Research Article

Epidemiology of myasthenia gravis in Northern Portugal: Frequency estimates and clinical epidemiological distribution of cases

Ernestina Santos MD

Neurology Department, Hospital Santo Antonio, Centro Hospitalar Porto, Porto, Portugal

Unit for Multidisciplinary Research in Biomedicine, Instituto de Ciencias Biomedicas de Abel Salazar, Universidade do Porto, Porto, Portugal

Search for more papers by this author

Ester Coutinho MD,

Ester Coutinho MD

Nuffield Department of Clinical Neurosciences, Oxford University Hospitals, University of Oxford, UK

Search for more papers by this author

Isabel Moreira MD,

Isabel Moreira MD

Neurology Department, Hospital Santo Antonio, Centro Hospitalar Porto, Porto, Portugal

Search for more papers by this author

Ana Martins Silva PhD,

Ana Martins Silva PhD

Neurology Department, Hospital Santo Antonio, Centro Hospitalar Porto, Porto, Portugal

Unit for Multidisciplinary Research in Biomedicine, Instituto de Ciencias Biomedicas de Abel Salazar, Universidade do Porto, Porto, Portugal

Search for more papers by this author

Dina Lopes MSc,

Dina Lopes MSc

Centro Hospitalar do Porto, Porto, Portugal

Search for more papers by this author

Henrique Costa MD,

Henrique Costa MD

Neurology Department, Hospital Sao Joao, Porto, Portugal

Search for more papers by this author

Fernando Silveira MD,

Fernando Silveira MD

Neurology Department, Hospital Sao Joao, Porto, Portugal

Search for more papers by this author

Goreti Nadais MD,

Goreti Nadais MD

Neurology Department, Hospital Sao Joao, Porto, Portugal

Search for more papers by this author

Hugo Morais MD,

Hugo Morais MD

Neurology Department, Centro Hospitalar de Vila Nova de Gaia e Espinho, Gaia, Portugal

Search for more papers by this author

João Martins MD,

João Martins MD

Neurology Department, Hospital Pedro Hispano, Matosinhos, Portugal

Search for more papers by this author

Maria Ceu Branco MD,

Maria Ceu Branco MD

Neurology Department, Hospital Pedro Hispano, Matosinhos, Portugal

Neurology Department, Hospital de Sao Pedro, Centro Hospitalar do Trás-os-Montes e Alto Douro, Vila Real, Portugal

Search for more papers by this author

Andreia Veiga MD,

Andreia Veiga MD

Neurology Department, Hospital de Sao Pedro, Centro Hospitalar do Trás-os-Montes e Alto Douro, Vila Real, Portugal

Search for more papers by this author

Rosa Santos Silva MD,

Rosa Santos Silva MD

Neurology Department, Centro Hospitalar do Alto Minho, Viana do Castelo, Portugal

Search for more papers by this author

Augusto Ferreira MD,

Augusto Ferreira MD

Neurology Department, Centro Hospitalar Entre Douro e Vouga, Feira, Portugal

Search for more papers by this author

Filipa Sousa MD,

Filipa Sousa MD

Neurology Department, Hospital de Braga, Braga, Portugal

Search for more papers by this author

Marta Freijo MD,

Marta Freijo MD

Neurology Department, Centro Hospitalar do Nordeste, Mirandela, Portugal

Search for more papers by this author

Ilda Matos MD,

Ilda Matos MD

Neurology Department, Centro Hospitalar do Nordeste, Mirandela, Portugal

Search for more papers by this author

Rui André MD,

Rui André MD

Neurology Department, Hospital de São Teotonio, Viseu, Portugal

Search for more papers by this author

Luís Negrão MD,

Luís Negrão MD

Neurology Department, Centro Hospitalar Universitario de Coimbra, Coimbra, Portugal

Search for more papers by this author

Carla Fraga MD,

Carla Fraga MD

Neurology Department, Centro Hospitalar do Vale do Sousa, Penafiel, Portugal

Search for more papers by this author

Manuela Santos MD,

Manuela Santos MD

Neuropediatrics Department, Centro Materno Infantil Norte, Centro Hospitalar Porto, Porto, Portugal

Search for more papers by this author

Mafalda Sampaio MD,

Mafalda Sampaio MD

Neuropediatrics Department, Hospital de Sao Joao, Porto, Portugal

Search for more papers by this author

Carlos Lopes PhD,

Carlos Lopes PhD

Instituto de Ciencias Biomedicas Abel Salazar, Universidade do Porto, Porto, Portugal

Search for more papers by this author

Maria Isabel Leite DPhil,

Corresponding Author

Maria Isabel Leite DPhil

Nuffield Department of Clinical Neurosciences, Oxford University Hospitals, University of Oxford, UK

Correspondence to: M.I. Leite; e-mail: [email protected]Search for more papers by this author

Guilherme Gonçalves PhD,

Guilherme Gonçalves PhD

Unit for Multidisciplinary Research in Biomedicine, Instituto de Ciencias Biomedicas de Abel Salazar, Universidade do Porto, Porto, Portugal

