Clinical Analysis Of40Cases With Clinical Manifestation Resembling Motor Neuron Disease

Objective: To investigate the clinical features of motor neuron disease(MND), along with the auxiliary examination and prognosis, in order toprovide clinical datas for further research in pathogenesis, diagnosis and thetreatment.Methods: We collected40cases with clinical manifestation resemblingMND, of which36cases were proved to be definitely or probablely motorneuron disease.We analyzed the clinical features of the36cases incl-udingthe onset age, gender, disease course, previous history and so on. Besi-des wealso analyzed the auxiliary examinations, misdiagnosis and prognosis.Results:1In the40cases with clinical manifestation resembling MND,34cases wereproved to be amotrophic lateral sclerosi (ALS). One was diagnosed asprogressive bulbar palsy (PBP).2cases were confirmed as Kennedy’s disease(KD). One was certified to be cerebrovascular disease associated withhyperthyroidism. The last two cases were paraneoplastic syndrome. Three ofthe34cases of ALS were special: one was associated with thyroiditis, anotherwas concurrent with thymic hyperplasia, the last one was flail arm syndrome(FAS).2The clinical characteristics, auxiliary examinations, misdiagnosis andprognosis of MND2.1Clinical characteristicsMND progressed slowly,the mean corse was13.94±12.36months andthe mean onset age was56.86±10.83years old. Sex ratio(male vs female) was1.5:1. Muscle weakness was the most commom first symptom.37%of thecases presented pyramidal signs,40%with hyperactive tendon launch,and14%with fasciculations. 2.2Auxiliary examinationsAll cases took electromyographies, in which showed an activeneurogenic disorder.57%of the cases were abnormal in sternocleidomastoid.26cases had pulmonary CT, of which11cases presented with lung infection.18out of the19cases with cervical MRI were not normal. And4cases weremisdiagnosed with cervical spondylosis in the early stage. One case wasdiagnosed with thyroiditis, and became normal after theropy, while theperformance of limbs weakness were gradually worsened.2cases wereHepatitis B, and one case was chronic hepatitis C.25cases tookimmunoglobulin check and17cases took complement check, MND patientshad significantly different compared with healthy crowd in IgA, C3and C4level.12cases had a higher level of NSE and it had statistically significantcompared with healthy people. It has statistical difference that serum folatelevel was reduced and homocysteine level was increased compared withhealthy people. After the examnition of serum ferritin and transferrin, wediscovered significantly decreased in transferring. The levels of cholesterol,triglycerides were similar in patients with MND and healthy people. However,high-density lipoprotein and low-density lipoprotein levels showed asignificant different in MND patients. The creatine kinase level of MNDpatients were significantly increased.2.3Misdiagnosis at the onset of the disease8cases were misdiagnosed at the onset of the disease. The misdiagnoserate was22.8%. Three cases were misdiagnosed as cerebrovascular disease.Four cases were misdiagnosed as cervical spondylosis. One was misdiagnosedas coronary heart disease.2.4Prognosis11of the23cases which were successfully followed up tookriluzole(31.4%). Four cases showed adverse reactions and relieves afterstopping.7people died. The mortality rate was30.4%, with an averagesurvival time of16months (16.14±4.06months). Respiratory failure is thefinal cause of death. Conclusion:1Many diseases may be mixed up with MND clinically. In this research,5cases with clinical manifestation resembling MND were proved to be KD,cerebrovascular disease combined with hyperthyroidism and paraneoplasticsyndrome respectively.2MND progressed slowly, the mean course was13.94±12.36months and themean onset age was56.86±10.83years old. Sex ratio(male vs female) was1.5:1. Muscle weakness was the most commom first symptom. The mostcommon symptom is the right upper extremity weakness(34%)。Electromyographies of the35cases revealed an active neurogenic disorder.57%of the cases were abnormal in sternocleidomastoid. MND patients hadsignificantly different compared with healthy crowd on IgA, C3and C4level,which suggested that the immune reactions could be one of the pathogenesisof motor neuron disease. A higher level of NSE indicated injuries of motorneuron, but lacked specificity. Compared with healthy crowd, reduced serumfolate level and increased homocysteine level were consistent with previousresearches, indicating that homocysteine metabolism was associated with thepathogenesis of MND. Transferrin levels of MND patients were lower thanhealthy people, suggesting that MND patients presented with iron metabolism.This also confirmed the fact that metal metabolic abnormalities may be one ofpathogenetic mechanisms of MND.34%of the cases had an increased CKlevel. There was not different between sexes, or between bulbar and limbonset patients. The misdiagnose rate was22.8%. Cases with upper limbweakness as the main symptom were misdiagnosed as cervical spondylosisusually. Others with glossolalia were easy to be misdiagnosed ascerebrovascular disease. Only34percent of cases took riluzole after diagnosis,and36%withdrawed due to the side effects.7of the23cases which weresuccessfully followed up died, with an average survival time of16months (16.14±4.06months). Respiratory failure is the final cause of death.