Clinical Features Analysis And Follow-Up Of Neuromyelitis Optica Spectrum Disorder

Objective to strengthen the understanding of the disease and application of new diagnostic criteria in clinical practice,and to explore the factors that affect the recurrence.Methods this study cases are from hospitalized patients and patients which to outpatient clinic of our hospital in April 2014 to April 2017,then have a re-diagnosis with the 2015 diagnostic criteria of NMOSD,finally,39 cases were confirmed.Results1.In this study,there are 2 cases are male and 37 cases are female,male to female is 1:18.5,the average age of onset is（39.2±13.9）years;2.With other diseases: four cases with other Autoimmune diseases（10.3%）;three cases with tumor（5.1%）;3.Season of the first onset: Spring accounted for 38%,Summer accounted for23%,Fall accounted for 18%,Winter accounted for 8%,the rest is unknown（13%）;4.Incentive of the first onset: 12.9% patients have incentives,including fatigue,cold and dirrrhea;87.1% patients have no obvious incentive;5.Incentive of recurrence: 43.6% patients have incentives,fatigue is the most common,also including cold,depression mood,prematurely stop taking hormones and suddenly reducing or stop taking Azathioprine,56.8% patients have no obvious incentive;6.The first treatment Department: Neurology accounts for 59%,Non-neurology accounts for 41%,Ophthalmology is the most common,also including Orthopedics,Gastroenterology,Dermatology;7.The first core clinical symptoms: mainly optic neuritis（33.3%）and myelitis（41%）;Posterior zone syndrome and Acute brain stem syndrome is also more common（15.4%）8.AQP4-IgG: 26 cases are positive,accouting for 72.2%,10 cases are negative,accouting for 27.7%;the rest were not detected;Autoimmune antibodies: ten cases are abnormal,ANA positive is the most common;9.CSF characteristics: there are 4 cases with elevated CSF pressure（220 ²80mm H₂O）,6 cases with elevated CSF cells（35¹50*10⁶/L）,5 cases with elevated CSF sugar（4.54 5.4mmol/L）;6 cases with elevated CSF protein（486.5 984.8mg/L）;3 cases of elevated CSF Chlorine（131 ¹34.7mmol/L）,one case of reduced CSF Chlorine（109mmol/L）;of the 9patients,only one case of CSF oligonucleus was weakly positive;of the 6patients,only one case of IgG index are slightly higher（0.77）,the rest is normal;10.Spinal cord MRI abnormality rate was 88.9%,the lesions are most in neck,chest pulp,96.9% patients involved more than three vertebral segment,3.1%patients involved less than three vertebral;T2WI images are long T2 signals,T1W1 can be equal or long T1 signal,mainly damaged grey matter,also can damage the white matter;Central-type damage accounts for 84.3%,eccentric accounts for 15.6%;of the 16 patients,only one case of the MR enhancement lesions were enhanced;11.Head MRI abnormality rate was 52.6%,the lesions are more common in Medulla oblongate,pons and lateral ventricle side;12.Follow-up: outpatient follow-up of 19 patients,the remaining symptoms include monocular or binocular vision decreasing,numbness,weakness,blurred speech and constipation.Of the elected 5 patients,the annual recurrence rate after taking azathioprine was lower than before taking.Conclusions1.NMOSD patients are mostly young and middle-aged women,the average age is（39.2±13.9）years;2.The spinal cord lesion is most common in the cervical and cervical thoracic segment,T2 WI images are long T2 signals,T1W1 can be equal or long T1 signal,mainly damaged grey matter,also can damage the white matter;Central-type damage accounts for 84.3%,eccentric accounts for 15.6%;Most of the MR enhancement lesions were not enhanced;more than fifty percent of the patient’s brain can be affected,the lesions are more common in Medulla oblongate,pons and lateral ventricle side3.Azathioprine may reduce the annual recurrence rate of NMOSD patients;4.Fatigue,cold,depression mood,prematurely stop taking hormones、suddenly reducing or stop taking Azathioprine can induce recurrence.