Abstract:
:Spinal muscular atrophy (SMA) is a severe neuromuscular disorder characterized by the degeneration of motor neurons in the spinal cord, and comprises a broad clinical spectrum. With the advent of new therapies (e.g., Nusinersen) for patients of all ages and disease stages, sensitive clinical measures are needed to detect slight changes in muscle force even in immobilized, severely affected patients often unable to move limbs. As for these patients, well-established outcome scales set out to evaluate motor function do not work properly, we propose measurement of maximum bite force which is able to detect subtle changes of bulbar function. Requirements for this approach are mentioned, challenges are discussed, and first insights from a pilot study are presented. Finally, a study design is proposed to evaluate the measurement of maximum bite force during the follow up of SMA patients with and without a disease modifying therapy.
journal_name
Front Neuroljournal_title
Frontiers in neurologyauthors
Kruse T,Lehmann HC,Braumann B,Fink GR,Wunderlich Gdoi
10.3389/fneur.2020.00139subject
Has Abstractpub_date
2020-02-25 00:00:00pages
139issn
1664-2295journal_volume
11pub_type
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journal_title:Frontiers in neurology
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abstract::Background: Neurofilament light chain (NFL) was believed to be a promising biomarker for the diagnosis of Amyotrophic lateral sclerosis (ALS) and disease burden evaluation. Objective: To determine the serum NFL level and its clinical relevance, including its association with disease severity [evaluated by the ALS Func...
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pub_type: 杂志文章,评审
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pub_type: 杂志文章,评审
doi:10.3389/fneur.2020.01055
更新日期:2020-09-17 00:00:00
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doi:10.3389/fneur.2018.00075
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doi:10.3389/fneur.2020.563373
更新日期:2020-09-29 00:00:00
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pub_type: 杂志文章
doi:10.3389/fneur.2018.00272
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abstract::Rhythmic auditory cueing is a well-accepted tool for gait rehabilitation in Parkinson's disease (PD), which can now be applied in a performance-adapted fashion due to technological advance. This study investigated the immediate differences on gait during a prolonged, 30 min, walk with performance-adapted (intelligent)...
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pub_type: 杂志文章,评审
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