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Muscle strength and motor function in children and adolescents with spinal muscular atrophy II and III.

Kroksmark, Anna-Karin (author)
Gothenburg University,Göteborgs universitet,Institutionen för kvinnors och barns hälsa,Institute for the Health of Women and Children
Beckung, Eva, 1950 (author)
Gothenburg University,Göteborgs universitet,Institutionen för kvinnors och barns hälsa,Institute for the Health of Women and Children
Tulinius, Mar, 1953 (author)
Gothenburg University,Göteborgs universitet,Institutionen för kvinnors och barns hälsa,Institute for the Health of Women and Children
 (creator_code:org_t)
Elsevier BV, 2001
2001
English.
In: European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society. - : Elsevier BV. - 1090-3798. ; 5:5, s. 191-8
  • Journal article (peer-reviewed)
Abstract Subject headings
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  • The aim of this study was to quantify isometric muscle strength and motor function in children and adolescents with spinal muscular atrophy (SMA) and to analyse the impact of reduced muscle strength on motor function. Six children and adolescents with SMA II and eight with SMA IlI were assessed regarding isometric muscle strength and motor function. Isometric muscle strength was tested with a myometer and the values obtained were compared with normative data. Motor function was videotaped and 20 movements were scored according to a three-point scale. All of the assessed children and adolescents with SMA II and SMA III showed reduced muscle strength, but there were great differences within the group. The typical pattern of muscle weakness in SMA, with proximal weakness greater than distal and the lower limbs more affected than the upper, was also seen in these children. The muscle weakness affected motor function in all assessed children. Walking, transfer from lying or sitting to the standing position and stair-climbing were possible in some of the children, despite marked reduction of muscle strength. The study increases our knowledge concerning the degree of muscle weakness in children with SMA and the impact of muscle weakness on motor function. The results increase our possibilities of understanding the prerequisites for everyday life in these children and planning therapeutic interventions. Repeated assessments with the methods used in this study may be used to monitor the course of the disease and to evaluate the efficacy of treatment.

Subject headings

SAMHÄLLSVETENSKAP  -- Annan samhällsvetenskap -- Övrig annan samhällsvetenskap (hsv//swe)
SOCIAL SCIENCES  -- Other Social Sciences -- Other Social Sciences not elsewhere specified (hsv//eng)

Keyword

Activities of Daily Living
classification
Adolescent
Child
Child
Preschool
Female
Humans
Isometric Contraction
physiology
Male
Motor Skills
physiology
Muscle Weakness
diagnosis
physiopathology
Neurologic Examination
Spinal Muscular Atrophies of Childhood
diagnosis
physiopathology

Publication and Content Type

ref (subject category)
art (subject category)

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Kroksmark, Anna- ...
Beckung, Eva, 19 ...
Tulinius, Mar, 1 ...
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SOCIAL SCIENCES
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University of Gothenburg

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