Paraneoplastic neurologic syndromes and autoimmune encephalitis – 10-year monocentric observational study

Czech version

Authors: P. Potužník ¹; J. Vejskal ¹; K. Štibraná ¹; M. Peterka ^1,2; R. Tupý ³
Published in: Cesk Slov Neurol N 2025; 88(2): 101-108
Category: Original Paper
doi: https://doi.org/10.48095/cccsnn2025101

Overview

Aim: To introduce the current diagnostic criteria of paraneoplastic neurologic syndromes and autoimmune encephalitis and to provide experience with diagnosis and treatment using our own cohort. Methods: We conducted a 10-year observational study of paraneoplastic neurologic syndromes and autoimmune encephalitis in our department from 2014 to 2023; it was a combination of a retrospective and prospective follow-up. Diagnosis, clinical syndrome, antibodies, tumor type, disease course, treatment, and prognosis were determined according to the 2021 diagnostic criteria. Results: 23 patients with autoimmune encephalitis and paraneoplastic neurologic syndromes were identified. The mean age of diagnosis was 57.6 years, and 69.6% were female. The most common paraneoplastic neurologic syndrome/autoimmune encephalitis was limbic encephalitis. Anti-Yo was the most frequently determined antibody. Paraneoplastic neurologic syndromes were most frequently associated with small cell lung cancer (5 patients). 95.7% of patients underwent immunomodulatory therapy. Death during follow-up was related to tumor or paraneoplastic neurologic syndrome/autoimmune encephalitis in 47.8% of patients. Conclusion: The study confirms a low incidence of autoimmune encephalitis and paraneoplastic neurologic syndromes, but also a high mortality rate despite evolving treatments and diagnostic methods.

Keywords:

autoimmunity – Antibodies – cancer – autoimmune encephalitis – cerebrospinal fl uid – paraneoplastic neurologic syndrome

Sources

1. Honnorat J, Antoine JC. Paraneoplastic neurological syndromes. Orphanet J Rare Dis 2007; 2 (1): 22. doi: 10.1186/1750-1172-2-22.

2. Darnell RB, Posner JB. Paraneoplastic syndromes involving the nervous system. N Engl J Med 2003; 349 (3): 1543–1554. doi: 10.1056/NEJMra023009.

3. Graus F, Vogrig A, Muñiz-Castrillo S et al. updated diagnostic criteria for paraneoplastic neurologic syndromes. Neurol Neuroimmunol Neuroinflamm 2021; 8 (4): e1014. doi: 10.1212/NXI.0000000000001014.

4. Vogrig A, Gigli GL, Segatti S et al. Epidemiology of paraneoplastic neurological syndromes: a population-based study. J Neurol 2019; 267 (1): 26–35. doi: 10.1007/s00415-019-09544-1.

5. H’ebert J, Riche B, Vogrig A et al. Epidemiology of paraneoplastic neurologic syndromes and autoimmune encephalitides in France. Neurol Neuroimmunol Neuroinflamm 2020; 7 (6): e883. doi: 10.1212/NXI.0000000000 000883.

6. Hanin A, Cespedes J, Huttner A et al. Neuropathology of New-Onset Refractory Status Epilepticus (NORSE). J Neurol 2023; 270 (8): 3688–3702. doi: 10.1007/s00415-023-11726-x.

7. Graus F, Titulaer MJ, Balu R et al. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol 2016; 15 (4): 391–404. doi: 10.1016/S1474-4422 (15) 00401-9.

8. Mojžišová H, Krýsl D, Hanzalová J et al. Antibody-negative autoimmune encephalitis: a single-center retrospective analysis. Neurol Neuroimmunol Neuroinflamm 2023; 10 (6): e200170. doi: 10.1212/NXI.0000000000200170.

9. Český statistický úřad, Obyvatelstvo Plzeňského kraje – 2021 [online]. Dostupné z URL: https: //csu.gov.cz/ produkty/obyvatelstvo-plzenskeho-kraje-2022.

