scholarly journals Measurement of muscle strength with a handheld dynamometer in patients with chronic spinal muscular atrophy

2010 ◽  
Vol 42 (3) ◽  
pp. 228-231 ◽  
Author(s):  
A Febrer ◽  
N Rodriguez ◽  
L Alias ◽  
E Tizzano
Keyword(s):  
Muscle Strength ◽  
Spinal Muscular Atrophy ◽  
Muscular Atrophy ◽  
Chronic Spinal Muscular Atrophy

SMN2 copy number predicts acute or chronic spinal muscular atrophy but does not account for intrafamilial variability in siblings

2005 ◽  
Vol 253 (1) ◽  
pp. 21-25 ◽  
Author(s):  
I. Cuscó ◽  
M. J. Barceló ◽  
R. Rojas–García ◽  
I. Illa ◽  
J. Gámez ◽  
...  
Keyword(s):  
Spinal Muscular Atrophy ◽  
Copy Number ◽  
Muscular Atrophy ◽  
Intrafamilial Variability ◽  
Chronic Spinal Muscular Atrophy

A distal form of chronic spinal muscular atrophy

Neurology ◽  
1969 ◽  
Vol 19 (1) ◽  
pp. 53-53 ◽  
Author(s):  
J. C. Meadows ◽  
C. D. Marsden
Keyword(s):  
Spinal Muscular Atrophy ◽  
Muscular Atrophy ◽  
Chronic Spinal Muscular Atrophy

Muscle strength and motor function in adolescents and adults with spinal muscular atrophy

Neurology ◽  
2020 ◽  
Vol 95 (14) ◽  
pp. e1988-e1998 ◽  
Author(s):  
Camiel A. Wijngaarde ◽  
Marloes Stam ◽  
Louise A.M. Otto ◽  
Bart Bartels ◽  
Fay-Lynn Asselman ◽  
...  
Keyword(s):  
Muscle Strength ◽  
Spinal Muscular Atrophy ◽  
Motor Function ◽  
Older Patients ◽  
Muscular Atrophy ◽  
Compound Muscle Action Potential ◽  
Adult Patients ◽  
Cross Sectional ◽  
Floor Effects ◽  
Adolescents And Adults

ObjectiveTo assess longitudinal patterns of muscle strength, motor function, and maximal compound muscle action potential amplitudes (CMAPMAX) in older patients with spinal muscular atrophy (SMA), hypothesizing a continued decline of motor function parameters throughout life.MethodsWe measured muscle strength (Medical Research Council), motor function (Hammersmith Functional Motor Scale Expanded [HFMSE] and Motor Function Measure), and CMAPMAX in treatment-naive patients. We used both longitudinal and cross-sectional data in mixed models to analyze natural history patterns.ResultsWe included 250 patients with SMA types 1c through 4. Median patient age at assessment was 26.8 years, the number of assessments per patient ranged from 1 to 6. Baseline muscle strength and motor function scores differed significantly between SMA types, but annual rates of decline were largely similar and mostly linear. HFMSE floor effects were present for all patients with SMA type 1c, and adolescents and adults with types 2 and 3a. CMAPMAX differed significantly between SMA types but did not decline significantly with increasing age. Muscle strength correlated very strongly with motor function (τ ≥ 0.8) but only moderately with CMAPMAX (τ ≈ 0.5–0.6).ConclusionMuscle strength and motor function decline in older patients with SMA are constant without periods of slower progression or a plateau phase. The floor effects of the HFMSE preclude its use for long-term follow-up of adult patients with SMA types 1c through 3a. Muscle strength sum scores represent an alternative, feasible outcome measure for adolescent and adult patients with SMA.

A genetic study of subacute and chronic spinal muscular atrophy in childhood

1978 ◽  
Vol 37 (3) ◽  
pp. 227-248 ◽  
Author(s):  
John Pearn ◽  
Sarah Bundey ◽  
C.O. Carter ◽  
J. Wilson ◽  
D. Gardner-Medwin ◽  
...  
Keyword(s):  
Spinal Muscular Atrophy ◽  
Genetic Study ◽  
Muscular Atrophy ◽  
Chronic Spinal Muscular Atrophy

Muscle strength and motor function throughout life in a cross-sectional cohort of 180 patients with spinal muscular atrophy types 1c-4

2018 ◽  
Vol 25 (3) ◽  
pp. 512-518 ◽  
Author(s):  
R. I. Wadman ◽  
C. A. Wijngaarde ◽  
M. Stam ◽  
B. Bartels ◽  
L. A. M. Otto ◽  
...  
Keyword(s):  
Muscle Strength ◽  
Spinal Muscular Atrophy ◽  
Motor Function ◽  
Muscular Atrophy ◽  
Cross Sectional

Chronic spinal muscular atrophy in adults

1969 ◽  
Vol 9 (3) ◽  
pp. 527-550 ◽  
Author(s):  
J.C. Meadows ◽  
C.D. Marsden ◽  
D.G.F. Harriman
Keyword(s):  
Spinal Muscular Atrophy ◽  
Muscular Atrophy ◽  
Chronic Spinal Muscular Atrophy
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