Intimal thickening of meningeal arteries after serial corticectomies for Rasmussen encephalitis

Wagner, Aaron S.; Yin, Nicole S.; Mathern, Gary W.; Vinters, Harry V.

doi:10.1016/j.humpath.2011.10.011

Cited by 4 publications

(2 citation statements)

References 21 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…A role for monocytes in RE pathogenesis is therefore possible as is the case in viral encephalitis [59]. Since monocytes also produce vascular endothelial growth factor [60], this may explain the vascular changes that have been observed in resected RE but not CD brain tissue [61]. …”

Section: Discussionmentioning

confidence: 99%

Differential expression of interferon-γ and chemokine genes distinguishes Rasmussen encephalitis from cortical dysplasia and provides evidence for an early Th1 immune response

Owens

Huynh

Chang

et al. 2013

J Neuroinflammation

View full text Add to dashboard Cite

BackgroundRasmussen encephalitis (RE) is a rare complex inflammatory disease, primarily seen in young children, that is characterized by severe partial seizures and brain atrophy. Surgery is currently the only effective treatment option. To identify genes specifically associated with the immunopathology in RE, RNA transcripts of genes involved in inflammation and autoimmunity were measured in brain tissue from RE surgeries and compared with those in surgical specimens of cortical dysplasia (CD), a major cause of intractable pediatric epilepsy.MethodsQuantitative polymerase chain reactions measured the relative expression of 84 genes related to inflammation and autoimmunity in 12 RE specimens and in the reference group of 12 CD surgical specimens. Data were analyzed by consensus clustering using the entire dataset, and by pairwise comparison of gene expression levels between the RE and CD cohorts using the Harrell-Davis distribution-free quantile estimator method.ResultsConsensus clustering identified six RE cases that were clearly distinguished from the CD cases and from other RE cases. Pairwise comparison showed that seven mRNAs encoding interferon-γ, CCL5, CCL22, CCL23, CXCL9, CXCL10, and Fas ligand were higher in the RE specimens compared with the CD specimens, whereas the mRNA encoding hypoxanthine-guanine phosphoribosyltransferase was reduced. Interferon-γ, CXCL5, CXCL9 and CXCL10 mRNA levels negatively correlated with time from seizure onset to surgery (P <0.05), whereas CCL23 and Fas ligand transcript levels positively correlated with the degree of tissue destruction and inflammation, respectively (P <0.05), as determined from magnetic resonance imaging (MRI) T2 and FLAIR images. Accumulation of CD4+ lymphocytes in leptomeninges and perivascular spaces was a prominent feature in RE specimens resected within a year of seizure onset.ConclusionsActive disease is characterized by a Th1 immune response that appears to involve both CD8+ and CD4+ T cells. Our findings suggest therapeutic intervention targeting specific chemokine/chemokine receptors may be useful in early stage RE.

show abstract

Section: Discussionmentioning

confidence: 99%

Differential expression of interferon-γ and chemokine genes distinguishes Rasmussen encephalitis from cortical dysplasia and provides evidence for an early Th1 immune response

Owens

Huynh

Chang

et al. 2013

J Neuroinflammation

View full text Add to dashboard Cite

show abstract

“…Discussions at a recent symposium/workshop on RE, involving experts on inflammatory, infectious and autoimmune CNS disorders, centered on whether RE is a brain infection resulting from an as yet undiscovered/unrecognized (probably viral) agent, an anomalous CNS response to a ubiquitous agent, or an autoimmune disease. Recently, interesting vascular changes have been described in resected RE cortical specimens, especially in the second of 'staged' operations for this disorder 26 .…”

Section: Table: Structural Lesions Associated With Intractable Epilepsymentioning

confidence: 99%

Neuropathologic Studies of 'Epileptogenic' Tissue

Vinters¹

2012

Can. J. Neurol. Sci.

Self Cite

View full text Add to dashboard Cite

Suppl. 6 -S20The surgical treatment of intractable epilepsy is now a widely accepted therapeutic modality. The University of Western Ontario (UWO) Epilepsy Surgery Programme, whose success was celebrated at this symposium in London, Ontario, has contributed greatly to that acceptance, and deserves hearty congratulations for this effort, which continues today. Indeed, as presentations during the celebratory day proved, the innovative UWO Epilepsy Programme moves from strength to strength.One result of the growth of epilepsy surgery programs worldwide is that this innovative treatment provides a bounty of interesting lesions for Neuropathologists to examine and study. Unfortunately, since epilepsy surgery is most commonly carried out in medical centers with excellent neuroimaging and EEG facilities, together with expensive intraoperative support including anesthesia, this procedure is often not available to thousands of individuals who would benefit from it 1 . In the early days of therapeutic epilepsy surgery, Neuropathologists often struggled with the appropriate nomenclature for unique lesions that originated within epileptogenic brain-and to some extent this remains a problem to the present time. However, the meticulous approach to specimen analysis, exemplified by the early work of Gordon Mathieson at the Montreal Neurological ABSTRACT: This mini-review will summarize some of the major contributions of Neuropathology to understanding the structural basis of several forms of intractable epilepsy. Because of space limitations, only selected morphologically defined brain abnormalities will be considered and illustrated. By definition, the types of specimen encountered by Neuropathologists are those resected to treat intractable seizure disorders. One can therefore infer that the 'lesions' encountered in some way caused or at least contributed to the epilepsy, though the leap from observed neuropathologic abnormalities to 'seizuregenesis' is not always straightforward-indeed often is very tortuous and obscure. Nevertheless, a Neuropathologist working with a team of neurologists, neurosurgeons, electrophysiologists, neuropsychologists, and others, can contribute to a richer understanding of why seizures are triggered within human brain tissue, and therefore how they may be more effectively treated.RÉSUMÉ: Études neuropathologiques du tissu épileptogène. Cette mini-revue constitue un sommaire de certaines des contributions majeures de la neuropathologie à la compréhension des éléments structuraux de base de plusieurs types d'épilepsies pharmacorésistantes. Nous avons choisi de traiter et d'illustrer certaines anomalies cérébrales définies au point de vue morphologique, étant donné les contraintes d'espace. Par définition, les spécimens qu'examinent les neuropathologistes sont ceux qui ont été prélevés au cours du traitement chirurgical de l'épilepsie pharmacorésistante. On peut donc déduire que les « lésions » observées ont causé en quelque sorte ou ont pour le moins contribué à l'épilepsie, bien que le lien entre ...

show abstract