Motor Function of Children with SMA1 and SMA2 Depends on the Neck and Trunk Muscle Strength, Deformation of the Spine, and the Range of Motion in the Limb Joints

Stępień, Agnieszka; Gajewska, Ewa; Rekowski, Witold

doi:10.3390/ijerph18179134

Cited by 9 publications

(19 citation statements)

References 36 publications

(40 reference statements)

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“…A manual containing all the procedures for applying the scales was used during the assessment. All items were tested without using a brace or orthoses [ 21 , 22 , 27 , 28 ].…”

Section: Methodsmentioning

confidence: 99%

“…The evaluated parameters included shoulder flexion (SF) and abduction (AbS), elbow flexion (EF), forearm pronation (PF) and supination (SF) in the upper limb, as well as hip flexion (HF), knee flexion (KF) and ankle dorsiflexion (AD—ankle dorsiflexion) in the lower limb. In addition, four parameters for detecting early musculoskeletal changes that predispose to scoliosis formation were examined [ 4 , 19 , 28 ]. The supine angle of trunk test (SATR), measuring the angle of upper (SATR-U) and lower (SATR-L) trunk rotation, and pelvic obliquity (PO), determining the value of pelvic tilt angle, were examined using a scoliometer.…”

Section: Methodsmentioning

confidence: 99%

“…Other parameters included cervical rotation test (CR), presenting the extent of cervical spine rotation, and hip extension (HE). Both parameters were measured using a plurimeter [ 4 , 19 , 28 ].…”

Section: Methodsmentioning

confidence: 99%

“…The current literature shows only one publication about the relationship between structural changes and motor function in children with SMA undergoing pharmacological treatment, which we believe affects physiotherapeutic assessment [ 28 ]. Moreover, previous publications presented functional improvement based only on total scale results, which is one of the limitations of the scales used in clinical practice.…”

Section: Introductionmentioning

confidence: 99%

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Prospective Analysis of Functional and Structural Changes in Patients with Spinal Muscular Atrophy—A Pilot Study

2022

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Spinal muscular atrophy (SMA) is a rare, autosomal recessive neuromuscular disease. Recent years have seen a significant development of therapeutic options for SMA patients. With the development of treatment methods, it has become necessary to adapt a physiotherapeutic approach to the evolving clinical picture of SMA patients. We presented an analysis of 40 SMA patients undergoing pharmacological treatment, examined twice in an average interval of 5 months. Twelve patients (non-sitters) were evaluated using CHOP-INTEND, while 28 (sitters) were tested using the Hammersmith scale. The research protocol consisted of measurements of upper and lower limb ranges of motion, and four tests for early detection of musculoskeletal changes. Both non-sitters and sitters patients showed motor improvement between the first and second examinations. Favorable changes in range of motion parameters were noted in most children, except for hip extension (HE) range, which deteriorated. An association was also observed between scale scores and the presence of contractures in the hip and knee joints depending on the group studied. Our findings showed that the presence of contractures at the hip and knee joint negatively affected functional improvement as measured by the scale scores.

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“…A manual containing all the procedures for applying the scales was used during the assessment. All items were tested without using a brace or orthoses [ 21 , 22 , 27 , 28 ].…”

Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Prospective Analysis of Functional and Structural Changes in Patients with Spinal Muscular Atrophy—A Pilot Study

2022

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“…In addition, the assessment with both baseline muscle MRI score and Cobb angle correlated with the nusinersen e cacy. Technical di culty of nusinersen administration in patients with severe scoliosis have been addressed [20,21], and it has been shown that deformation of spine correlates to the motor function in patients with SMA [22]. However, there were scarce information on how the severity of scoliosis affects the effect of nusinersen.…”

Section: Discussionmentioning

confidence: 99%

Muscle impairment and nusinersen affect variability in patients with spinal muscular atrophy type 2 and 3: a retrospective cohort study

Shimizu‐Motohashi

Chiba

Mizuno

et al. 2022

Preprint

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BACKGROUNDReal-world data have shown variability in treatment responses to nusinersen in patients with 5q spinal muscular atrophy (SMA). It could be hypothesized that the degree of muscle impairment may confer to the drug response variability. Magnetic resonance imaging (MRI) is a useful tool to identify muscle involvement patterns in SMA; therefore, we investigated whether the magnitude of muscle involvement assessed by MRI at baseline can predict the change in gross motor function after the initiation of nusinersen treatment.RESULTSWe retrospectively assessed the clinical outcome of our treated cohort, in relevance to the thigh and pelvic MRI taken before the nusinersen treatment. A total of 16 patients with SMA types 2 and 3 (age = mean (SD); 9.2 (4.6) year) receiving nusinersen treatment were enrolled in the study. Hammersmith Functional Motor Scale-Expanded (HFMSE) was assessed to observe the change in motor function. The T1-weighted MR images of the pelvis and thigh were scored for muscle fatty infiltration and atrophy, and the interrater reliabilities of MR image evaluation were verified in all 16 images. The minimally clinically important difference (MCID) was considered as gaining at least 3 points of HFMSE from baseline. Of these 16 individuals, 14 had been treated for at least 15 months and had baseline data. At 15 months, seven individuals obtained MCID in HFMSE, and seven did not. Baseline muscle MRI score could not differentiate the two groups; however, individuals who obtained MCID had significantly less severe scoliosis. In addition, there was a significant and negative relationship between baseline MRI score and the change of score in HFMSE after 15 months of treatment. Further, baseline Cobb angle along with MRI score also correlated to the degree of change in motor function.CONCLUSIONIt was indicated that the degree of muscle damage may confer the variability in response to nusinersen for individuals with SMA types 2 and 3. Muscle MRI score in addition to the severity of scoliosis assessed at baseline may help to predict the gross motor function change after the drug initiation.

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An eight-degree-of-freedom upper extremity exoskeleton rehabilitation robot: design, optimization, and validation

et al. 2022

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Motor Function of Children with SMA1 and SMA2 Depends on the Neck and Trunk Muscle Strength, Deformation of the Spine, and the Range of Motion in the Limb Joints

Cited by 9 publications

References 36 publications

Prospective Analysis of Functional and Structural Changes in Patients with Spinal Muscular Atrophy—A Pilot Study

Prospective Analysis of Functional and Structural Changes in Patients with Spinal Muscular Atrophy—A Pilot Study

Muscle impairment and nusinersen affect variability in patients with spinal muscular atrophy type 2 and 3: a retrospective cohort study

An eight-degree-of-freedom upper extremity exoskeleton rehabilitation robot: design, optimization, and validation

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