Upper extremity function and activity in facioscapulohumeral dystrophy and limb-girdle muscular dystrophies: a systematic review

Bergsma, Arjen; Cup, Edith; Geurts, Alexander C. H.; Groot, I. de

doi:10.3109/09638288.2014.948138

Cited by 15 publications

(14 citation statements)

References 30 publications

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“…Furthermore, effect sizes varied greatly between participants which could reflect environmental and personal factors, such as device-specific facilitations or otherwise undoable ADLs and disease-specific affected muscle regions. In muscular dystrophy, such as Duchenne and limb girdle, proximal regions of the upper extremity are affected first which limits upper arm elevations [ 4 , 41 ]. In addition, ADLs such as eating and drinking can be performed with various contributions from the shoulder and elbow joint and may not depend on the ability to utilize both.…”

Section: Discussionmentioning

confidence: 99%

Daily Life Benefits and Usage Characteristics of Dynamic Arm Supports in Subjects with Neuromuscular Disorders

Essers

Murgia

Peters

et al. 2020

Sensors

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Neuromuscular disorders cause progressive muscular weakness, which limits upper extremity mobility and performance during activities of daily life. Dynamic arm supports can improve mobility and quality of life. However, their use is often discontinued over time for unclear reasons. This study aimed to evaluate whether users of dynamic arm supports demonstrate and perceive quantifiable mobility benefits over a period of two months. Nine users of dynamic arm supports were included in this observational study. They had different neuromuscular disorders and collectively used four different arm supports. They were observed for three consecutive weeks during which they were equipped with a multi-sensor network of accelerometers to assess the actual use of the arm support and they were asked to provide self-reports on the perceived benefits of the devices. Benefits were experienced mainly during anti-gravity activities and the measured use did not change over time. The self-reports provided contextual information in domains such as participation to social life, in addition to the sensor system. However self-reports overestimated the actual use by up to three-fold compared to the accelerometer measures. A combination of objective and subjective methods is recommended for meaningful and quantifiable mobility benefits during activities of daily life.

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Section: Discussionmentioning

confidence: 99%

Daily Life Benefits and Usage Characteristics of Dynamic Arm Supports in Subjects with Neuromuscular Disorders

Essers

Murgia

Peters

et al. 2020

Sensors

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“…To the best of the researchers' knowledge, this is the first study to explore upper extremity (UE) pain, stiffness, activity limitations and related participation restrictions in people with facioscapulohumeral muscular dystrophy (FSHD). [8] The most important result is the frequent presence of pain and stiffness in the shoulders and upper arms, which was associated with UE activity limitations as assessed with the CUE. Besides pain and stiffness, time since diagnosis was independently associated with basic UE activity limitations.…”

Section: Discussionmentioning

confidence: 99%

“…[6,7] In addition, no clear correlations have been found between muscle strength and gender, age or duration of symptoms. [8] Although the clinical picture of muscle weakness is variable, most persons with FSHD experience activity limitations when elevating the arms above shoulder-level. [9,10] It is also known that the execution of many activities of daily living (ADL) takes considerably longer in persons with FSHD compared to healthy subjects.…”

Section: Introductionmentioning

confidence: 99%

“…Hand function itself remains largely intact. [8] Interestingly, increased muscular cocontraction has been observed in the UEs of people with FSHD, [4,9] as well as enhanced electromyography activity in the shoulder muscles during arm elevation movements. [9] To develop tailored training interventions and new supportive aids, it is not only important to better understand UE impairments and limitations in basic UE activities, but also to obtain knowledge about limitations in more complex, instrumented UE activities and restrictions in social participation.…”

Section: Introductionmentioning

confidence: 99%

“…Currently, little is known about the UE capacity and performance of daily activities in people with FSHD. [8] Hence, the aim of this study was to obtain a better understanding of the UE impairments and related limitations in activities and restrictions in participation in people with FSHD. In a previous study of boys and men with Duchenne muscular dystrophy (DMD), a web-based survey that consisted of a set of questionnaires covering all relevant dimensions of the International Classification of Functioning Disability and Health [11] was used.…”

Section: Introductionmentioning

confidence: 99%

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Upper limb function and activity in people with facioscapulohumeral muscular dystrophy: a web-based survey

Bergsma¹,

Cup²,

Janssen³

et al. 2016

Disability and Rehabilitation

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Purpose To investigate the upper extremity (UE) at the level of impairments and related activity limitations and participation restrictions in people with facioscapulohumeral muscular dystrophy (FSHD). Methods The study was conducted using web-based questionnaires that were distributed amongst people with FSHD in the Netherlands. Eighty-eight respondents started the survey, and 71 completed it. The questionnaires covered the following dimensions: Function, Activity and Participation of the International Classification of Functioning Disability and Health. Results More than 40% of the respondents experienced pain in one arm or both the arms. Increased pain and stiffness scores and longer disease duration were associated with increased limitation scores. For basic activities, lifting the arm above shoulder-level was most frequently reported as most limited, coherent with the clinical picture of FSHD. Among the respondents, 50% indicated restrictions at school, 78% indicated restrictions at work and more than 80% indicated restrictions whilst participating in sports, hobbies, household activities and romantic relationships. Conclusions This study has shown that alongside the well-known problem of lifting the arms above shoulder-level, UE activities below shoulder height during vocational and occupational activities are also problematic in patients with FSHD. Alongside disease duration, pain and stiffness are associated with UE activity limitations. Implications for Rehabilitation Attention is needed for pain and experienced stiffness in the upper extremity as it is frequently present in patients with FSHD. Rehabilitation professionals need to be aware that patients with FSHD not only experience problems with activities above shoulder height, but also with activities below shoulder height. At least 50% of the patients with FSHD experience restrictions in participation as a result of limitations in their UE.

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Chemotherapy dosing and toxicity in a patient with muscular dystrophy

Lomma

Ransom

2018

Cancer Reports

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BackgroundChemotherapy dosing has traditionally been based on a body surface area (BSA) calculation despite BSA dosing being problematic in a number of conditions, such as renal failure, liver failure, obesity, and sarcopenia. This case highlights another condition in which BSA dosing is problematic.CaseA 57‐year‐old man with limb‐girdle muscular dystrophy presents with stage IIA inoperable squamous cell carcinoma of the lung. He is treated with chemotherapy and radiotherapy with curative intent but develops significant chemotherapy related toxicity affecting chemotherapy scheduling and dosing. Later, his cancer progresses, and he is commenced on palliative chemotherapy resulting in further significant chemotherapy toxicity.ConclusionSarcopenia is known to increase risk of chemotherapy toxicity. This case postulates that changes in muscle mass seen in muscular dystrophy increases risk of chemotherapy toxicity, similar to sarcopenia.

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Upper extremity function and activity in facioscapulohumeral dystrophy and limb-girdle muscular dystrophies: a systematic review

Cited by 15 publications

References 30 publications

Daily Life Benefits and Usage Characteristics of Dynamic Arm Supports in Subjects with Neuromuscular Disorders

Daily Life Benefits and Usage Characteristics of Dynamic Arm Supports in Subjects with Neuromuscular Disorders

Upper limb function and activity in people with facioscapulohumeral muscular dystrophy: a web-based survey

Chemotherapy dosing and toxicity in a patient with muscular dystrophy

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