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Journal of Neurology

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24.09.2019 | Original Communication

Epidemiology of paraneoplastic neurological syndromes: a population-based study

Erschienen in: Journal of Neurology | Ausgabe 1/2020

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Abstract

Background

The epidemiology of paraneoplastic neurological syndromes (PNS) remains to be defined. We present here the first population-based incidence study and report the clinical spectrum and antibody profile of PNS in a large area in Northeastern Italy.

Methods

We performed a 9-year (2009–2017) population-based epidemiological study of PNS in the provinces of Udine, Pordenone and Gorizia, in the Friuli-Venezia Giulia region (983,190 people as of January 1, 2017). PNS diagnosis and subgroups were defined by the 2004 diagnostic criteria. Age- and sex-adjusted incidence rates were calculated.

Results

We identified 89 patients with a diagnosis of definite PNS. Median age was 68 years (range 26–90), 52% were female. The incidence of PNS was 0.89/100,000 person-years. PNS incidence rates increased over time from 0.62/100,000 person-years (2009–2011), 0.81/100,000 person-years (2012–2014) to 1.22/100,000 person-years (2015–2017). The prevalence of PNS was 4.37 per 100,000. Most common PNS were limbic encephalitis (31%), cerebellar degeneration (28%) and encephalomyelitis (20%). Among antibody (Ab)-positive cases, most frequent specificities included: Yo (30%), Hu (26%), and Ma2 (22%), while the most frequent associated tumors were lung (17%) and breast cancer (16%), followed by lymphoma (12%). PNS developed in 1 in every 334 cancers in our region. Statistically significant associations were observed between cancer type and Ab-specificity (P < 0.001), and between neurological syndrome and Ab-specificity (P < 0.001).

Conclusions

This first population-based study found an incidence of PNS that approximates 1/100,000 person-years and a prevalence of 4/100,000. Moreover, the incidence of PNS is increasing over time, probably due to increased awareness and improved detection techniques.

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

Honnorat J, Antoine J-C (2007) Paraneoplastic neurological syndromes. Orphanet J Rare Dis 2:22. https://doi.org/10.1186/1750-1172-2-22 CrossRefPubMedPubMedCentral

Dalmau J, Graus F (2018) Antibody-mediated encephalitis. N Engl J Med 378:840–851. https://doi.org/10.1056/NEJMra1708712 CrossRefPubMed

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

Vogrig A, Joubert B, Ducray F et al (2018) Glioblastoma as differential diagnosis of autoimmune encephalitis. J Neurol 265:669–677. https://doi.org/10.1007/s00415-018-8767-1 CrossRefPubMed

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

Bettini M, Chaves M, Cristiano E et al (2017) Incidence of autoimmune myasthenia gravis in a health maintenance organization in Buenos Aires, Argentina. Neuroepidemiology 48:119–123. https://doi.org/10.1159/000477733 CrossRefPubMed

Breiner A, Widdifield J, Katzberg HD et al (2016) Epidemiology of myasthenia gravis in Ontario, Canada. Neuromuscul Disord 26:41–46. https://doi.org/10.1016/j.nmd.2015.10.009 CrossRefPubMed

Martinka I, Fulova M, Spalekova M, Spalek P (2018) Epidemiology of myasthenia gravis in Slovakia in the years 1977–2015. Neuroepidemiology 50:153–159. https://doi.org/10.1159/000487886 CrossRefPubMed

10.

Popperud TH, Boldingh MI, Brunborg C et al (2017) Juvenile myasthenia gravis in Norway: a nationwide epidemiological study. Eur J Paediatr Neurol 21:312–317. https://doi.org/10.1016/j.ejpn.2016.09.001 CrossRefPubMed

11.

Monaco S, Turri E, Zanusso G, Maistrello B (2004) Treatment of inflammatory and paraproteinemic neuropathies. Curr Drug Targets Immune Endocr Metabol Disord 4:141–148CrossRef

12.

Naing NN (2000) Easy way to learn standardization: direct and indirect methods. Malays J Med Sci 7:10–15PubMedPubMedCentral

13.

Keyfitz N (1966) Finite approximations in demography. Popul Stud (Camb) 19:281–295. https://doi.org/10.1080/00324728.1966.10406017 CrossRef

14.

Vogrig A, Joubert B, André-Obadia N et al (2019) Seizure specificities in patients with antibody-mediated autoimmune encephalitis. Epilepsia 60:1508–1525. https://doi.org/10.1111/epi.16282 CrossRefPubMed

15.

Kanikannan MA, Sirisha Y, Uppin MS et al (2015) Incidence and spectrum of paraneoplastic neurological syndromes: single center study. J Neurooncol 125:197–206. https://doi.org/10.1007/s11060-015-1898-7 CrossRefPubMed

16.

Chan AM, Baehring JM (2019) Paraneoplastic neurological syndromes: a single institution 10-year case series. J Neurooncol 141:431–439. https://doi.org/10.1007/s11060-018-03053-3 CrossRefPubMed

17.

Hayat MJ, Howlader N, Reichman ME, Edwards BK (2007) Cancer statistics, trends, and multiple primary cancer analyses from the Surveillance, Epidemiology, and End Results (SEER) Program. Oncologist 12:20–37. https://doi.org/10.1634/theoncologist.12-1-20 CrossRefPubMed

18.

Ducray F, Graus F, Vigliani MC et al (2010) Delayed onset of a second paraneoplastic neurological syndrome in eight patients. J Neurol Neurosurg Psychiatry 81:937–939. https://doi.org/10.1136/jnnp.2009.190199 CrossRefPubMed

19.

