Neurological Syndromes Associated with Antibodies against Neuronal Surface Antigens


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‘ workplace: 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: Cesk Slov Neurol N 2015; 78/111(4): 453-458
Category: Short Communication
doi: https://doi.org/10.14735/amcsnn2015453

Overview

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-GABABR, 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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Labels
Paediatric neurology Neurosurgery Neurology

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Czech and Slovak Neurology and Neurosurgery

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