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- W122937263 abstract "This chapter reviews that sporadic amyotrophic lateral sclerosis (ALS) is a degenerative disease of motor neurones, resulting in progressive loss of voluntary muscle strength. It is probable that familial ALS is genetically heterogeneous and can be caused by mutations in more than one gene. Individuals, who are known SOD1 mutation carriers, constitute a unique opportunity to study pre-clinical ALS. The chapter discusses that patients with substantial chronic denervation could maintain normal muscle twitch tension until loss of about 70%–80% of motor units occur when collateral reinnervation was unable to provide further compensation. This indicates that considerable (>70%) motor neurone loss could occur before the onset of symptoms or weakness in ALS patients. It reviews that in order to determine if significant loss of motor neurones occur years before the onset of disease, a cross-sectional study is performed at risk asymptomatic individuals— that is, no neurological symptoms or signs as determined by a neurologist." @default.
- W122937263 created "2016-06-24" @default.
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- W122937263 date "2009-01-01" @default.
- W122937263 modified "2023-09-27" @default.
- W122937263 title "Motor unit number estimation in asymptomatic familial amyotrophic lateral sclerosis" @default.
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- W122937263 doi "https://doi.org/10.1016/s1567-424x(08)00016-0" @default.
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