Gökçen Ünverengil1, Ebru Nur Vanli Yavuz2, Erdem Tüzün3, Ece Erdağ3, Sevil Kabadayi2, Bilge Bilgiç1, Betül Baykan2. 1. Department of Pathology, İstanbul University İstanbul School of Medicine, İstanbul, Turkey. 2. Department of Neurology, İstanbul University İstanbul School of Medicine, İstanbul, Turkey. 3. Department of Neurology, İstanbul University Institute of Experimental Medicine Research, İstanbul, Turkey.
Abstract
INTRODUCTION: Antibodies directed against neuronal surface antigens have recently been identified in patients with focal temporal lobe epilepsy (TLE) of unknown cause and mesial TLE with hippocampal sclerosis (MTLE-HS), thereby emphasizing the role of autoimmunity in TLE. Antibodies to contactin-associated protein-like 2 (CASPR2) are prevalent in MTLE-HS patients. We aimed to find out whether anti-neuronal autoimmunity might be involved in CASPR2 antibody-related MTLE-HS. METHODS: Surgically resected medial temporal lobe specimens of seropositive and seronegative MTLE-HS patients were examined with hematoxylin and eosin and immunohistochemical staining using specific immune cell markers. RESULTS: Two of 5 CASPR2 antibody-positive MTLE-HS patients showed polymorphonuclear and mononuclear cells infiltrating the subarachnoidal region. One of these patients also showed mononuclear cell infiltration in the parenchyma of the temporal lobe cortex. Subarachnoidal and parenchymal infiltrates contained CD3+, CD8+, and CD68+ cells. None of the 13 seronegative MTLE-HS patients displayed cellular infiltrates in their brain samples, and all MTLE-HS patients showed marked neuronal cell loss but no immune cell infiltration in their hippocampi. CONCLUSION: Our results show that CASPR2 antibody-associated MTLE-HS can present with central nervous system inflammation; thus, this subtype of MTLE-HS might have an autoimmune origin.
INTRODUCTION: Antibodies directed against neuronal surface antigens have recently been identified in patients with focal temporal lobe epilepsy (TLE) of unknown cause and mesial TLE with hippocampal sclerosis (MTLE-HS), thereby emphasizing the role of autoimmunity in TLE. Antibodies to contactin-associated protein-like 2 (CASPR2) are prevalent in MTLE-HSpatients. We aimed to find out whether anti-neuronal autoimmunity might be involved in CASPR2 antibody-related MTLE-HS. METHODS: Surgically resected medial temporal lobe specimens of seropositive and seronegative MTLE-HSpatients were examined with hematoxylin and eosin and immunohistochemical staining using specific immune cell markers. RESULTS: Two of 5 CASPR2 antibody-positive MTLE-HSpatients showed polymorphonuclear and mononuclear cells infiltrating the subarachnoidal region. One of these patients also showed mononuclear cell infiltration in the parenchyma of the temporal lobe cortex. Subarachnoidal and parenchymal infiltrates contained CD3+, CD8+, and CD68+ cells. None of the 13 seronegative MTLE-HSpatients displayed cellular infiltrates in their brain samples, and all MTLE-HSpatients showed marked neuronal cell loss but no immune cell infiltration in their hippocampi. CONCLUSION: Our results show that CASPR2 antibody-associated MTLE-HS can present with central nervous system inflammation; thus, this subtype of MTLE-HS might have an autoimmune origin.
Authors: F Cendes; F Andermann; P Gloor; A Evans; M Jones-Gotman; C Watson; D Melanson; A Olivier; T Peters; I Lopes-Cendes Journal: Neurology Date: 1993-04 Impact factor: 9.910
Authors: Sarosh R Irani; Sian Alexander; Patrick Waters; Kleopas A Kleopa; Philippa Pettingill; Luigi Zuliani; Elior Peles; Camilla Buckley; Bethan Lang; Angela Vincent Journal: Brain Date: 2010-07-27 Impact factor: 13.501