Abstract
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
References
- KAYNAKLAR: 1)Goodfellow JA, Mackay GA. Autoimmune encephalitis. J R Coll Physicians Edinb. 2019 Dec;49(4):287-294. doi: 10.4997/JRCPE.2019.407.
- 2) Li Z, Cui T, Shi W, Wang Q. Clinical analysis of leucine-rich glioma inactivated-1 protein antibody associated with limbic encephalitis onset with seizures. Medicine (Baltimore). 2016 Jul;95(28):e4244. doi: 10.1097/MD.0000000000004244.
- 3)Andrade DM, Tai P, Dalmau J ve ark.Tonic seizures: a diagnostic clue of anti-LGI1 encephalitis? Neurology. 2011 Apr 12;76(15):1355-7. doi:10.1212/WNL.0b013e3182152808.
- 4)Irani SR, Michell AW, Lang B ve ark.Faciobrachial dystonic seizures precede LGI1 antibody limbic encephalitis. Ann Neurol. 2011 May;69(5):892-900. doi:10.1002/ana.22307.
- 5)Griffith SP, Malpas CB, Alpitsis R, O'Brien TJ, Monif M. The neuropsychological spectrum of anti-LGI1 antibody mediated autoimmune encephalitis. J Neuroimmunol. 2020 Aug 15;345:577271. doi: 10.1016/j.jneuroim.2020.577271
- 6)Li X, Yuan J, Liu L, Hu W. Antibody-LGI 1 autoimmune encephalitis manifesting as rapidly progressive dementia and hyponatremia: a case report and literature review. BMC Neurol. 2019 Feb 7;19(1):19. doi: 10.1186/s12883-019-1251-4.
- 7)Irani SR, Gelfand JM, Bettcher BM, Singhal NS, Geschwind MD. Effect of rituximab in patients with leucine-rich, glioma-inactivated 1 antibody-associated encephalopathy. JAMA Neurol. 2014 Jul 1;71(7):896-900. doi:10.1001/jamaneurol.2014.463.
- 8)Shin YW, Lee ST, Shin JW ve ark. VGKC-complex/LGI1-antibody encephalitis: clinical manifestations and response to immunotherapy. J Neuroimmunol. 2013 Dec 15;265(1-2):75-81. doi: 10.1016/j.jneuroim.2013.10.005.
- 9)Shen CH, Fang GL, Yang F ve ark. Seizures and risk of epilepsy in anti-NMDAR, anti-LGI1, and anti-GABAB R encephalitis. Ann Clin Transl Neurol. 2020 Aug;7(8):1392-1399. doi: 10.1002/acn3.51137.
Abstract
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.
Keywords
References
- KAYNAKLAR: 1)Goodfellow JA, Mackay GA. Autoimmune encephalitis. J R Coll Physicians Edinb. 2019 Dec;49(4):287-294. doi: 10.4997/JRCPE.2019.407.
- 2) Li Z, Cui T, Shi W, Wang Q. Clinical analysis of leucine-rich glioma inactivated-1 protein antibody associated with limbic encephalitis onset with seizures. Medicine (Baltimore). 2016 Jul;95(28):e4244. doi: 10.1097/MD.0000000000004244.
- 3)Andrade DM, Tai P, Dalmau J ve ark.Tonic seizures: a diagnostic clue of anti-LGI1 encephalitis? Neurology. 2011 Apr 12;76(15):1355-7. doi:10.1212/WNL.0b013e3182152808.
- 4)Irani SR, Michell AW, Lang B ve ark.Faciobrachial dystonic seizures precede LGI1 antibody limbic encephalitis. Ann Neurol. 2011 May;69(5):892-900. doi:10.1002/ana.22307.
- 5)Griffith SP, Malpas CB, Alpitsis R, O'Brien TJ, Monif M. The neuropsychological spectrum of anti-LGI1 antibody mediated autoimmune encephalitis. J Neuroimmunol. 2020 Aug 15;345:577271. doi: 10.1016/j.jneuroim.2020.577271
- 6)Li X, Yuan J, Liu L, Hu W. Antibody-LGI 1 autoimmune encephalitis manifesting as rapidly progressive dementia and hyponatremia: a case report and literature review. BMC Neurol. 2019 Feb 7;19(1):19. doi: 10.1186/s12883-019-1251-4.
- 7)Irani SR, Gelfand JM, Bettcher BM, Singhal NS, Geschwind MD. Effect of rituximab in patients with leucine-rich, glioma-inactivated 1 antibody-associated encephalopathy. JAMA Neurol. 2014 Jul 1;71(7):896-900. doi:10.1001/jamaneurol.2014.463.
- 8)Shin YW, Lee ST, Shin JW ve ark. VGKC-complex/LGI1-antibody encephalitis: clinical manifestations and response to immunotherapy. J Neuroimmunol. 2013 Dec 15;265(1-2):75-81. doi: 10.1016/j.jneuroim.2013.10.005.
- 9)Shen CH, Fang GL, Yang F ve ark. Seizures and risk of epilepsy in anti-NMDAR, anti-LGI1, and anti-GABAB R encephalitis. Ann Clin Transl Neurol. 2020 Aug;7(8):1392-1399. doi: 10.1002/acn3.51137.
Details
| Primary Language | Turkish |
|---|---|
| Subjects | Neurology and Neuromuscular Diseases |
| Journal Section | Case Report |
| Authors | |
| Publication Date | January 1, 2022 |
| Submission Date | March 8, 2021 |
| Acceptance Date | March 29, 2021 |
| Published in Issue | Year 2022 Volume: 15 Issue: 1 |
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