Details

Autor(en) / Beteiligte

• 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

Titel

A continuous repetitive task to detect fatigability in spinal muscular atrophy

Ist Teil von

• Orphanet journal of rare diseases, 2018-09, Vol.13 (1), p.160-160, Article 160

Ort / Verlag

England: BioMed Central Ltd

Erscheinungsjahr

2018

Link zum Volltext

Quelle

EZB-FREE-00999 freely available EZB journals

Beschreibungen/Notizen

• 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 < 0.005) and disease controls were slower than healthy controls (p < 0.05). Patients with SMA type 2 performed round five 27% slower than round one, while healthy controls performed round five 14% faster than round one (p = 0.005). There was no difference between SMA type 3a, type 3b/4 or disease controls and healthy controls (p > 0.4). Time needed to complete each round during the five-round task increased in 15 patients with SMA type 2 (65%), 4 with type 3a (36%), 4 with type 3b/4 (22%), 9 disease controls (31%) and 1 healthy control (6%). There was no effect of age at disease onset or disease duration in SMA type 2 (p = 0.39). Test-retest reliability was high. Fatigability of remaining arm function is a feature of SMA type 2 and can be determined with continuous repetitive tasks.