Abstract
Amaç: Hastalık ile ilgili farkındalığının artmasına paralel olarak son yıllarda otoimmün ensefalit tanısı alan hasta sayısı artmaktadır. Merkezi sinir sistemi enfeksiyonlarında görmeye alışık olduğumuz ateş, ense sertliği, bulantı, kusma ve konfüzyon gibi semptomların daha arka planda olduğu ve bilişsel bozukluklar, davranış sorunları ve psikiyatrik bulguların ön planda olduğu otoimmün ensefalit vakalarında tanı gecikmektedir. Özellikle hücre yüzey antijenlerine karşı antikor oluşumu olan vakalarda erken tanı ve tedavi ile prognoz iyidir.
Yöntemler: Bu yazıda hücre yüzey antijenlerine karşı antikor oluşumu ile seyreden otoimmün ensefalitler grubunda yer alan Kontaktin ilişkili protein-2’e (CASPR-2) karşı antikor pozitifliği ile seyreden otoimmün ensefalit olgusu sunulmuştur.
Bulgular: Hastamız immünoterapiye iyi yanıt vermiş, takip eden 1 yıl içerisinde rekürens gözlenmemiş ve malignite saptanmamıştır.
Sonuç: Subakut bir süreçte konfüzyon, epileptik nöbetler, halüsinasyonlar ve nonspesifik duysal semptomlarla gelen hastalarda ayırıcı tanıda otoimmün ensefalit düşünülmelidir. Erken tanı ve tedavi şansı kaçırılmamalıdır.
References
- Kocasoy Orhan E, Çakar A, Akman-Demir G. Sinir Sistemi İnfeksiyonları. Öge AE, Baykan B, Bilgiç B (Ed). Nöroloji Ders e-kitabı. 4. baskı. İstanbul: Nobel Tıp Kitapevleri, 2020.
- Dervişoğlu P, Kösecik M. Meninks İrritayon Bulgularıyla Başvuran Atipik Kawasaki Hastalığı. Sakarya Tıp Dergisi 2016, 6(3):175-178.
- Nissen MS, Ryding M, Meyer M, Blaabjerg M. Autoimmune Encephalitis: Current Knowledge on Subtypes, Disease Mechanisms and Treatment. CNS & Neurological Disorders-Drug Targets. Volume 19, Issue 8, 2020. Page:572-583.
- Graus F, Titulaer MJ, Balu R, Benseler S, Bien CG, Cellucci T et al. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol 2016;15(4):391-404
- Saint-Martin M, Joubert B, Pellier-Monnin V, Pascual O, Noraz N, Jérôme Honnorat. Contactin-associated protein-like 2, a protein of the neurexin family involved in several human diseases. Eur J Neurosci 2018;48:1906-1923.
- Pinatel D, Hivert B, Saint-Martin M, Noraz N, Savvaki M, Karagogeos D et al. The Kv1-associated molecules TAG-1 and Caspr2 are selectively targeted to the axon initial segment in hippocampal neurons. J Cell Sci 2017;130(13):2209-2220.
- Arancibia-Carcamo IL, Attwell D. The node of Ranvier in CNS pathology. Acta Neuropathol 2014;128:161-75.
- Breis LC, Schlindwein MAM, Gonçalves MVM. Anti-CASPR2 antibodies clinical significance and its main phenotypes. Neurosciences 2020;7:330-334.
- Ahmed A, Simmons Z. Isaacs syndrome: a review. Muscle Nerve 2015;52:5-12.
- Qin X, Yang H, Zhu F, Wang Q, Shan W. Clinical Character of CASPR2 Autoimmune Encephalitis: A Multiple Center Retrospective Study. Front Immunol. 2021; 12: 652864. https://doi.org/10.3389/fimmu.2021.652864.
- Nikolaus M, Jackowski-Dohrmann S, Prüss H, Schuelke M, Knierim E. Morvan syndrome associated with CASPR2 and LGI1 antibodies in a child. Neurology 2018;90:183-185.
- Irani SR, Pettingill P, Kleopa KA, Schiza N, Waters P, Mazia C et al. Morvan syndrome: Clinical and serological observations in 29 cases. Ann Neurol 2012;72(2):241-255.
- Song J, Jing S, Quan C, Lu J, Qiao X, Kai Qiao et al. Isaacs syndrome with CASPR2 antibody: A series of three cases. J Clin Neurosci 2017;41:63-6.
- Sawlani K, Katirji B. Peripheral nerve hyperexcitability syndromes. Continuum (Minneap Minn) 2017;23:1437-50.
