Clinical characteristics of contactin-associated protenin-like 2 antibody positive autoimmune encephalitis

Abstract

Objective \nTo investigate the clinical manifestation, imaging changes, laboratory examination, treatment plan and prognosis of Caspr2 antibody positive autoimmune encephalitis. \n \n \nMethods \nThis paper reported 1 case of Caspr2 antibody positive autoimmune encephalitis admitted to Neurology Department in the First Affiliated Hospital of Zhengzhou University in June 2017, and summarized 9 cases of Caspr2 antibody positive autoimmune encephalitis reported in the literature. \n \n \nResults \nThere were 6 males and 4 females in 10 patients with autoimmune encephalitis with positive Caspr2 antibody. The median age of onset was 53 years (22-75 years). Among the 10 patients, 8 cases were positive for Caspr2 antibody (2 cases also positive for Caspr2 antibody in cerebrospinal fluid, 3 cases also positive for LGI1 antibody) and 2 cases were only positive for Caspr2 antibody in cerebrospinal fluid. Seven cases of abnormal cranial MRI manifested mainly temporal lobe and hippocampal T2 phase hyperintensity. All the 10 patients had subacute onset, of them the clinical manifestations included cognitive impairment, psychobehavioral abnormality, epilepsy, sleep disorder, autonomic neurological symptoms, pain and so on. All of the 10 patients had more than one symptom superimposed. Symptoms were improved in 8 cases after hormone and/or gamma globulin treatment, and 2 cases were on the mend after symptomatic treatment. \n \n \nConclusions \nCaspr2 antibody positive autoimmune encephalitis patients have a variety of clinical manifestations, and this sisease can implicate the central and peripheral neuromuscular system and the independent nervous system. Immunosuppressive therapy is effective on it. \n \n \nKey words: \nContactin-associated protein-like 2;\xa0Autoimmune encephalitis;\xa0Immunotherapy