Search for more papers by this author

Ernestina Santos MD,

Ernestina Santos MD

Neurology Department, Hospital Santo Antonio, Centro Hospitalar Porto, Porto, Portugal

Unit for Multidisciplinary Research in Biomedicine, Instituto de Ciencias Biomedicas de Abel Salazar, Universidade do Porto, Porto, Portugal

Search for more papers by this author

Ester Coutinho MD,

Ester Coutinho MD

Nuffield Department of Clinical Neurosciences, Oxford University Hospitals, University of Oxford, UK

Search for more papers by this author

Isabel Moreira MD,

Isabel Moreira MD

Neurology Department, Hospital Santo Antonio, Centro Hospitalar Porto, Porto, Portugal

Search for more papers by this author

Ana Martins Silva PhD,

Ana Martins Silva PhD

Neurology Department, Hospital Santo Antonio, Centro Hospitalar Porto, Porto, Portugal

Unit for Multidisciplinary Research in Biomedicine, Instituto de Ciencias Biomedicas de Abel Salazar, Universidade do Porto, Porto, Portugal

Search for more papers by this author

Dina Lopes MSc,

Dina Lopes MSc

Centro Hospitalar do Porto, Porto, Portugal

Search for more papers by this author

Henrique Costa MD,

Henrique Costa MD

Neurology Department, Hospital Sao Joao, Porto, Portugal

Search for more papers by this author

Fernando Silveira MD,

Fernando Silveira MD

Neurology Department, Hospital Sao Joao, Porto, Portugal

Search for more papers by this author

Goreti Nadais MD,

Goreti Nadais MD

Neurology Department, Hospital Sao Joao, Porto, Portugal

Search for more papers by this author

Hugo Morais MD,

Hugo Morais MD

Neurology Department, Centro Hospitalar de Vila Nova de Gaia e Espinho, Gaia, Portugal

Search for more papers by this author

João Martins MD,

João Martins MD

Neurology Department, Hospital Pedro Hispano, Matosinhos, Portugal

Search for more papers by this author

Maria Ceu Branco MD,

Maria Ceu Branco MD

Neurology Department, Hospital Pedro Hispano, Matosinhos, Portugal

Neurology Department, Hospital de Sao Pedro, Centro Hospitalar do Trás-os-Montes e Alto Douro, Vila Real, Portugal

Search for more papers by this author

Andreia Veiga MD,

Andreia Veiga MD

Neurology Department, Hospital de Sao Pedro, Centro Hospitalar do Trás-os-Montes e Alto Douro, Vila Real, Portugal

Search for more papers by this author

Rosa Santos Silva MD,

Rosa Santos Silva MD

Neurology Department, Centro Hospitalar do Alto Minho, Viana do Castelo, Portugal

Search for more papers by this author

Augusto Ferreira MD,

Augusto Ferreira MD

Neurology Department, Centro Hospitalar Entre Douro e Vouga, Feira, Portugal

Search for more papers by this author

Filipa Sousa MD,

Filipa Sousa MD

Neurology Department, Hospital de Braga, Braga, Portugal

Search for more papers by this author

Marta Freijo MD,

Marta Freijo MD

Neurology Department, Centro Hospitalar do Nordeste, Mirandela, Portugal

Search for more papers by this author

Ilda Matos MD,

Ilda Matos MD

Neurology Department, Centro Hospitalar do Nordeste, Mirandela, Portugal

Search for more papers by this author

Rui André MD,

Rui André MD

Neurology Department, Hospital de São Teotonio, Viseu, Portugal

Search for more papers by this author

Luís Negrão MD,

Luís Negrão MD

Neurology Department, Centro Hospitalar Universitario de Coimbra, Coimbra, Portugal

Search for more papers by this author

Carla Fraga MD,

Carla Fraga MD

Neurology Department, Centro Hospitalar do Vale do Sousa, Penafiel, Portugal

Search for more papers by this author

Manuela Santos MD,

Manuela Santos MD

Neuropediatrics Department, Centro Materno Infantil Norte, Centro Hospitalar Porto, Porto, Portugal

Search for more papers by this author

Mafalda Sampaio MD,

Mafalda Sampaio MD

Neuropediatrics Department, Hospital de Sao Joao, Porto, Portugal

Search for more papers by this author

Carlos Lopes PhD,

Carlos Lopes PhD

Instituto de Ciencias Biomedicas Abel Salazar, Universidade do Porto, Porto, Portugal

Search for more papers by this author

Maria Isabel Leite DPhil,

Corresponding Author

Maria Isabel Leite DPhil

Nuffield Department of Clinical Neurosciences, Oxford University Hospitals, University of Oxford, UK

Correspondence to: M.I. Leite; e-mail: [email protected]Search for more papers by this author

Guilherme Gonçalves PhD,

Guilherme Gonçalves PhD

Unit for Multidisciplinary Research in Biomedicine, Instituto de Ciencias Biomedicas de Abel Salazar, Universidade do Porto, Porto, Portugal

Search for more papers by this author

First published: 06 February 2016

https://doi.org/10.1002/mus.25068

Citations: 34

Disclosure: M.I.L. is supported by the NHS National Specialised Commissioning Group for Neuromyelitis Optica, UK, and by the NIHR Oxford Biomedical Research Centre, and has received travel grant and speaking honoraria from Biogen Idec and a travel grant from Novartis and Genzyme.