10. Krýsl D, Elišák M. Autoimunitní encefalitidy. Cesk Slov Neurol N 2015; 78/111 (1): 7–23. doi: 10.14735/am csnn20151.

11. van Sonderen A, Thijs RD, Coenders EC et al. Anti-LGI1 encephalitis: clinical syndrome and long-term follow-up. Neurology 2016; 87 (14): 1449–1456. doi: 10.1212/WNL.0000000000003173.

12. Irani SR, Stagg CJ, Schott JM et al. Faciobrachial dystonic seizures: the influence of immunotherapy on seizure control and prevention of cognitive impairment in a broadening phenotype. Brain 2013; 136 (Pt 10): 3151–3162. doi: 10.1093/brain/awt212.

13. Gadoth A, Pittock SJ, Dubey D et al. Expanded phenotypes and outcomes among 256 LGI1/CASPR2-IgG-positive patients. Ann Neurol 2017; 82 (1): 79–92. doi: 10.1002/ana.24979.

14. Yang J, Sun Q, Yang G. Pilomotor seizures in a patient with LGI1 encephalitis. Front Neurol 2020; 11: 61. doi: 10.3389/fneur.2020.00061.

15. de Bruijn MAAM, van Sonderen A, van Coevorden-Hameete MH et al. Evaluation of seizure treatment in anti-LGI1, anti-NMDAR, and anti-GABABR encephalitis. Neurology 2019; 92 (19): e2185–e2196. doi: 10.1212/WNL. 0000000000007475.

16. Dürr M, Nissen G, Sühs KW et al. CSF Findings in acute NMDAR and LGI1 antibody-associated autoimmune encephalitis. Neurol Neuroimmunol Neuroinflamm 2021; 8 (6): e1086. doi: 10.1212/NXI.00000000000 01086.

17. Uy CE, Binks S, Irani SR. Autoimmune encephalitis: clinical spectrum and management. Pract Neurol 2021; 21 (5): 412–423. doi: 10.1136/practneurol-2020-002567.

18. McKeon A, Tracy JA, Pittock SJ et al. Purkinje cell cytoplasmic autoantibody type 1 accompaniments: the cerebellum and beyond. Arch Neurol 2011; 68 (10): 1282–1289. doi: 10.1001/archneurol.2011.128.

19. Pranzatelli MR, Tate ED, McGee NR. Demographic, clinical, and immunologic features of 389 children with opsoclonus-myoclonus syndrome: a cross-sectional study. Front Neurol 2017; 8: 468. doi: 10.3389/fneur.2017.00468.

20. Graus F, Keime-Guibert F, Reñe R et al. Anti-Hu-associated paraneoplastic encephalomyelitis: analysis of 200 patients. Brain 2001; 124 (Pt 6): 1138–1148. doi: 10.1093/brain/124.6.1138.

21. Dubey D, Lennon VA, Gadoth A et al. Autoimmune CRMP5 neuropathy phenotype and outcome defined from 105 cases. Neurology 2018; 90 (2): e103–e110. doi: 10.1212/WNL.0000000000004803.

22. Dubey D, Jitprapaikulsan J, Bi H et al. Amphiphysin-IgG autoimmune neuropathy: a recognizable clinicopathologic syndrome. Neurology 2019; 93 (20): e1873–e1880. doi: 10.1212/WNL.00000000000 08472.

23. Graus F, Delattre JY, Antoine JC et al. Recommended diagnostic criteria for paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatry 2004; 75 (8): 1135–1140. doi: 10.1136/jnnp.2003.034447.

24. Graus F, Keime-Guibert F, Reñe R et al. Anti-Hu-associated paraneoplastic encephalomyelitis: analysis of 200 patients. Brain 2001; 124 (Pt 6): 1138–1148. doi: 10.1093/brain/124.6.1138.

25. Yu Z, Kryzer TJ, Griesmann GE et al. CRMP-5 neuronal autoantibody: marker of lung cancer and thymoma-related autoimmunity. Ann Neurol. 2001; 49 (2): 146–154.

26. Verma N, Jaffer MH, Kolli AS et al. Updates in the management of paraneoplastic syndrome. Semin Neurol 2024; 44 (1): 36–46. doi: 10.1055/s-0043-1777353.