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Differential expression of major histocompatibility complex class I in developmental glioneuronal lesions

Avanita S Prabowo1, Anand M Iyer1, Jasper J Anink1, Wim GM Spliet2, Peter C van Rijen2 and Eleonora Aronica134*

Author Affiliations

1 Department of (Neuro)Pathology, Academic Medical Center, University of Amsterdam, Meibergdreef 9, Amsterdam, 1105 AZ, The Netherlands

2 Departments of Pathology and Neurosurgery /Rudolf Magnus Institute for Neuroscience, University Medical Center Utrecht, Utrecht, The Netherlands

3 SEIN – Stichting Epilepsie Instellingen Nederland, Heemstede, The Netherlands

4 Swammerdam Institute for Life Sciences, Center for Neuroscience, University of Amsterdam, Amsterdam, The Netherlands

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Journal of Neuroinflammation 2013, 10:12  doi:10.1186/1742-2094-10-12

Published: 24 January 2013



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.


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.


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.


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.

Focal cortical dysplasia; Ganglioglioma; Cortical tubers; Neurons; Microglia; Major histocompatibility complex (MHC) class I