Neurological Syndromes Associated with Antibodies against Neuronal Surface Antigens

Česká verzia

Authors: M. Elišák ¹; D. Krýsl ^1,2; J. Hanzalová ^1,3; I. Gažová ¹; I. Doležalová ^4,5; J. Slonková ⁶; P. Marusič ¹
Authors place of work: Neurologická klinika 2. LF UK a FN Motol, Praha ¹; Klinisk Neurofysiologi, Sahlgrenska Universitetssjukhuset, Göteborg, Švédsko ²; Ústav imunologie, 2. LF UK a FN Motol, Praha ³; Centrum pro epilepsie Brno, I. neurologická klinika LF MU a FN u sv. Anny v Brně ⁴; CEITEC – Středoevropský technologický institut, MU, Brno ⁵; Neurologická klinika LF OU a FN Ostrava ⁶
Published in the journal: Cesk Slov Neurol N 2015; 78/111(4): 453-458
Category: Short Communication
doi: https://doi.org/10.14735/amcsnn2015453

Summary

Background:
Neuronal surface antibodies are associated with numerous neurological symptoms. Better knowledge of these symptoms may improve identification of potential candidates for immunotherapy.

Aim:
Characterize clinical signs in patients with neuronal surface antibodies positivity.

Methods:
We detected neuronal surface antibodies in 11/2011–12/2013 in 224 patients (224 in serum and 37 in cerebrospinal fluid). We investigated anti-NMDAR, anti-AMPAR1, anti-AMPAR2, anti-GABA_BR, anti-LGI1, anti-CASPR2 using cell-based assays for indirect immunofluorescence (Euroimmun AG). We retrospectively analyzed clinical characteristics of patients with positive neuronal surface antibodies in serum or cerebrospinal fluid other than anti-NMDAR positive patients.

Results:
Neuronal surface antibodies were detected in 11 patients (seven males, median age 58). Six patients had anti-LGI1, four anti-CASPR2 and two anti-AMPAR1 antibodies (one patient had both anti-CASPR2 and anti-AMPAR1 antibodies). Clinical symptoms included chronic epilepsy (n = 5), acute encephalopathy (n = 5) accompanied by epileptic seizures in four patients and one patient presented with cerebellar syndrome and cognitive deficit. Two patients had coincidence of paraneoplastic antibodies (anti-Hu, anti-Ma2). Tumor (small cell lung carcinoma) was diagnosed in one patient (anti-AMPAR1). Eight patients improved following immunotherapy (corticosteroids, IVIG). Early immunotherapy was associated with better outcome.

Conclusion:
NS-Abs were mostly associated with limbic encephalitis and chronic temporal lobe epilepsy. Immunotherapy had better effect when applied early in the disease course.

Key words:
autoimmune diseases – encephalitis – antibodies to cell-surface proteins – limbic encephalitis

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE “uniform requirements” for biomedical papers.

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