Tedaviye dirençli Anti-LGI1 ilişkili otoimmün ensefalit olgusu

Öz: Anti-LGI1ilişkili otoimmün ensefalit hafıza kusuru, çeşitli nöropsikiyatrik semptomlar, davranış bozuklukları ve nöbetler gibi limbik sistemin tutulumuna işaret eden klinik belirtilerle karakterizedir. Nöbetler genel olarak antiepileptik ilaçlara dirençlidir. Ancak immün tedavi ile oldukça dramatik bir nöbet kontrolü sağlanabilir. Burada sunulan 67 yaşındaki erkek hasta dirençli sol fasiobrakial distonik nöbetler nedeniyle ile başvurmuştur. LGI1 otoantikoru pozitif olan ve kognitif defisit tespit edilen hasta, kısa süreli immün tedavilerden fayda görmüşse de 3 ay içinde başlayan nöbet nüksü ve kognitif kötüleşme ancak uzun süreli immünsupresyon ile kontrol altına alınabilmiştir. Bu olgu bazında anti-LGI1 ilişkili otoimmün ensefalitin prezentasyonu, tanısı ve bazı dirençli vakalarda uzun süreli immunsupresif tedavinin nöbet kontrolüne ve kognitif düzelmeye katkısının vurgulanmıştır.

Anahtar Kelimeler:

otoimmünensefalit, epilepsi, nöbet, steroid

A case with treatment resistant Anti-LGI1 related autoimmune encephalitis

Abstract: Anti-LGI1 associated autoimmune encephalitis is characterized by cognitive deficits, various neuro-psychiatric symptoms, behavioral disorders and seizures that point to involvement of the limbic system. Seizures are frequently resistant to antiepileptic medications, however they can be dramatically controlled by immunological treatment modalities. In our case, a 67-year-old male patient presented with refractory epilepsy. His left fasciobrachial dystonic seizures recurring several times a day were patognomonic for anti-LGI1 associated encephalitis. Besides frequent seizures, he also had significant cognitive deficit. Short term immune therapies improved cognition and he remained seizure free for 3 months. Unfortunately, after 3 months his seizure frequency and cognitive problems increased gradually. In this case report, we discussed the presenting features and diagnosis of antibody-LGI1 autoimmune encephalitis and emphasized the contribution of long term immunosuppressive therapy to seizure control and cognitive improvement.

Keywords:

Autoimmuneencephalitis, epilepsy, seizure, steroid,

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