Repeated Electrical Stimulation To The Value Of Amyotrophic Lateral Sclerosis And Sporadic Amyotrophic Lateral Sclerosis Gene Mutation And Polymorphism

Background and Objectives Thereare two neural electrophysiological ways to diagnosis neuromuscular junction disease:repetitive nerve stimulation(RNS) and single fiber electromyography(SFEMG).RNS is the most common method used to diagnosis myasthenia gravis and myasthenia syndrome.SFEMG is used to MG,ALS, and muscle diseases.Amyotrophic lateral sclerosis can induce neuromuscular disorders in addition to upper and lower motor neuron damage.SFEMG is generally used for diagnosis and differential diagnosis of ALS.However,there are some defects of SFEMG,such as technically demanding,time consuming,more invasive and so on although the high sensitivity of it.Therefore,there is a clinical need to find a new method to alternative to SFEMG.Some studies suggest that ALS patients may be a decrement with stimulation at slow rate on RNS.But,small samples were used in all of these studies.Moreover,there are international standards that the reduced low-frequency RNS in ALS should not exceed 20%.In addition,those were unknown what is the relationship between RNS and onset of the detailed parts,clinical severity,disease staging or disease progression,what is the consistency in the comparison of RNS and SFEMG,and what's the meaning of the differential diagnosis by RNS study between muscle atrophy in non-ALS and in ALS patient.In this study, larger samples of ALS cases confirmed and suspected by clinical and electrophysiological study have been exanimated using RNS and SFEMG at the same time.We performed following works:(1) To investigate the RNS positive rate and the decrement degree of ALS patients,the area difference of responses to RNS, and if NI index,onset site,clinical disease classification(light-in-weight-critical), progression rate of disease,gender and age are related with RNS.(2) To compare RNS with SFEMG in the diagnosis of ALS.(3) To investigate the RNS positive rate in non-ALS with muscular atrophy and weakness,and if RNS can be used for ALS diagnosis and differential diagnosis.Methods 101 ALS patients registered with detailed information in the neurology out-patient or ward of Beijing Union Medical College Hospital Since May 2008 to April 2009.RNS examination was employed for all patients and SFEMG examination only for Some patients(43cases) at the same time.Another 40 cases of non-ALS out-patient with muscle atrophy and weakness were examinated by RNS.Reslts(1) ALS was manifest on RNS as a decrement with stimulation at slow rate.The ALS-positive rate was 53%.No case of high-frequency increments occurred.(2) The positive rate of axillary nerve,accessorius nerve,radial nerve, ulnar nerve,facial nerve,tibial nerve is 30.6%,25%,15.5%,7.8%,1%,0%, respectively.(3) No correlation between RNS-positive group and sex,age(P>0.05); higher RNS-positive rate of limb onset than that of bublar onset(P<0.05);The progression rate of disease of RNS positive group was significantly increased than negative RNS group(P<0.05);The ulnar nerve CMAP(motor nerve action potential) of RNS positive group was significantly lower than the RNS negative group(P<0.05); No correlation between RNS positive rate and disease duration(...