| Objectives:A retrospective study was conducted to analyze and summarize the characteristics of the clinical,electrophysiological data and other auxiliary examination results of patients with ALS,so as to strengthen the clinical workers' understanding of ALS and their ability of early diagnosis,and promote the early detection,diagnosis and treatment of patients with ALS.Methods:Screening ALS patients who met El Escorial revised diagnostic criteria and were hospitalized in the first affiliated hospital of nanchang university during2017.01.01-2020.02.07.All cases were reviewed and analyzed,the patient history information,neuroelectrophysiological examination results and other related auxiliary examination results were recorded,and the collected data were statistically analyzed by SPSS26.Results:Among the 96 patients with ALS,61 were males and 35 were females,with a sex ratio of 2.1 to 1.The average age of onset was 57.26±11.8.The average age of males was 56.98±11.45.The average age of females was 57.74±12.54.There was no statistically significant difference in the age of onset between men and women(t test results =0.05,P=0.763).Among the 96 patients,20 were smokers,13 were drinkers,and 11 were smokers or drinkers.Of the 96 patients,1 had a family history of motor neurone disease and 1 had a family history of diabetes.There were 53 patients with previous medical history,among which the most complicated surgical diseases were fractures and fracture surgery(30%),followed by cervical spine surgery(13%).Hypertension(31%)was the most common medical complication in the past history,followed by diabetes(8%).There were 10 patients(10.4%)with subacute onset(within 3 months)and no obvious acute onset.The majority of patients had chronic onset,with a total of 86 cases(89.6%).The shortest course of disease was 1 month,and the longest was 180 months.M50(P25,P75)is 12 months(6 months,12 months).70 patients were characterized by single parts(cervical spinal,medulla oblongata,lumbosacral pulp,pulp chest)involvement have disease,accounting for 73% of all examination and diagnosis of patients,patients with onset of the single parts in 33 cases of cervical spinal have disease is the most,accounting for 34% of all patients,followed by 20 cases(21%)and 16 cases of onset of the medulla oblongata(17%),the onset of the lumbosacral spinal thoracic pulp have disease is only 1 case,accounted for 1%.The frequency of the top five symptom descriptions in ALS patients were: fatigue(23%);Tongue muscle atrophy,muscle atrophy(19%);Slurred and unclear speech(7%);Drinking water and diet(6%);Meat hop(6%).The frequency of the top five positive signs were: muscular atrophy(22%);Myopathy(16%);Hyperreflexia of tendon(8%);Hoffmann positive(7%);Dysarticulation and dysarticulation(6%).There were 22 cases of fast-progressive ALS(23%)and 74 cases of slow-progressive ALS(77%).The median ALSFRS-R score M50(P25,P75)was 44(42,46).The average score of ALSFRS-R was 42±7.Among the 604 results of motor nerve conduction velocity examination,the overall abnormal rates of the median nerve,common peroneal nerve,tibial nerve,ulnar nerve,radial nerve,axillary nerve,femoral nerve,musculocutaneous nerve,facial nerve and suprapathal nerve were 76.20%,51.80%,43.90%,51.60%,78.60%,52.40%,34.2%,53.10%,75% and100%,respectively.The mean amplitude of median CMAP was 5.1±4.1mV,the mean amplitude of ulnar nerve CAMP was 9.2±6.8mV,and the amplitude of ulnar CMAP was significantly greater than that of median nerve(t=1.979,P= 0.004).Similarly,the mean amplitude of tibial CMAP(16.2±7.9mV)was significantly greater than that of peroneal CMAP(6.5±3.3mV)(t=1.981 P= 0.000).The abnormal rate of conduction velocity of 346 sensory nerves was 38.5% of median nerve,33.90% of superficial peroneal nerve,53.80% of ulnar nerve and 50% of radial nerve,respectively.Only 1(8%)of the 13 patients with RNS showed a decreasing amplitude of the right facial nerve.The average latency of F wave in 108 median nerves was 24.6±3.9ms,and the abnormal rate of F wave was 73.6%.The mean incubation period of 24 ulnar nerves was 23.6±3.2ms.The abnormal rate of F wave was 60.9%.The average latency of H reflex of 106 tibial nerves was 31.2±7.8ms.The abnormal rate of H reflection was49%.In total,309 muscles had fibrillation and positive phase potential in resting state.MUAP time duration widened and amplitude increased during light contraction,and neurogenic damage was shown in simple phase,mixed phase and equal pathological interference during strong contraction.The detection results of three muscles showed that during light contraction,MUAP time constriction was narrowed and amplitude was decreased,presenting myogenic damage.The residual muscle detection results showed that there was no obvious fibrillation positive phase potential in the resting state,the amplitude and time limit were generally normal in light contraction,and there was interference phase in strong contraction,with no obvious myogenic or neurogenic damage.There were some differences in the results of other imaging and laboratory tests,but there was no specificity.Conclusions:1.ALS tends to occur in the middle-aged and elderly aged 40-70 years,with slightly more males than females.2.Most cases of ALS are caused by single site involvement,among which the most common causes are upper limb fatigue and muscular atrophy caused by the involvement of the cervical spinal cord,followed by the medulla oblongata and the lumbosacral spinal cord.3.The site of involvement of ALS patients is usually adjacent to the site of onset,presenting a continuous pattern,but there are some cases that involve other sites by jumping from the site of onset.4.Some patients with ALS may have paresthesia.If there are neuroelectrophysiological results of paresthesia,the diagnosis of ALS should be considered carefully or combined with other diseases.5.The neuroelectrophysiological examination plays an important role in the diagnosis of ALS patients.The common abnormal results in the motor nerve conduction project are the decrease of CMAP amplitude,the slow conduction velocity,and the prolonged incubation period in turn.The abnormal needle pole emg shows extensive neurogenic damage,especially abnormal spontaneous potential at rest,MUAP time broadening and amplitude increasing in light contraction state,and simple phase in recruitment phase during vigorous contraction. |
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