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Milev, Evelin; Selby, Victoria; Wolfe, Amy; Rohwer, Annemarie; Tillmann, Ricarda; Ramsey, Danielle; Iodice, Mario; Hogrel, Jean‐Yves; Baranello, Giovanni; Scoto, Mariacristina; Muntoni, Francesco
Muscle & nerve, March 2024, Letnik: 69, Številka: 3Journal Article
Introduction/Aims Current upper limb assessments in pediatric spinal muscular atrophy (SMA) may not adequately capture change with disease progression. Our aim was to examine the relationship between motor function, strength, and hand/finger mobility of the upper limb in treatment‐naïve children with SMA Types 2 and 3 to assess new methods to supplement current outcomes. Methods The Revised Upper Limb Module (RULM), grip and pinch strength, and hand/finger mobility data were collected from 19 children with SMA Types 2 and 3 aged 5.2–16.9 years over a year. Results A median loss between 0.5 and 2.5 points in the RULM was seen across all SMA subgroups with the biggest median loss recorded between 10 and 14 years of age. The grip strength loss was −0.06 kg (−4.69 to 3.49; IQR, 1.21); pinch improvement of 0.05 (−0.65 to 1.27; IQR, 0.48); hand/finger mobility test improvement of 4 points (−24 to 14; IQR, 6.75) for the whole cohort. Significant correlations were found between the RULM and grip strength (p < .001), RULM and pinch strength (p < .001), RULM and revised Brooke (p < .001), grip strength and pinch strength (p < .001). Discussion The combined use of the RULM, dynamometry, and hand mobility provide insight about correlations between function and strength in children with SMA. The RULM and grip strength assessments captured a significant decline in upper limb function, whereas the pinch and finger/hand mobility showed an improvement over the course of 1 year and these results should be considered for future studies. Upper limb function, strength, and hand/finger mobility in treatment‐naïve children with spinal muscular atrophy Types 2 and 3 were assessed by the Revised Upper Limb Module (RULM) and high‐precision dynamometry.
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JCR | SNIP | JCR | SNIP | JCR | SNIP | JCR | SNIP |
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Vir: Osebne bibliografije
in: SICRIS
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