Correlation Between ALS-FRS-R And Electromyography In Patients With Amyotrophic Lateral Sclerosis

ObjectiveAmyotrophic lateral sclerosis(ALS), the commonest clinical presentation of motorneuron disease (MND), is an adult-onset and age-related neurodegenerative disordercharacterised by loss of upper motor neurons (UMNs, including the Betz cells of themotor cortex), and lower motor neuron(LMNs, anterior horn cells of the spinal cord andbrainstem nuclei). The term MND is largely synonymous with ALS, reflecting theobservation that most patients demonstrate combined LMN-related loss of muscle as aresult of denervation (amyotrophy), and UMN degeneration of the lateral corticospinaltract and its cortical origins manifesting as gliosis, or hardening (sclerosis). ALS isstrikingly heterogeneous in both its presentation, with an average one-year delay fromfirst symptoms to diagnosis, and subsequent rate of clinical progression. About half ofpatients succumb within3–5years of symptom onset. Death in ALS is typically due togradual ventilatory failure. Choking is exceptional as a mode of death, a significantminority survives into a second decade. Despite numerous therapeutic trials, there is noreversible treatment for what is typically a catastrophic collapse of a previouslyapparently normally functioning motor system. In view of the fatal severity of ALS andALS brings heavy burden to the patients and families physically, mentally andeconomically, so early diagnosis can provide more chances to patients to arrange their lives, jobs and treatment reasonably. ALS-FRS-R can reflect the severity of ALSpatients, electromyography examination has important significance for the diagnosis ofALS, but so far, there are rare papers about the electromyography can or not reflect theseverity of ALS patients. The study is the preliminary study of correlation betweenALS-FRS-R and amplitude, duration ofrelated muscles’ EMG MUAP with lightcontraction in patients with ALS, to know whether amplitude and duration of relatedmuscles’MUAP with light contractioncould evaluate the severityof the disease inpatients with ALS.MethodsAll25patients with ALS were collected in the Department of Neurology, the FirstAffiliated Hospital of Dalian Medical University, from January，2011to April，2012.Clinical diagnosis conforms to ALS diagnostic criteria made in Spain in1994and therevised standard in1998. The ALS-FRS-R were finished in all25patients with ALS.The motor and sensory nerve conduction velocity of bilateral ulnar nerve, median nerve,pretibial nerve and pretibial nerve were finished in all25patients. The EMG of bilateralmusculus extensor digitorum communis（MEDC）, musculus tibialis anterior（MTA）,musculus rectus abdominali（sMRA）, sternocleidomastoi（dSCM）and paraspinal musclewere finished in most of the patients and F wave were finished in all25patients, Theoccurrence rate of F wave and EMG amplitude, duration of bilateral MTA, bilateralMEDC, MRA and SCM with light contraction of all25patients with ALS wererecorded respectively. Lastly, the correlations between ALS-FRS-R and EMG amplitude,duration of bilateral MTA, bilateral MEDC, MRA and SCM with light contraction wereanalyzed respectively.Results1. There was correlation between ALS-FRS-R and EMG amplitude of right MTA withlight contraction in23patients with ALS（P<0.05）；2. The EMG amplitude of left MTA（21patients）, bilateral MEDC（20patients）, MRA（20patients）, SCM（25patients） with light contraction had no correlationwiththeir ALS-FRS-R of all25patients with ALS； 3. The EMG duration of all the related muscles with light contraction had nocorrelation with their ALS-FRS-R.Conclusions1. There was correlation between ALS-FRS-R and electromyography amplitude ofindividual related muscles with light contraction in23patients with ALS.Consequently，EMG could only be used to diagnose ALS.2. The EMG amplitude, duration of related muscles with light contraction could notbe apply to evaluate the severity of the disease in patients with ALS.