A continuous repetitive task to detect fatigability in spinal muscular atrophy

To determine the value of a continuous repetitive task to detect and quantify fatigability as additional dimension of impaired motor function in patients with hereditary proximal spinal muscular atrophy (SMA). In this repeated measure case-control study 52 patients with SMA types 2-4, 17 healthy and...

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Veröffentlicht in:Orphanet journal of rare diseases 2018-09, Vol.13 (1), p.160-160, Article 160
Hauptverfasser: Stam, Marloes, Wadman, Renske I, Bartels, Bart, Leeuw, Maureen, Westeneng, Henk-Jan, Wijngaarde, Camiel A, van den Berg, Leonard H, van der Pol, W Ludo
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Sprache:eng
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Zusammenfassung:To determine the value of a continuous repetitive task to detect and quantify fatigability as additional dimension of impaired motor function in patients with hereditary proximal spinal muscular atrophy (SMA). In this repeated measure case-control study 52 patients with SMA types 2-4, 17 healthy and 29 disease controls performed five consecutive rounds of the Nine-Hole Peg test to determine the presence of fatigability. We analysed differences in test performance and associations with disease characteristics. Five patients with SMA type 2 (22%) and 1 disease control (3%) could not finish five rounds due to fatigue (p = 0.01). Patients with SMA type 2 performed the test significantly more slowly than all other groups (p 
ISSN:1750-1172
1750-1172
DOI:10.1186/s13023-018-0904-5