A Retrospective Study Of 34 Cases With Motor Neuron Disease

Background and Objective : Motor neuron disease(MND)is a group of the unkown neurodegenerative diseases,charaterized progressive motor neurons loss in brain stem and spinal cord,leading to muscle atrophy and weakness.And motor tracts between cortex and subcortical structure may also occur spasmodic rigidity,increased muscle tone.But sensory system and autonomic nervous system is often not involved.With the progress of the disease,most patients eventually lose the ability to self-care.Patients often die in 2-5 years after diagnosis,usually die of respiratory failure caused by muscle weakness or lung infection.Motor neuron disease can be divided into four types: 1.amyotrophic lateral sclerosis(ALS).2.progressive muscular atrophy(PMA).3.progressive bulbar palsy(PBP).4.The primary lateral sclerosis(PLS).C linical most common motor neuron disease is amyotrophic lateral sclerosis.But the diagnosis of MND are not specific for the standards,mainly combined with clinical symptoms and signs,electrophysiological examination,neuroimaging,and after the exclusion of other diseases in order to make a clinical diagnosis.This article aims to study clinical and neurophysiological or other examinations characteristics of MND,through retrospective study,and promote clinicians understanding and diagnosing MND.Materi MND and Methods:Through the First Affiliated Hospital of Dalian Medical University electronic medical records queries,collecting the clinical date of 34 patients in the the First Affiliated Hospital of Dalian Medical University from September 2013 to January 2016 who have been diagnosed MND accordding to the standards of 2006 Awaji-Shima and 2012 China MND clinical and neurophysiological diagnostic criteria.Clinical data collected included the patient's age,sex,nationality,occupation,place of residence,past history,bad habits,family history,the first symptom,duration,neurological signs,laboratory examinations,test results,etc.And then they were summarized and analyzed.The auxiliary check: EMG,CSF,brain MRI,head CT,cervical MRI,lung CT,immunological tests,thyroid function,muscle enzymes,lipids,virology testing.These outcome mesures were analyzed by SPSS 19.0.Results:Of all the 34 patients,male 20 cases,female 14 patients,the age onset is 21-84 years,and the average age is(61.32 ± 14.29)years.The proportion of male and female patients with MND is about 1.43: 1.O f all the MND patients found no less than 20 years,the minimum age for the first time in 21 years,with increasing age,MND increase in the number of patients.MND patients first overall peak incidence is 60-69 years.The number of MND patients 80 years of age are significantly reduced.34 cases of MND patients are Han.In 34 cases of MND patients,seven patients had previous history of hypertension,four patients had a history of diabetes,11 patients have surgery,five patients have a history of trauma,four patients had a history of cerebral infarction,4 patients had a history of cancer,2 patients had erysipelas history.Before the onset,30 persons has not a clear incentive.MND patients with distal onset are 25 cases.The main symptoms and signs of 34 MND patients at presentation is muscle weakness,muscle atrophy and vertebral tract signs positive.C lassic amyotrophic lateral sclerosis is 29 cases,accounting for the majority.31 cases were taken EMG.EMG results suggest that there is widespread neurogenic 28 cases of MND patients,motor unit action potentials(MUAP)are present extend the time limit,the phenomenon of increased volatility.Repetitive nerve stimulation(RNS)shows high and low frequency stimulation,no significant increase or attenuation change.Neuroimaging is mainly used for differential diagnosis of MND,the exclusion of other diseases.And the other auxiliary examination is no specificity.Conclusion: 1.MND is more seen in middle-aged men,average age is 61.32 ± 14.29 years,peak incidence in 60-69 years of age.The diease is occult onset,chronic progressive course.2.The motor neuron disease is more seen in classic ALS patients.Weakness and muscle atrophy is the most common clinicl manifestations.In some patients,neurological examination occurs positive pyramidal tract signs.3.The most common seen is upper limb onset,followed by the lower limbs,as well as some direct involvement in patients with bulbar onset.As the disease progresses,there may be involvement of the medulla oblongata.4.Neurophysiological examination of MN D diagnosis is important.EMG is often abnormal spontaneous potential,MUAP extend the time limit,amplitude increased,and when the strong contraction phase occurs simply raising phase.The diagnosis is not clear that patients should review the EMG after three months.NCV,F wave and RNS are mostly normal.5.MND patients can been easily misdiagnosed as cervical disease,cerebrovascular disease.MRI can help rule out.Cerebrospinal fluid and blood test are no specific.