Objective To summarize the clinical characteristics of patients with anti-contactin-associated protein 2(CASPR2)antibody-associated encephalitis.Methods and Results Total 13 patients diagnosed and treated for anti-CASPR2 antibody-associated encephalitis at The Third Affiliated Hospital,Sun Yat-Sen University from August 2017 to August 2022 were collected.Initial manifestations:9 patients exhibited limbic system symptoms,2 patients presented with peripheral nerve symptoms(both with limb pain),one patient had cerebellar ataxia,and one patient had limb weakness.Anti-CASPR2 antibody detection:all the 13 patients underwent serum anti-CASPR2 antibody testing,which was positive in all cases;10 patients underwent cerebrospinal fluid(CSF)anti-CASPR2 antibody testing,with 5 cases testing positive;3 patients had other concomitant positive autoimmune antibodies.Head MRI examination:among the 13 patients,2 patients head T2-FLAIR showed hyperintensity in the medial temporal lobes either unilaterally or bilaterally;2 patients had cerebral microbleeds.Treatment:2 patients were treated only with steroids,4 patients received only intravenous immunoglobulin(IVIg),5 patients were treated with steroids and IVIg,one patient received rituximab only,and one patient received a combination of IVIg,steroids,protein A immunoadsorption and rituximab.Follow-up:follow-up ranged from 1-5 years,with 2 patients lost to follow-up;one patient relapsed after self-discontinuation of medication and achieved a modified Rankin Scale(mRS)score of 0 after treatment;of the remaining 10 patients,one patient showed no symptom improvement,one patient had some symptoms preventing normal life,6 patients had normal life,2 patients died.Conclusions Anti-CASPR2 antibody-associated encephalitis has complex clinical characteristics,and serum antibody testing is crucial for diagnosis.
维普
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