RESUMO
Objective@#To investigate the clinicopathologic features of Rasmussen syndrome (RS) and to raise awareness of this rare disease.@*Methods@#Clinicopathologic data of 4 cases of RS were retrospectively analyzed at Beijing Haidian Hospital from 2008 to 2016.@*Results@#The clinical manifestations included epilepsia partialis continua and progressive neurologic deficits in all patients.MRI demonstrated unihemispheric focal cortical atrophy in all cases. The histopathologic changes included variable degrees of lymphocytic infiltrate within the cortex, subarachnoid space and perivascular cuffing.Microglial nodules and neuronophagia were seen. Mild to severe neuronal loss was noted with variable degrees of reactive gliosis. Spongy edema and cavitation were observed in focal cortex. Inflammation involving hippocampus was seen in one case. Three cases were accompanied by focal cortical dysplasia (FCD) Ⅲd. Immunohistochemical staining showed that the infiltrative lymphocytes were positive for CD3, CD8, granzyme B and TIA1 and the proliferating microglial cells were positive for CD68. NeuN positive neurons decreased significantly and reactive astrocytes were GFAP positive.@*Conclusions@#Pathologic changes of RS are similar to viral encephalitis and the inflammation is progressive and multifocal involving the hemisphere. The diagnosis of RS relies on pathologic features combined with clinical findings and neuroradiological examinations.
RESUMO
Epilepsies are the second most common neurological disease. The pathological mechanisms of this disease are not fully unders- tood. Several studies claim that inflammation plays a significant role both in structural and physiological changes that lead to the emergence of seizures. Although in some epilepsies, such as Rasmussen?s encephalitis, the inflammation has definite importance, in several other epileptic syndromes, the participation of inflammatory reaction still lacks evidence. In such cases, the experimental models are useful for reveal how cytokines, molecules that modulate the inflammatory response, may affect seizures and how seizures may change the expression of these inflammatory molecules. Even with these works, much remains to be clarified with regard to the influence of inflammation on epileptic syndromes. The purpose of this brief review is to discuss the links between inflammatory processes, the origin of crises, and tissue damages in epilepsy.
As epilepsias são a segunda doença neurológica mais frequentes. Os mecanismos patológicos dessa doença ainda não são completamente compreendidos. Vários trabalhos alegam que a inflamação tem um papel importante tanto nas alterações estruturais quanto fisiológicas que levam à geração de crises. Embora em alguns tipos de epilepsia, como a encefalite de Rasmussen, a inflamação tenha importância evidente, em várias outras síndromes epilépticas ainda faltam evidências para confirmar a participação da reação inflamatória. Nesses casos, os modelos experimentais são úteis para revelar como as citocinas, moléculas que modulam a resposta inflamatória, podem afetar as crises e como as crises podem alterar a expressão dessas moléculas inflamatórias. Mesmo com esses trabalhos, muito ainda precisa ser esclarecido com relação à influência da inflamação sobre as síndromes epilépticas. O objetivo desta breve revisão foi discutir as ligações entre os processos inflamatórios, a origem das crises e os danos teciduais na epilepsia.
Las epilepsias son la segunda enfermedad neurológica más común. Los mecanismos patológicos de esta enfermedad no se entienden completamente. Varios estudios afirman que la inflamación juega un papel importante tanto en los cambios estructurales como en los fisiológicos que conducen a la generación de las convulsiones. Aunque en algunos tipos de epilepsia, tales como la encefalitis de Rasmus- sen, la inflamación tiene una importancia evidente, en varios otros síndromes epilépticos todavía carecen de pruebas para confirmar la participación de la reacción inflamatoria. En estos casos, los modelos experimentales son útiles para revelar cómo las citoquinas, molé- culas que modulan la respuesta inflamatoria, pueden afectar a las convulsiones y cómo las convulsiones pueden cambiar la expresión de estas moléculas inflamatorias. Incluso con estos trabajos, queda mucho por aclarar con respecto a la influencia de la inflamación en los síndromes epilépticos. El propósito de esta breve revisión es discutir los vínculos entre los procesos inflamatorios, el origen de la crisis y el daño tisular en la epilepsia.