Clinical Analysis Of87Cases Of Motor Neuron Disease

Objectives:This study collected87confirmed cases of motor neurondisease (MND) of basic information,history of present illness,history of pastillness,bad habits,clinical manifestations,results of auxiliary examinations,firstclinical diagnosis,diagnostic classification,treatment etc,and analyzed thelaws.Hope that through this study we can provide references for clinicalworkers,and reduce the misdiagnosis rate.Methods:This study collected data of cases diagnosed motor neurondisease from the first affiliated hospital of Guangxi medical university fromJanuary2003to August2013.87patients are collected according to the standardset and exclusion criteria.Analyze basic information,history of presentillness,history of past illness,bad habits,clinical manifestations, biochemicalresults,radiological results,nerve electrophysiology,first clinical diagnosis,diag-nostic classification, treatment of87cases of the MND.Then according to theresearch purpose and the different data types,choose different statistical tablesand statistical figure for statistical description.For the measurementdata,statistical description in (mean±standard deviation);For counting informa-tion to relative statistical description.Results:(1) Cases into the group of87cases,9cases of hereditary motor neuron disease patients were all spinal muscular atrophy,and4cases weremale,female in5cases;Non-hereditary motor neuron disease patients in28werefemale,male in50cases.(2)Spinal muscular atrophy (SMA) in SMA Ⅰtypepatients were5cases (55.6%),SMAⅡ type patients in3cases(33.3%),SMA Ⅲt ype patients in1case(11.1%);In non-hereditary motor neuron diseasepatients, amyotrophic lateral sclerosis(ALS) patients were47cases(60.3%),progressive muscular atrophy (PMA)in26cases (33.3%),progressive bulbarpalsy(PBP) in2cases (2.6%),primary lateral sclerosis(PLS) in3patients(3.8%).(3) In hereditary motor neuron disease patients,there were6cases withdouble lower limbs weakness,and1case with arms and legs weakness,2casesof diseased parts unknown.(4)Onset age of patients with non-hereditary motorneuron disease was between12years old to73years old,and the peak incidenceof40～60years old.(5)In non-hereditary motor neuron disease patients,therewere78cases with muscle weakness,67cases with muscle atrophy,48cases withfasciculation,51cases with pyramidal sign,54cases with bulbar paralysis,9caseswith dyspnea,9cases with autonomic dysfunction(including defecation andurination disorders,abnormal secretion of sweat),13cases with Subjective feelingunusually,4cases with objective disturbance of sensation,3cases with chestdistress,3cases with dizziness.(6)ALS patients with upper limb onset,lower limbonset,medulla oblongata onset were All11cases(Each accounted for23.4%ofALS patients);11patients of26cases of PMA were upper and lower limbonset,in7patients with upper limb onset,in5patients with lower limb onset,in1patients with medulla oblongata onset.(7)In24cases with spontaneousactivity,7cases appeared in the sternomastoid muscle,15cases in the little fingerabductor muscle,12cases in thumb short abductor muscle,17cases in pretibialmuscle,10cases in T10spinal muscle.(8)In35non-hereditary motor neuron disease patients with head MRI inspection,including15cases of normalities and20cases of abnormal.There were13cases (13/35,37.1%) of the white matterdemyelination,mainly distributing in internal capsule,Angle of the lateralventricle and half egg circle center.(9)Non-hereditary motor neuron diseasepatients were misdiagnosed in49cases (73.1%).And ALS had the highestpercentage of misdiagnosed as cervical spondylosis, accounting for29.0%,butPMA had the highest percentage of misdiagnosed as myopathy, accounting for35.3%.Conclusion:In this group of motor neuron disease patients,non-hereditarymotor neuron disease was common,and hereditary motor neuron disease wasrare;SMA typeⅠ was the most common in SMA patients,and SMA patients wereoften in the onset of the symmetry, lack of power of lower limbs;Non-hereditarymotor neuron disease men were more than women, and the peak incidence of40~60years old.In non-hereditary motor neuron disease patients,muscleweakness and/or muscle atrophy and pyramidal tract damage were the mostcommon clinical manifestations,at the same time there would be a small numberof patients with symptoms of sensory nerve or autonomic nerve damage;ALSpatients often were misdiagnosed as cervical spondylosis,and PMA patientsoften were misdiagnosed as myopathy;The highest proportion of non-hereditarymotor neuron disease patients was ALS, secondly PMA, PBP and PLS wererare;.ALS patients with onset of upper limb, lower limb, the onset of the medullaoblongata were the most common,and PMA patients with the upper and lowerlimb onset at the same time were the most common; In non-hereditary motorneuron disease patients,lumbosacral segment in pretibial muscle in patients withspontaneous activity detection rate was highest, cervical cord in a little fingerabductor muscle segment with the highest detection rate, thoracic cord in T10 spinal muscle with the highest detection rate,medulla oblongata in sternocleido-mastoid with the highest detection rate.