Dubey D, Pittock SJ, Kelly CR et al (2018) Autoimmune encephalitis epidemiology and a comparison to infectious encephalitis. Ann Neurol 83:166–177. https://doi.org/10.1002/ana.25131 CrossRefPubMedPubMedCentral

20.

Talbott EO, Malek AM, Lacomis D (2016) The epidemiology of amyotrophic lateral sclerosis. Handb Clin Neurol 138:225–238. https://doi.org/10.1016/B978-0-12-802973-2.00013-6 CrossRefPubMed

21.

Zarei S, Carr K, Reiley L et al (2015) A comprehensive review of amyotrophic lateral sclerosis. Surg Neurol Int. https://doi.org/10.4103/2152-7806.169561 CrossRefPubMedPubMedCentral

22.

Giometto B, Grisold W, Vitaliani R et al (2010) Paraneoplastic neurologic syndrome in the PNS Euronetwork database: a European study from 20 centers. Arch Neurol 67:330–335. https://doi.org/10.1001/archneurol.2009.341 CrossRefPubMed

23.

Vogrig A, Ferrari S, Tinazzi M et al (2015) Anti-Ma-associated encephalomyeloradiculopathy in a patient with pleural mesothelioma. J Neurol Sci 350:105–106. https://doi.org/10.1016/j.jns.2015.01.028 CrossRefPubMed

24.

Dalmau J (2004) Clinical analysis of anti-Ma2-associated encephalitis. Brain 127:1831–1844. https://doi.org/10.1093/brain/awh203 CrossRefPubMed

25.

Vogrig A, Joubert B, Maureille A et al (2018) Motor neuron involvement in anti-Ma2-associated paraneoplastic neurological syndrome. J Neurol 266:398–410. https://doi.org/10.1007/s00415-018-9143-x CrossRefPubMed

26.

Vogrig A, Fouret M, Joubert B et al (2019) Increased frequency of anti-Ma2 encephalitis associated with immune checkpoint inhibitors. Neurol Neuroimmunol Neuroinflamm 6:e604. https://doi.org/10.1212/NXI.0000000000000604 CrossRefPubMedPubMedCentral

27.

Maureille A, Fenouil T, Joubert B et al (2018) Isolated seizures are a common early feature of paraneoplastic anti-GABA_B receptor encephalitis. J Neurol. https://doi.org/10.1007/s00415-018-9132-0 CrossRefPubMed

28.

Vogrig A, Pauletto G, Belgrado E et al (2018) Effect of thymectomy on refractory autoimmune status epilepticus. J Neuroimmunol 317:90–94. https://doi.org/10.1016/j.jneuroim.2018.01.009 CrossRefPubMed

29.

Camdessanché J-P, Jousserand G, Ferraud K et al (2009) The pattern and diagnostic criteria of sensory neuronopathy: a case-control study. Brain 132:1723–1733. https://doi.org/10.1093/brain/awp136 CrossRefPubMedPubMedCentral

30.

Shams’ili S, Grefkens J, de Leeuw B et al (2003) Paraneoplastic cerebellar degeneration associated with antineuronal antibodies: analysis of 50 patients. Brain 126:1409–1418CrossRef

31.

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

32.

Ventura L, Giorgi D, Giordano L et al (2015) Mammographic breast cancer screening in Italy: 2011–2012 survey. Epidemiol Prev 39:21–29PubMed

33.

Vogrig A, Zanoni T, Moretto G (2016) Nystagmus and lower extremity hyperalgesia after colectomy. JAMA 316:1488–1489. https://doi.org/10.1001/jama.2016.13658 CrossRefPubMed

34.

Do L-D, Chanson E, Desestret V et al (2017) Characteristics in limbic encephalitis with anti-adenylate kinase 5 autoantibodies. Neurology 88:514–524. https://doi.org/10.1212/WNL.0000000000003586 CrossRefPubMed

35.

Spatola M, Petit-Pedrol M, Simabukuro MM et al (2017) Investigations in GABA_A receptor antibody-associated encephalitis. Neurology 88:1012–1020. https://doi.org/10.1212/WNL.0000000000003713 CrossRefPubMedPubMedCentral

36.

Gaig C, Graus F, Compta Y et al (2017) Clinical manifestations of the anti-IgLON5 disease. Neurology 88:1736–1743. https://doi.org/10.1212/WNL.0000000000003887 CrossRefPubMedPubMedCentral

37.

Hara M, Ariño H, Petit-Pedrol M et al (2017) DPPX antibody–associated encephalitis: main syndrome and antibody effects. Neurology 88:1340–1348. https://doi.org/10.1212/WNL.0000000000003796 CrossRefPubMedPubMedCentral

38.

Mandel-Brehm C, Dubey D, Kryzer TJ et al (2019) Kelch-like protein 11 antibodies in seminoma-associated paraneoplastic encephalitis. N Engl J Med 381:47–54. https://doi.org/10.1056/NEJMoa1816721 CrossRefPubMedPubMedCentral

Titel: Epidemiology of paraneoplastic neurological syndromes: a population-based study
Publikationsdatum: 24.09.2019
Erschienen in: Journal of Neurology / Ausgabe 1/2020
Print ISSN: 0340-5354
Elektronische ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-019-09544-1

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Epidemiology of paraneoplastic neurological syndromes: a population-based study

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Results

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