By Kasugai, Yu; Machtoub, Lina
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Additional info for Amyotrophic lateral sclerosis : advances and perspectives of neuro-nanomedicine
2 Electrophysiology in the Revised EEC The revised EEC state that EMG/NCV (nerve conduction velocity) examination is an extension of the clinical examination used to identify LMN dysfunction, which can be deﬁned by electromyographic concentric needle examination to provide evidence of active and chronic denervation. Signs of active denervation consist of ﬁbrillation potentials and positive sharp waves, while fasciculation potentials are not mentioned in this context. Signs of chronic denervation consist of large motor unit potentials of increased duration with an increased proportion of polyphasic potentials often of increased amplitude and reduced interference pattern with ﬁring rates higher than 10 Hz, unless there is a signiﬁcant UMN component, in which case the ﬁring rate may be lower than 10 Hz and the motor unit potentials unstable.
In approximately 10%–15% of cases a familial occurrence of MND has been reported. On the basis of documented cases, the proportion in the present study is 5% (27 out of 580), but if cases described in the family histories are included, the proportion increases to 10% (64 out of 617). In 24 families with 124 cases of typical ALS, inheritance was autosomal dominant. According to these studies, the present series parentto-child transmission occurred in 80%, supporting the theory of autosomal-dominant inheritance.
It has been indicated that the overall risk from BMAA is probably low and only a few people are genetically at risk, and that even if BMAA causes common disorders like Alzheimer’s and Parkinson’s, that still doesn’t mean we should shun seafood. Commercial ﬁshermen generally do not work in areas heavily machtoub–ALS 31 October 23, 2015 12:42 PSP Book - 9in x 6in 32 Environmental Factors contaminated with cyanobacteria, so the danger of exposure in the United States and Canada should be modest for those who eat typical store-bought or homegrown food and avoid drinking contaminated water.
Amyotrophic lateral sclerosis : advances and perspectives of neuro-nanomedicine by Kasugai, Yu; Machtoub, Lina