伴黑质超声异常的副肿瘤性僵人综合征1例报道

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...

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

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 active movement.Serum GAD65 antibody positive,EMG showed myotonic,benzodiazepines are the preferred drug for the treatment of this disease,combined with GABA receptor agonist baclophene,the best effect.Immunosuppressive agents should be added to patients with paraneoplastic syndrome.