%0 Journal Article
%T Differential expression of major histocompatibility complex class I in developmental glioneuronal lesions
%A Prabowo Avanita S
%A Iyer Anand M
%A Anink Jasper J
%A Spliet Wim GM
%J Journal of Neuroinflammation
%D 2013
%I BioMed Central
%R 10.1186/1742-2094-10-12
%X 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+ 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+, T-cytotoxic) with granzyme-B+ 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¨Cbrain barrier dysfunction, in these developmental lesions.
%K Focal cortical dysplasia
%K Ganglioglioma
%K Cortical tubers
%K Neurons
%K Microglia
%K Major histocompatibility complex (MHC) class I
%U http://www.jneuroinflammation.com/content/10/1/12