| Background and purpose: Motor Neuron Disease(MND)is a rare clinical degenerative Disease of the nervous system.Amyotrpnic Lateral Sclerosis(ALS)is the main type of MND.With the aging of the population and the aggravation of environmental pollution,the incidence of ALS has been on the rise in recent years [1-3]."Rare diseases" are no longer rare.The onset of ALS is insidiously hidden,and many patients have no specific symptoms when seeking medical treatment.Patients go from department of neurology to department of orthopedics,etc.Many physicians,even neurologists,have insufficient understanding of the early manifestations of the disease,leading to the early delayed diagnosis of patients[4-6].In recent years,the development of neuroelectrophysiology and neuroimaging technology makes the early diagnosis of ALS possible.In this study,clinical,electrophysiological and neuroimaging data of ALS patients were retrospectively analyzed in order to find the clinical rules of ALS and provide theoretical basis for the early diagnosis and treatment of ALS.Materials and methods: In this study,"motor neurone disease" or "amyotrophic lateral sclerosis" were used as search terms to search the electronic medical record inquiry system of the first affiliated hospital of dalian medical university in order to collect the detailed information(including general data,onset age,starting symptoms,diagnosis and treatment,neuroelectrophysiology,neural imaging examination,laboratory examination,etc.)of ALS patients who was hospitalized from January 2016 to October2019.SPSS25.0 software was used for statistical analysis and description.Results: a total of 51 ALS patients were collected,including 31 males(60.78%)and 20females(39.22%),with a male-female ratio of 1.55:1.There were 47 cases of han nationality,2 cases of manchu nationality and 2 cases of Korean nationality.There were 49 cases in liaoning province,1 case in heilongjiang province and 1 case in jilin province.Occupational composition: 4 cases of workers,8 cases of farmers,12 cases of employees,6 cases of unemployed,21 cases of occupation unknown.16 patients had a history of hypertension,11 patients had a history of type 2 diabetes,5 patients had a history of ischemic cerebrovascular disease,2 patients had a history of coronary heart disease,2 patients had a history of depression,1 patient had a history of anxiety disorder,1 patient had a history of hashimoto's thyroiditis,1 patient with thyroid cancer underwent total thyroidectomy,7 patients had a history of cervical spondylosis,4patients had a history of lumbar disease,5 patients had a history of trauma.The youngest onset age was 34 years,the oldest 79 years,the average onset age was 60.41±9.89 years,and the peak onset age was 50-79 years.The mean time from onset to first visit was10.00±10.32 months,and the mean time from onset to diagnosis was 15.40±14.12 months.First symptoms: bulbous symptoms(unclear speech,dysphagia,water choking,shortness of breath)in 11 cases(21.57%),muscle weakness in 35 cases(68.63%),muscle weakness with muscular atrophy in 1 case(1.96%),muscle atrophy in 2 cases(3.92%),and muscle jumping in 2 cases(3.92%).Location of onset: bulbous symptoms occurred in 11 cases(21.57%),bilateral upper limbs in 4 cases(7.84%),bilateral lower limbs in 5 cases(9.80%),unilateral upper limbs in 18 cases(35.29%),unilateral lower limbs in 9 cases(17.65%),and limbs in 4 cases(7.84%).23 patients were misdiagnosed,mainly as cerebrovascular disease in 3 cases(13.04%),cervical spondylosis in 4 cases(17.39%)and lumbar disease in 3 cases(13.04%).Among the 51 patients,12 were clinically definite ALS(23.53%),23 were clinically suspected ALS(45.10%),and 16 were clinically possible ALS(31.37%).There was no correlation between the first symptoms and the first diagnosis time,and the patients' first symptoms did not affect the first diagnosis time.There was no correlation between the first symptoms and the time of diagnosis,and the first symptoms did not affect the time of diagnosis.There was no correlation between the time of first diagnosis and the time of diagnosis,and the time of first diagnosis did not affect the time of diagnosis.Routine electromyography was performed on 50 patients,of which 46 had extensive neurogenic lesions,2 had neurogenic lesions in the cervical and lumbar segments,2 had neurogenic lesions in the bulbar and cervical segments,and 2 had suspicious neurogenic lesions in the thoracic and lumbar segments.RNS was performed in 22 patients,and low-frequency attenuation was observed in 10 patients,mainly in the axillary nerve,accessory nerve,facial nerve and trapezius muscle.In this study,brain CT,head MRI,spinal MRI and other neuroimaging examinations were mainly used to exclude other diseases similar to ALS,and there were no specific findings in laboratory examination.After diagnosis?A total of 34 patients were followed up,among which 33 patients were sickened,19 patients died,16 patients died of respiratory failure,and 3 patients died of heart failure.The average course of disease was 32.8±13.29 months.Of the 51 patients,only 14 were treated with riluzole,11 were treated with the drug for more than six months,and 5 died.Of the 51 patients,11 received ventilator assisted breathing,and 5 underwent tracheotomy after their condition worsened.Five patients underwent percutaneous gastrostomy.Conclusion:1.ALS mainly occurs in middle-aged and elderly people,mostly males,with the peak onset between 50 and 79 years old,and the average age of onset is 60.41±9.89 years old.2.The first symptoms of ALS are mainly muscle weakness,speech loss,dysphagia,water choking,shortness of breath and other ball symptoms.3.ALS usually starts in the limbs,among which unilateral upper limb is the most common.4.ALS is mainly misdiagnosed as cerebrovascular disease,cervical spondylosis and lumbar vertebra disease,and neuroelectrophysiological and neuroimaging examinations are helpful for differential diagnosis.5.The average course of disease from onset to death was 32.8±13.29 months,and the patients died mainly from respiratory failure.The course of disease of patients with bulbous symptoms was shorter. |
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