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Yıl:2007; Cilt: 44; Sayı: 3>> Özet
TAM METİN
Nöropsikiyatri Arşivi; 2007;44(3):101-107
Hücre Membranı Antijenlerine Karşı Antikorlarla İlişkili Limbik Ensefalit
E Tüzün
Istanbul Üniversitesi istanbul Tıp Fakültesi, Deneysel Tıp Araştırma Enstitüsü, Çapa, İstanbul, Turkey
Limbik ensefalit hastaları genellikle hızlı ilerleyişli bellek kaybı, psikiyatrik semptomlar, nöbetler, inflamatuar BOS bulguları ve temporal loblarda EEG ve MR değişiklikleri ile başvururlar. Bu bulgulara viral enfeksiyonlar ve sistemik otoimmün hastalıklar da sebep olabilmekle beraber, pek çok limbik ensefalit hastasında serum veya BOS'da antinöronal antikorlarla karakterize immun-aracılı bir etiyoloji (paraneoplastik veya non-paraneoplastik) mevcuttur. Bir zamanlar nadir olduğu düşünülen non-viral immun-aracılı limbik ensefalit ol­gularına giderek daha fazla rastlanmakta ve yeni immünolojik fenotipler ta­nımlanmaktadır. Yeni tanı tekniklerinin ortaya çıkmasıyla, önceden seronega-tif olduğu sanılan limbik ensefalit hastalarının çoğunda yeni serum veya BOS antikorları saptanmıştır. Bu antikorlar, büyük ölçüde, hipokampus ve serebel-lum nöropillerinde bulunan voltaj bağımlı potasyum kanalları ve N-metil-D-aspartat reseptörleri gibi hücre membranı antijenlerine karşı gelişmekte ve muh­temelen patojenik rol oynamaktadırlar. Ayrıca bu immünolojik fenotip diğer limbik ensefalit varyantlarına kıyasla kanser ve sitotoksik T hücresi infiltrasyonu ile daha az ilişkili olup immün sistemi baskılayıcı tedavi yöntemlerine daha iyi yanıt vermektedir
Limbic Encephalitis Associated with Antibodies to Cell Membrane Antigens
Limbic encephalitis patients usually present with rapidly progressive memory deficit, psychiatric symptoms, seizures, CSF inflammatory findings and EEG or MRI abnormalities in the temporal lobes. While these findings might be caused by viral infections or systemic autoimmune disorders, many patients with limbic encephalitis have an immune-mediated etiology (paraneoplastic or not) cha­racterized with serum or CSF antineuronal antibodies. Once considered rare, non-viral immune mediated limbic encephalitis is now increasingly recognized and new immunophenotypes are identified. With the advent of new diagnostic techniques, novel serum or CSF antibodies have been detected in the majority of limbic encephalitis patients previously regarded as seronegative. These an­tibodies are mainly directed against the cell membrane antigens such as volta­ge-gated potassium channels and N-methyl-D-aspartate receptors expressed by the hippocampal and cerebellar neuropil and play a significant pathogenic role. Furthermore, this immunopehonotype associates less frequently with can­cer or cytotoxic T cell infiltration than other limbic encephalitis variants and responds significantly better to immunosuppressive treatment methods.

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