Clinical analysis of 7 cases with anti-Caspr2 antibody-associated autoimmune encephalitis

Abstract

Objective: To investigate the clinical features, laboratory tests, imaging features, electroencephalogram (EEG) manifestations, treatment and prognosis of anti-CASPR2 antibody-associated autoimmune encephalitis with the purpose to improve the understanding of the disease. Methods: Seven patients diagnosed with CASPR2 antibody-associated encephalitis in the Fifth and first Affiliated Hospital of Zhengzhou University from January 2017 to January 2019 were selected. The clinical manifestations, course of disease, imaging, treatment plan and prognosis of the patients were retrospectively analyzed. Results: Of the 7 patients, 4 were male and 3 were female. The median age of onset was 34 years old (range, 11-66 years). Seizures (6/7), memory decline(5/7), and neuropsychiatric disorders (4/7) were common clinical manifestations. CASPR2 antibody was detected in the CSF of 3 patients (3/7) and the serum of all patients (7/7). Notable imaging changes were observed in 5 patients (5/7). All patients received immunotherapy and were followed up for 1 month to 2 years. One patient was diagnosed with rectal cancer with a poor prognosis; all the others got a good prognosis. Conclusions: CASPR2 antibody-related encephalitis is a rare autoimmune disease. It is considered to present with seizures, memory loss, psychiatric symptoms, dizziness and sleep disorders as the main clinical manifestations. Early identification and treatment of the disease can help the patients achieve a good prognosis.