Abstract
Objective: The objective of the study was to determine the incidence of antibodies against neuronal surface antigens (NSA-ab) in patients with different types of epilepsy, in comparison with the subjects diagnosed with immune-mediated disorders.Methods: Forty patients with drug-resistant epilepsy (DRE) of unknown origin, 16 with post-stroke epilepsy, and 23 with systemic autoimmune disorders (SAD) with CNS involvement were included. NSA-ab were sought in serum using indirect immunofluorescence method. Relationships were analyzed between presence of NSA-ab and clinical presentation.Results: NSA-ab was detected in the sera from five patients: anti-DPPX in one patient, anti-AMPAR1/R2 in two, anti-LGI1 in one and, in one case, both anti-CASPR2 and DPPX IgG. Out of these five patients, three represented the SAD subgroup and two the DRE subgroup. None of the patients with post-stroke epilepsy was positive for NSA-ab.Significance: Autoimmune etiology is worth considering in patients with drug-resistant epilepsy of unknown origin. The presence of NSA-ab in patients with systemic autoimmune disorders may be caused by unspecifically enhanced autoimmune reactivity. NSA-ab seem not to be related to epilepsy resulting from ischemic brain injury.
Highlights
Epilepsy affects up to 1.0% of the world population, and its effective management still constitutes a challenge
In 2017, the International League Against Epilepsy (ILAE) Classification of the Epilepsies recognized a range of etiologic groups, with autoimmune background regarded for the first time as an independent cause for epilepsy [1]
antibodies against neuronal surface antigens (NSA-ab) was detected in the sera from five (6, 3%) patients: in two patients anti-AMPAR1/R2, in one patient anti-CASPR2, in two patients anti-DPPX, in one patient anti-LGI1
Summary
Epilepsy affects up to 1.0% of the world population, and its effective management still constitutes a challenge. Some isolated autoimmune CNS disorders have been identified with seizures as one of their core symptoms [2, 3]. IgG autoantibodies against proteins on the Autoimmune Epilepsy neuronal surface (NSA-ab) are considered as main pathogens and markers in these disorders. Apart from NSA-ab, antibodies targeted against intracellular antigens (Hu, Ma, CV2, amphiphysin, Delta/Notch-like EGF-related receptor, Sox, and glutamic acid decarboxylase) may account for a specific group of immune-mediated CNS orders, usually of paraneoplastic origin and infrequently associated with epileptic seizures [5]. The antibodies that target molecules on the surface of neurons seem most relevant in epileptology, the full spectrum of autoantibody-related epilepsies is yet to be understood [6]. Other autoantibodies target intracellular antigens, such as those directed against the enzyme glutamic acid decarboxylase (GAD) and onconeuronal antibodies
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