Analysis Of Clinical Characteristics Of Neuromyelitis Optica Spectrum Disorders With Painful Tonic Spasm

Objectives:Painful tonic spasm(PTS)is a common symptom in patients with Neuromyelitis optica spectrum disorder(NMOSD).The purpose of this study is to study and compare the clinical characteristics of NMOSD with and without PTS,to analyze the related factors of PTS,to explore the pathophysiological mechanism of PTS,assist in the diagnosis and treatment of clinical NMOSD patients with PTS.Methods:We reviewed the medical records and interviewed patients with NMOSD who attended the First Affiliated Hospital of Dalian Medical University between July2015 to November 2020.Patients who fulfilled the 2015 diagnostic criteria for NMOSD published by the International Panel of NMO Diagnosis(IPND)were enrolled in this study.We judge whether the patient had painful tonic spasm attack according to the definition of PTS and the evaluation method of Shanghai Huashan Super Super Research team with The spinal cord health injury scale(SCI-HQ).Collected these patients’ demographic data,clinical symptoms,imaging,laboratory examination,treatment and prognosis information.Analysis of risk factors of PTS by Logistic regression.Results:1.A total of 80 cases of NMOSD were collected,including 33 cases of NMOSD with PTS,the prevalence of PTS in NMOSD is 41.25%,only when spinal cord was involved,those only with visual acuity or brain damage did not have PTS.Among the 33 patients with NMOSD with PTS,the first symptoms were limb numbness and limb weakness,all of which were 10 cases(30.3%).In 33 patients with PTS,the spastic site involved the upper limbs more than the lower limbs.Among the 33 patients with PTS,flexors were the most common during spasms,and 1 patient had mixed spasm.The drug treatment effect of this patient was not satisfactory.The median NRS pain score of 33 NMOSD patients with PTS was 5.According to PSFS(Penn spasm frequency scale),there were 11 mild to moderate patients and 22 severe patients.Among the 33 patients,3 patients did not take medication because of the low frequency of PTS attacks,and the rest were treated with drugs,23 patients took carbamazepine,of these 23 patients treated with carbamazepine,6 cases had previously taken baclofen and 2 cases had taken gabapentin,all of which were replaced with carbamazepine because of poor effect.Among them,1 cases were treated combined with clonazepam because of the poor effect of carbamazepine alone.2 cases were replaced by oxcarbazepine because of serious adverse reactions(liver function damage)of carbamazepine,and 17 cases(73.9%)had good response using PGI-I score.2.The course of disease in NMOSD with PTS group was longer than that in non-PTS group,and the length of spinal cord involvement in NMOSD group was longer than that in simple NMOSD group,the proportion of cervical spinal cord involvement and the positive rate of AQP4 antibody in the patients with PTS were significantly higher than those in the NMOSD group(P < 0.05).There was no significant difference in age of onset,EDSS score,neutrophil count / lymphocyte count between the two groups.3.Logistic regression analysis of factors related to PTS in patients with NMOSD:Univariate analysis showed that the course of disease,cervical spinal cord lesions(that is,cervical spinal cord involvement)and AQP4 antibody were possible related factors,taking the significant factors of univariate analysis as independent variables,the course of disease(OR=1.009,95%CI:1.001-1.018,P=0.035)and cervical spinal cord lesions(OR=5.797,95%CI:1.884-17.840,P=0.002)were obtained by multivariate analysis.That is,the course of disease and cervical spinal cord lesions are independent risk factors for PTS in patients with NMOSD.The longer the course of disease and the involvement of cervical spinal cord lesions,the more likely to develop PTS in NMOSD patients.The ROC curve showed that the area under the curve of the course of disease (AUC)was 0.617.When the course of the disease was 78(months),the Jordan index reached the maximum(1.318).At this time,the prediction sensitivity was 89.3% and the specificity was 42.4%.Conclusions:Upon analysis,our results showed that PTS was a common symptom in NMOSD,PTS has a significant impact on daily life and requires active intervention.Our results showed that PTS was a common symptom in NMOSD.Our study shows that the prevalence of PTS in NMOSD is 41.25%,and PTS is associated with myelitis and is a common symptom of NMOSD.Cervical spinal cord involvement may be the anatomical basis of PTS.The longer the course of the disease,the more likely it is to develop PTS.PTS occurs in the remission stage of the disease,and hormone therapy is ineffective.Sodium channel blockers such as carbamazepine and oxcarbazepine showed good therapeutic effects.We recommend carbamazepine as the first choice for the treatment of PTS.Oxcarbazepine can replace carbamazepine if patiens had an adverse reaction such as liver function damage and exfoliative dermatitis caused by carbamazepine.In order to confirm these conclusions,it is necessary to conduct a prospective study on a large number of patients.