathy. Neurorehabilitation and Neural Repair, 26(9), 1080-1088. https://doi.org/10.1177/1545968312441682
Keller, Jennifer L. ; Wang, Joseph I. ; Kang, Jonathan Y. ; Hanson, Joseph A. ; Kamath, Priya ; Swain, Jennifer O. ; Raymond, Gerald ; Zackowski, Kathleen M. / Strength : A relevant link to functional performance in the neurodegenerative disease of adrenomyeloneuropathy. In: Neurorehabilitation and Neural Repair. 2012 ; Vol. 26, No. 9. pp. 1080-1088.
@article{f17f51a744894b5e81e504049d71f84d,
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. Measures 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.",
author = "Keller, {Jennifer L.} and Wang, {Joseph I.} and Kang, {Jonathan Y.} and Hanson, {Joseph A.} and Priya Kamath and Swain, {Jennifer O.} and Gerald Raymond and Zackowski, {Kathleen M.}",
year = "2012",
month = "11",
day = "1",
doi = "10.1177/1545968312441682",
language = "English (US)",
volume = "26",
pages = "1080--1088",
journal = "Neurorehabilitation and Neural Repair",
issn = "1545-9683",
publisher = "SAGE Publications Inc.",
number = "9",

}

Strength : A relevant link to functional performance in the neurodegenerative disease of adrenomyeloneuropathy. / Keller, Jennifer L.; Wang, Joseph I.; Kang, Jonathan Y.; Hanson, Joseph A.; Kamath, Priya; Swain, Jennifer O.; Raymond, Gerald; Zackowski, Kathleen M.

In: Neurorehabilitation and Neural Repair, Vol. 26, No. 9, 01.11.2012, p. 1080-1088.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Strength

T2 - A relevant link to functional performance in the neurodegenerative disease of adrenomyeloneuropathy

AU - Keller, Jennifer L.

AU - Wang, Joseph I.

AU - Kang, Jonathan Y.

AU - Hanson, Joseph A.

AU - Kamath, Priya

AU - Swain, Jennifer O.

AU - Raymond, Gerald

AU - Zackowski, Kathleen M.

PY - 2012/11/1

Y1 - 2012/11/1

N2 - 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. Measures 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.

AB - 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. Measures 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.

UR - http://www.scopus.com/inward/record.url?scp=84867289319&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84867289319&partnerID=8YFLogxK

U2 - 10.1177/1545968312441682

DO - 10.1177/1545968312441682

M3 - Article

C2 - 22544816

AN - SCOPUS:84867289319

VL - 26

SP - 1080

EP - 1088

JO - Neurorehabilitation and Neural Repair

JF - Neurorehabilitation and Neural Repair

SN - 1545-9683

IS - 9

ER -