Document Detail


Physical capacity in non-ambulatory people with Duchenne muscular dystrophy or spinal muscular atrophy: a longitudinal study.
MedLine Citation:
PMID:  12227617     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The purpose of this study was to describe functional ability, muscle strength, forced vital capacity, and clinical events in participants with Duchenne muscular dystrophy (DMD) or spinal muscular atrophy (SMA) in the non-ambulatory stages of the diseases. Nineteen non-ambulatory participants with DMD (all males; 13 to 24 years) and 13 with SMA (six males, seven females; 11 to 57 years) were assessed once a year over 5 years. The assessments comprised functional ability measured with the EK scale and upper extremity grade, muscle strength measured with the manual muscle test, and forced vital capacity defined as a percentage of normal values (FVC%). In the DMD group all variables measured deteriorated and there was a direct correlation between them. In the SMA group only muscle strength and FVC% deteriorated and there was no close relation between the variables measured. In the DMD group, 16 participants had cardiorespiratory clinical events leading to death in five cases. In the SMA group only four participants had respiratory clinical events, none leading to death. Although the participants with SMA had been extremely weak and non-ambulatory since early childhood they were older and less exposed to life-threatening events than the participants with DMD.
Authors:
Birgit F Steffensen; Søren Lyager; Birgit Werge; Jes Rahbek; Eva Mattsson
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Developmental medicine and child neurology     Volume:  44     ISSN:  0012-1622     ISO Abbreviation:  Dev Med Child Neurol     Publication Date:  2002 Sep 
Date Detail:
Created Date:  2002-09-13     Completed Date:  2002-09-24     Revised Date:  2009-11-11    
Medline Journal Info:
Nlm Unique ID:  0006761     Medline TA:  Dev Med Child Neurol     Country:  England    
Other Details:
Languages:  eng     Pagination:  623-32     Citation Subset:  IM    
Affiliation:
Institute for Neuromuscular Diseases, Arhus, Denmark. bist@muskelsvindfonden.dk
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MeSH Terms
Descriptor/Qualifier:
Activities of Daily Living*
Adolescent
Adult
Child
Disabled Persons*
Female
Humans
Longitudinal Studies
Male
Middle Aged
Muscle Weakness
Muscular Atrophy, Spinal / complications*,  pathology
Muscular Dystrophy, Duchenne / complications*,  pathology
Vital Capacity

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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