Expanded phenotypes and outcomes among 256 LGI1/CASPR2-IgG positive patients

Abstract

Objective: To describe an expanded phenotypic spectrum and longitudinal outcome in 256 LGI1-IgG or CASPR2-IgG seropositive patients. Methods: Patients were identified through service neural autoantibody evaluation. Ninety-five had longitudinal follow-up (7-456 months; median 35). Results: Among 3,910 patients tested, 196 were LGI1-IgG-positive; 51 were CASPR2-IgG-positive and 9 were dual-positive. CSF-testing was less sensitive than serum-testing, detecting only 24/38 LGI1-IgG-positive (63%) and 5/6 CASPR2-IgG-positive (83%). LGI1-IgG-positive specimens had higher VGKC-IgG immunoprecipitation values [0.33 nmol/L (0.02-5.14)] than CASPR2-IgG-positives [0.10 nmol/L (0.00-0.45; p<0.001)]. Of patients presenting with pain or PNS manifestations, 39% were LGI1-IgG-seropositive (7% had solely neuropathy or pain). Multivariate analysis identified age as the only significant predictor of CNS vs PNS involvement (>50 years; OR 15, p<0.001). “Paroxysmal-dizziness-spells,” a unique LGI1-IgG accompaniment (14% of patients), frequently delayed the diagnosis. T2-mesiotemporal hyperintensity was more common in LGI1-IgG-positives (41%) than in CASPR2-IgG-positives (p=0.033). T1-bright basal ganglia were confined to LGI1-IgG-positives with faciobrachial-dystonic seizures 9/39 (31%). Cancer was found in 44% of LGI1-IgG/CASPR2-IgG dual-seropositive patients (one third thymoma). Response to initial immunotherapy was favorable in 97%; mean onset modified-Rankin score was 3 (range 1-5), and at last follow-up was 1.74 (range 0-6), with 9% having severe refractory disability, 20% asymptomatic, 28% receiving immunotherapy and 58% antiepileptic medication. Interpretations: Older age is a strong predictor of CNS involvement in patients seropositive for CASPR2-IgG or LGI1-IgG. Pain, peripheral manifestations and stereotypic “paroxysmal-dizziness-spells” are common with LGI1-IgG. Response to initial immunotherapy is often favorable, but some patients remain severely disabled, requiring long-term immunotherapy and/or antiepileptic medications. This article is protected by copyright. All rights reserved.

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