伴黑质超声异常的副肿瘤性僵人综合征1例报道  

A report of one case of paraneoplastic rigidity syndrome with abnormal substantia nigra ultrasound

作　　者：唐晶[1] 赵丽丽 张硕 姜立刚 TANG Jing;ZHAO Lili;ZHANG Shuo;JIANG Ligang(Affiliated Hospital of Jilin Medical College,Jilin 132013,China)

机构地区：[1]吉林医药学院附属医院,吉林吉林132013

出　　处：《中国实用神经疾病杂志》2021年第3期258-264,共7页Chinese Journal of Practical Nervous Diseases

基　　金：吉林省教育厅“十三五”科学技术研究规划项目(编号:JJKH20200459KJ);吉林市科技局医疗卫生指导性计划项目(编号:201830481)。

摘　　要：目的探究僵人综合征发病临床表现、免疫学检查、神经电生理(肌电图、黑质超声)特点及治疗方法。方法分析1例僵人综合征患者,并整理其临床资料,同时对临床特点、辅助检查(腹部增强CT、肺部CT、肌电图、黑质超声、血清免疫学检查、血清副肿瘤性神经综合征11项)及治疗经过进行详细梳理。结果本病例临床表现为左下肢肌肉抽搐伴僵硬、疼痛感,肌肉僵直抽搐后伴无力感,共跌倒5~6次,同时伴颈脊旁肌、束脊肌、腹肌肌肉僵直,随之出现肌肉痛性痉挛,免疫学检查超敏C反应蛋白6.4 mg/L(0.1~5.0 mg/L),抗链球菌溶血素“O”36.7 IU/mL(0~200.0 IU/mL),红细胞沉降率40.0 mm/h(0~20.0 mm/h);血清副肿瘤性神经综合征11项:抗GAD65+;多肿瘤标志物11项:甲胎蛋白(AFP)>500 ng/mL(0~10.0 ng/mL)。超声提示黑质区回声Ⅱ级。肌电图:针电极提示静息状态下可见正常运动电位单元,被动运动后可见大量运动单位电位,给予地西泮注射后呈静息状态下运动单位电位发放减弱,给予劳拉西泮及巴氯芬治疗后临床症状改善。结论僵人综合征是中枢神经系统自身免疫性疾病引起的一种以下肢肌肉过度收缩和发作性躯干肌、颈脊旁肌、束脊肌及腹部肌肉强直伴疼痛,肌肉僵硬时明显影响患者主动运动为主要特征的临床罕见病。血清GAD65抗体阳性、肌电图显示肌强直,治疗首选药物为苯二氮类药物,还需联用GABA受体激动剂巴氯芬,效果最佳。合并副肿瘤综合征者需加用免疫抑制剂。Objective To explore the clinical manifestations,immunological examination,neuroelectrophysiological(EMG,substantia nigra ultrasound)characteristics and treatment of stiff person syndrome.Methods The clinical work to find and 1 case of frozen syndrome patients were analyzed,and 1 case of frozen syndrome in patients with clinical data,as well as the clinical characteristics,auxiliary examinations(abdominal computed tomography(CT),lung CT enhancement,EMG,substantia nigra ultrasound and serum immunology examination,serum tumor nerve syndrome)and the treatment after a detailed comb,to improve the diagnosis and treatment of rare diseases of nervous system is of important reference value.Results The case presented as the left leg muscle twitching with stiffness,pain,muscle rigidity after convulsions with powerlessness,fall in the total 5-6 times,at the same time accompanied by cervical spinal muscle,ridge beam muscle and abdominal muscle rigidity,as a result,a muscle cramp pain,immunology examination allergic C-reactive protein 6.4mg/L(0.1-5.0mg/L),hemolysin against streptococcus“O”36.7IU/mL(0-200.0IU/mL).Erythrocyte sedimentation rate 40.0mm/h(0-20.0mm/h).Serum paraneoplastic neurological syndrome 11 items:anti-GAD65+.There were 11 tumor markers:alpha-fetoprotein(AFP)>500.0ng/mL(0-10.0ng/mL).Ultrasound indicated:echo levelⅡin the substantia nigra.Electromyography(EMG):the needle electrode indicated that normal motor potential units could be seen in resting state,and a large amount of motor unit potential could be seen after passive movement.After diazepam injection,the release of motor unit potential was weakened in resting state.After treatment with lorazepam and baclofen,the clinical symptoms were improved.Conclusion Rigor Mortis syndrome is an autoimmune disease of the central nervous system.It is a rare clinical disease characterized by excessive contraction of lower limb muscles and pain associated with parietal cervical,fascicular and abdominal muscle rigidity,which significantly affects the patient’s ac

关 键 词：僵人综合征 中枢神经系统自身免疫性疾病 抗GAD65 肌电图 黑质超声 苯二氮类 免疫抑制剂 

分 类 号：R746[医药卫生—神经病学与精神病学]