Clinical Manifestations And Neuroelectrophysiologic Features In The Early Diagnosis Of Motor Neuron Disease

ObjectiveMotor Neuron Disease(MND)/Amyotrophic Lateral Sclerosis(ALS)is a progressively degenerative disease of nervous system that mainly affects spinal cord,brain stem,and cerebral cortex motor neurons.Due to the unclear pathogenesis,early clinical symptoms are occult and heterogeneous,and lack of biological markers and characteristic neuroimaging changes.At present,there are difficulties in its early diagnosis and correct diagnosis.The purpose of this study was to explore potential biomarkers,possible therapeutic drugs,and factors that affect early diagnosis by reviewing the literature and collecting case data and discuss their potential for improvement.MethodsWe retrospectively analyzed 56 patients with ALS in department of neurology in Xi'an Gaoxin Hospital from december 2006 to march 2017.The gender,age at onset,time of diagnosis,first site,first symptom,and neurophysiological examination of ALS patients were collected,including the first time of neurophysiological examination,needle electromyography(EMG)data,and nerve conduction velocity data.ResultsThere were 56 patients with ALS in this group.There were 44 cases of clinically definite ALS,which accounted for 78.6%of the total number of cases.There were 7 cases of clinically probable ALS,which accounted for 12.5%of the total number of cases.There were 5 cases of clinically possible ALS,accounting for 8.9%of the total number cases.The type of diagnosed in this group of patients showed that 60.7%of amyotrophic lateral sclerosis(ALS),8.9%of progressive bulbar paralysis(PBP),and 30.4 % of progressive spinal muscular atrophy(PMA).The diagnostic level of clinical were improved in this group of ALS patients by EMG examination.It improved 4 cases from clinically possible to clinically probable(7.1%of total cases),6 cases from clinically probable to clinically definite(10.7%of total cases),2cases from clinically possible to clinically definite(3.6%of total cases).There were 39 males and 17 females patients in this group of ALS.The ratio of male to female was 2.3:1.Among them,46.4%patients were upper limb onset,21.4%medullary muscle onset,23.2%lower limbonset,no respiratory muscle onset case and 8.9%patient were started atypical.The age was between 10 and 72,the mean onset age was 52.5±12.0.The onset age in the medullary muscle group was 56.2±13.0,in the upper limb group was 54.3±8.6,in the lower limb group was45.1±14.5.The median time of total diagnosis was 12.5 months.The median time of the first part of the diagnosis time was 7 months,the diagnosis time was positively correlated with the first part of the diagnosis time(rank correlation analysis rs=0.31,p=0.039<0.05).The median time of diagnosis in the medullary muscle group was 8 months,in the upper limb group was12.5 months and in the lower limb group was 30 months.The median time to first electromyography was 11 months.In the upper limb group,the median amplitude of the CMAP of the median nerve was lower than the lower limit of normal,and the median amplitude of the CMAP of the ulnar nerve was greater than normal,indicating that the median nerve was more susceptible to involvement.The clinical manifestations and electrophysiological were incompatible in this group of patients with ALS.There were EMG abnormalities of 57.1%in this group of patients with asymptomatic limb muscle,especially the upper and lower limbs.Physiological evidence of "split hands" existed in different groups,accounting for 85.7 % of the cervical spinal cord group.Sensory nerve conduction abnormalities in 10%of this group of patients.Conclusion1.Early EMG examination in this group of patients with ALS increased the diagnostic level of 21.4%of patients.2.The diagnosis time of medullary muscle onset in this group of patients with ALS was short.The diagnosis time of lower limb onset,misdiagnosis at the first consultation,and onset age <40 or >60 was longer.3.The first part of the diagnosis time of ALS patients accounted for a large proportion of the time delay in diagnosis(56 %).The delay in second part of the diagnosis time were related to misdiagnosis.4.In this group of ALS patients,85.70%of patients in the upper limb onset group had "split hand" electrophysiological characteristics,45 % of patients in the medullary muscle onset group had "split hands" electrophysiological characteristics and 41%of patients in the lowerlimb onset group had "split hands" electrophysiological characteristics.