부산대학교 도서관

Purpose The expression of the major histocompatibility complex class I (MHC-I) in the brain has received considerable interest not only because of its fundamental role in the immune system, but also for its non-immune functions in the context of activity-dependent brain development and plasticity. Methods In the present study we evaluated the expression and cellular pattern of MHC-I in focal glioneuronal lesions associated with intractable epilepsy. MHC-I expression was studied in epilepsy surgery cases with focal cortical dysplasia (FCD I, n = 6; FCD IIa, n = 6 and FCD IIb, n = 15), tuberous sclerosis complex (TSC, cortical tubers; n = 6) or ganglioglioma (GG; n = 15) using immunocytochemistry. Evaluation of T lymphocytes with granzyme-B.sup.+ granules and albumin immunoreactivity was also performed. Results All lesions were characterized by MHC-I expression in blood vessels. Expression in both endothelial and microglial cells as well as in neurons (dysmorphic/dysplastic neurons) was observed in FCD II, TSC and GG cases. We observed perivascular and parenchymal T lymphocytes (CD8.sup.+, T-cytotoxic) with granzyme-B.sup.+ granules in FCD IIb and TSC specimens. Albumin extravasation, with uptake in astrocytes, was observed in FCD IIb and GG cases. Conclusions Our findings indicate a prominent upregulation of MHC-I as part of the immune response occurring in epileptogenic glioneuronal lesions. In particular, the induction of MHC-I in neuronal cells appears to be a feature of type II FCD, TSC and GG and may represent an important accompanying event of the immune response, associated with blood-brain barrier dysfunction, in these developmental lesions. Keywords: Focal cortical dysplasia, Ganglioglioma, Cortical tubers, Neurons, Microglia, Major histocompatibility complex (MHC) class I
Author(s): Avanita S Prabowo[sup.1] , Anand M Iyer[sup.1] , Jasper J Anink[sup.1] , Wim GM Spliet[sup.2] , Peter C van Rijen[sup.2] and Eleonora Aronica[sup.1,3,4] Introduction Recent clinical and neuropathological evidence [...]