Autoimmune encephalitis with CASPR2 antibody: A case series and updated literature review.
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OBJECTIVE: Contactin-associated protein-like 2 (CASPR2) encephalitis is an under-recognized and relatively rare type of autoimmune encephalitis (AE). Here, we investigated the clinical manifestations and outcomes in patients with CASPR2 encephalitis. METHODS: The data of patients with CASPR2 encephalitis were collected retrospectively, and their clinical features were analyzed. Additionally, clinical features and prognoses of CASPR2 encephalitis in previously reported studies were summarized and analyzed. RESULTS: Fifteen patients (median age: 52 years; age range: 10-81 years, nine were male) were studied. The main clinical manifestations studied included limbic system symptoms (73.3 %), seizures (53.3 %), mental-behavioral anomalies (20 %), peripheral nerve hyperexcitability (PNH) symptoms (20 %), paresthesia of limbs (20 %), fasciculation (13.3 %), ataxia (13.3 %), impaired consciousness (13.3 %), Morvan syndrome (13.3 %), cerebellar symptoms (13.3 %), as well as high tumor markers (26.7 %). The values of abnormal signals in brain magnetic resonance imaging (MRI) and abnormal electroencephalogram (EEG) were 33.3 % and 40 %, respectively. Thirteen (86.7 %) patients received first-line immunotherapy, 12 (75 %) showed complete recovery at the last follow-up (18 months) on the modified ranking scale (mRS = 0), but two (13.3 %) patients experienced relapses. In addition, we investigated the clinical data of 440 patients with CASPR2 encephalitis available in the literature and included 25 relevant publications, which involved 315 men. Furthermore, 37 patients developed tumors during the disease or recurrence. At the follow-up, 212 patients displayed a mRS score of ≤2, and the mRS scores of 50 patients ranged from 3 to 6. In total, 43 patients displayed disease relapse during discharge until the last follow-up, and immunotherapy improved their symptoms. Nevertheless, 14 patients died during the follow-up. We have summarized here the included cases in the light of relevant previous studies, and the associated results conformed to those from previous studies. CONCLUSIONS: Patients with CASPR2 encephalitis exhibited diverse clinical features and presented several symptoms, including limbic system symptoms, PNH symptoms, Morvan syndrome, and cerebellar symptoms. Most of the patients in the present study responded well to immunotherapy and exhibited a lower chance of recurrence in the short term. The review of the relevant literature on CASPR2 encephalitis from the viewpoint of clinical characteristics and prognoses of CASPR2 encephalitis further deepened our understanding of the disease. Thus, recurrence and concomitant tumors in patients with CASPR2 encephalitis should be continuously monitored.