Clinical analysis of 11 cases of autoimmune encephalitis with antibodies against contactin-associated protein-like 2

Abstract

Objective To explore the clinical features, auxiliary examinations, therapies and prognoses of patients with antibodies against contactin-associated protein-like 2 (CASPR2). Methods The clinical data of 11 anti-CASPR2 encephalitis patients who were admited to the People′s Hospital of Zhengzhou University from March 2015 to April 2018 were retrospectively analyzed. Results The age of these 11 cases was (35.6±19.4) years (ranged 20-74 years), and eight cases were females. There were seven cases with limbic encephalitis which included six cases of epilepsy, four cases of memory impairment, two cases of mental and behavioral abnormalities. Four cases had peripheral nerve hyperexcitability. Four cases had neuropathic pain. There were six cases with autonomic dysfunction including five cases of constipation, three cases of tachycardia, two cases of hyperhidrosis, two cases of urinary disorder. Seven cases had sleep disorder. Four cases had weight loss. Two cases showed cerebellar symptoms and two cases had hyponatremia. Magnetic resonance imaging scan of the brain showed abnormal signal in two cases, mainly involved medial temporal lobe and the hippocampus. Six cases underwent 18F-fluorodeoxyglucose positron emission tomography-computed tomography (PET-CT) examination, and three cases showed abnormalities, including two with temporal hypermetabolism and one with cortical hypermetabolism. Chest enhanced CT and PET-CT showed thymoma in one case. All cases received immunotherapy, and after treatment their symptoms were improved. Long-term follow-up was performed in nine cases, and three cases relapsed. Conclusions The major clinical manifestations of anti-CASPR2 encephalitis were limbic encephalitis, peripheral nerve hyperexcitability, neuropathic pain, autonomic dysfunction, insomnia and so on. Immunotherapy was effective and some patients may have recurrence. Key words: Autoantibodies; Encephalitis; Autoimmune encephalitis; Contactin associated protein-like 2