[doi: 10.5505/2017ichc.PP-208]
Rasmussen’s Encephalitis: A Case Report
İrsel Tezer Filik1, Ayşegül Fırat2, Işık Ünal3, Burçak Bilginer4, Figen Kaymaz3, Figen Söylemezoğlu5, Serap Saygı12Department of Anatomy, Faculty of Medicine, Hacettepe University, Ankara, Turkey
3Department of Histology and Embryology, Faculty of Medicine, Hacettepe University, Ankara, Turkey
4Department of Neurosurgery, Faculty of Medicine, Hacettepe University, Ankara, Turkey
5Department of Pathology, Faculty of Medicine, Hacettepe University, Ankara, Turkey
Introduction & Objectives
Rasmussen’s encephalitis is a progressive disease characterised by drug-resistant focal epilepsy, progressive hemiplegia, and cognitive decline with unihemispheric brain atrophy. It is a rare disease and affects children and young adults. The characteristic histopathological findings of Rasmussen’s encephalitis are cortical inflammation, neuronal loss, and gliosis located in one cerebral hemisphere. Microglial and lymphocytic nodules, perivascular infiltration and neuronal death are the most common pathological features. End-stage features include cortical cavitation, marked astrogliosis, and neuronal cell loss. There is a growing interest to the immunological and inflammatory mechanisms of the neurological diseases. Our aim in this study was to evaluate some antibody related immunohistological parameters of a Rasmussen’s encephalitis case.
Materials & Methods
Our study was composed of a pathologically proven case of Rasmussen’s encephalitis treated and followed in our neurology clinic. The diagnosis of the case was confirmed with magnetic resonance imaging and ictal-interictal electroencephalographic findings. The patient was pharmacoresistant to the seizures and therefore surgically operated by left hemispherectomy. Pathological examinations confirmed encephalitis and hippocampal sclerosis. Postsurgically, frozen brain tissue was prepared to be stained with anti-NMDA antibody, anti-VKGC antibody, anti-GAD antibody, anti-NeuN antibody, anti-GFAP antibody and anti CD-3 and anti CD-8 antibodies to evaluate the immunological parameters semi-quantitatively.
Results
In the sections perivascular inflammatory cells and lymphocyte infiltration were prominent in temporal lobe, hippocampus and frontal lobe sections. Neuronal atrophy was also prominent in all regions. Especially GFAP (+) gliosis was a common finding.
Conclusions
Immunotherapies of patients with neurological diseases has been changing very rapidly. Such descriptive studies with immunohistochemistry will help to evaluate the clinical outcome and improve the treatment of the cases.
Figure-1
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Hippocampal neurons, anti-NeuN antibody, X200.
Figure-2
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Temporal lobe, anti human IgG, x 100