Contactin-associated protein-like 2 antibody-mediated neurological disease:clinical characters analysis of 12 cases / 中风与神经疾病杂志

ABSTRACT

@#Abstract Objective　To investigate clinical features,treatment and prognosis of patients with CASPR2-Ab associated neurological disease.Methods　The clinical data of 12 patients with CASPR2-Ab in Xuanwu Hospital,Capital Medical University from September 2016 to September 2019 were analyzed.Results　The age of 12 patients was 44.42±15.541 years old,and the ratio of male to female was 1∶1.Initial presenting symptoms comprised seizures (6/12),limb pain (2/12),limb weakness (3/12) and cerebellar speech (1/12).All patients developed central nervous system problems,presenting as seizures (8/12),mental abnormality (4/12) or memory decline (4/12).Five cases suffered from peripheral nervous system impairment,including muscle cramps (3/12),limb numbness (4/12),limb weakness (1/12),and neuropathic pain (2/12).Five patients got autonomic nervous system involved,manifested as hyperhidrosis.EMG showed abnormal spontaneous firing in four patients.Diffuse slow wave activity on background of EEG was found in six patients.Brain MRI in two cases showed hyperintensity lesions in bilateral temporal lobes on T2WI/FLAIR sequences,and tumor screening of one patient revealed benign thymoma.Ten patients got remission after immunotherapy.Conclusion　CASPR2-Ab associated disease tended to be susceptible to elderly male and middle-aged female,which extensively affected various nervous system.Early immunotherapy was effective in patients,especially those with seizure as the only symptom or with limbic encephalitis.