Abstract
Objective: In parallel with the increase in awareness about the disease the number of patients diagnosed with autoimmune encephalitis has been increasing in recent years. Diagnosis is delayed in cases of autoimmune encephalitis where symptoms such as fever, neck stiffness, nausea, vomiting and confusion, which we are used to seeing in central nervous system infections, are more in the background and cognitive disorders, behavioral problems and psychiatric findings are at the forefront. The prognosis is good with early diagnosis and treatment especially in cases with the formation of antibodies against cell surface antigens.
Methods: In this article a case of autoimmune encephalitis with Contactin-related protein-2 (CASPR-2) antibody positivity, which is in the group of autoimmune encephalitis with the formation of antibodies against cell surface antigens is presented.
Results: Our patient responded well to immunotherapy, no recurrence was observed during the following 1 year and no malignancy was detected.
Conclusion: In patients presenting with confusion, epileptic seizures, hallucinations and non-specific sensory symptoms during a subacute process autoimmune encephalitis should be considered in the differential diagnosis. The chance for early diagnosis and treatment should not be missed.
Keywords
Autoimmune encephalitis Contactin-Related Protein-2 (CASPR-2) Early diagnosis and treatment
References
- Kocasoy Orhan E, Çakar A, Akman-Demir G. Sinir Sistemi İnfeksiyonları. Öge AE, Baykan B, Bilgiç B (Ed). Nöroloji Ders e-kitabı. 4. baskı. İstanbul: Nobel Tıp Kitapevleri, 2020.
- Dervişoğlu P, Kösecik M. Meninks İrritayon Bulgularıyla Başvuran Atipik Kawasaki Hastalığı. Sakarya Tıp Dergisi 2016, 6(3):175-178.
- Nissen MS, Ryding M, Meyer M, Blaabjerg M. Autoimmune Encephalitis: Current Knowledge on Subtypes, Disease Mechanisms and Treatment. CNS & Neurological Disorders-Drug Targets. Volume 19, Issue 8, 2020. Page:572-583.
- Graus F, Titulaer MJ, Balu R, Benseler S, Bien CG, Cellucci T et al. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol 2016;15(4):391-404
- Saint-Martin M, Joubert B, Pellier-Monnin V, Pascual O, Noraz N, Jérôme Honnorat. Contactin-associated protein-like 2, a protein of the neurexin family involved in several human diseases. Eur J Neurosci 2018;48:1906-1923.
- Pinatel D, Hivert B, Saint-Martin M, Noraz N, Savvaki M, Karagogeos D et al. The Kv1-associated molecules TAG-1 and Caspr2 are selectively targeted to the axon initial segment in hippocampal neurons. J Cell Sci 2017;130(13):2209-2220.
- Arancibia-Carcamo IL, Attwell D. The node of Ranvier in CNS pathology. Acta Neuropathol 2014;128:161-75.
- Breis LC, Schlindwein MAM, Gonçalves MVM. Anti-CASPR2 antibodies clinical significance and its main phenotypes. Neurosciences 2020;7:330-334.
- Ahmed A, Simmons Z. Isaacs syndrome: a review. Muscle Nerve 2015;52:5-12.
- Qin X, Yang H, Zhu F, Wang Q, Shan W. Clinical Character of CASPR2 Autoimmune Encephalitis: A Multiple Center Retrospective Study. Front Immunol. 2021; 12: 652864. https://doi.org/10.3389/fimmu.2021.652864.
- Nikolaus M, Jackowski-Dohrmann S, Prüss H, Schuelke M, Knierim E. Morvan syndrome associated with CASPR2 and LGI1 antibodies in a child. Neurology 2018;90:183-185.
- Irani SR, Pettingill P, Kleopa KA, Schiza N, Waters P, Mazia C et al. Morvan syndrome: Clinical and serological observations in 29 cases. Ann Neurol 2012;72(2):241-255.
- Song J, Jing S, Quan C, Lu J, Qiao X, Kai Qiao et al. Isaacs syndrome with CASPR2 antibody: A series of three cases. J Clin Neurosci 2017;41:63-6.
- Sawlani K, Katirji B. Peripheral nerve hyperexcitability syndromes. Continuum (Minneap Minn) 2017;23:1437-50.
Details
| Primary Language | English |
|---|---|
| Subjects | Clinical Sciences (Other) |
| Journal Section | Case Report |
| Authors | |
| Early Pub Date | October 2, 2024 |
| Publication Date | September 30, 2024 |
| Submission Date | July 30, 2024 |
| Acceptance Date | September 11, 2024 |
| Published in Issue | Year 2024 Volume: 14 Issue: 3 |
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