Share a link

Email
Wechat
Bluesky

ABSTRACT

Introduction: In this study we estimated the prevalence, incidence, and mortality of myasthenia gravis (MG) in northern Portugal and characterized the clinical features of the patients identified. Methods: We used 2 data sources: clinical records from the hospitals and pyridostigmine prescription registers. Results: On December 31, 2013, we estimated a point prevalence of 111.7 patients per million population. The highest prevalence was observed in the group >65 years of age, especially in men (288.1 per million). During 2013, we estimated an incidence rate of 6.3 per million per year. Among women, the incidence rate was highest in the 15–49-year age group; in men, incidence increased with age up to 22.1 per million in those >65 years old. The MG-related mortality rate was 0.5 per million. Conclusions: These figures are in keeping with similar studies and emphasize the importance of diagnosis and management of MG in elderly populations. Muscle Nerve 54: 413–421, 2016

REFERENCES

1Hughes T. The early history of myasthenia gravis. Neuromuscul Disord 2005; 15: 878–886.
10.1016/j.nmd.2005.08.007
PubMed Web of Science® Google Scholar
2Vincent A. Unravelling the pathogenesis of myasthenia gravis. Nat Rev Immunol 2002; 10: 797–804.
10.1038/nri916
CAS Web of Science® Google Scholar
3Compston DA, Vincent A, Newsom-Davis J, Batchelor JR. Clinical, pathological, HLA antigen and immunological evidence for disease heterogeneity in myasthenia gravis. Brain 1980; 103: 579–601.
10.1093/brain/103.3.579
CAS PubMed Web of Science® Google Scholar
4Aarli JA. Late-onset myasthenia gravis: a changing scene. Arch Neurol 1999; 56: 25–27.
10.1001/archneur.56.1.25
CAS PubMed Web of Science® Google Scholar
5Heldal AT, Eide GE, Gilhus NE, Romi F. Geographical distribution of a seropositive myasthenia gravis population. Muscle Nerve 2012; 45: 815–819.
10.1002/mus.23271
CAS PubMed Web of Science® Google Scholar
6Aragonès JM, Altimiras J, Roura P, Alonso F, Bufill E, Munmany A, et al. Prevalence of myasthenia gravis in the Catalan county of Osona. Neurologia 2014.
10.1016/j.nrl.2014.09.007
PubMed Google Scholar
7Aragonès JM, Roura-Poch P, Hernández-Ocampo EM, Alonso F, Pont-Lluelles M, Xandri I, et al. Myasthenia gravis: a disease of the very old. J Am Geriatr Soc 2014; 62: 196–197.
10.1111/jgs.12629
PubMed Web of Science® Google Scholar
8Pedersen EG, Hallas J, Hansen K, Jensen PE, Gaist D. Late-onset myasthenia not on the increase: a nationwide register study in Denmark, 1996–2009. Eur J Neurol 2013; 20: 309–314.
10.1111/j.1468-1331.2012.03850.x
CAS PubMed Web of Science® Google Scholar
9Pallaver F, Riviera AP, Piffer S, Ricciardi R, Roni R, Orrico D, et al. Change in myasthenia gravis epidemiology in Trento, Italy, after twenty years. Neuroepidemiology 2011; 36: 282–287.
10.1159/000328863
PubMed Web of Science® Google Scholar
10Montomoli C, Citterio A, Piccolo G, Cioccale R, Ferretti VV, Fratti C, et al. Epidemiology and geographical variation of myasthenia gravis in the province of Pavia, Italy. Neuroepidemiology 2012; 38: 100–105.
10.1159/000336002
PubMed Web of Science® Google Scholar
11Andersen JB, Heldal AT, Engeland A, Gilhus NE. Myasthenia gravis epidemiology in a national cohort; combining multiple disease registries. Acta Neurol Scand Suppl 2014;(198): 26–31.
10.1111/ane.12233
PubMed Web of Science® Google Scholar
12Heldal AT, Owe JF, Gilhus NE, Romi F. Seropositive myasthenia gravis: a nationwide epidemiologic study. Neurology 2009; 73: 150–151.
10.1212/WNL.0b013e3181ad53c2
PubMed Web of Science® Google Scholar
13Somnier FE. Increasing incidence of late-onset anti-AChR antibody-seropositive myasthenia gravis. Neurology 2005; 27;65: 928–930.
10.1212/01.wnl.0000176067.32186.a3
CAS PubMed Web of Science® Google Scholar
14Poulas K, Tsibri E, Kokla A, Papanastasiou D, Tsouloufis T, Marinou M, et al. Epidemiology of seropositive myasthenia gravis in Greece. J Neurol Neurosurg Psychiatry 2001; 71: 352–356.
10.1136/jnnp.71.3.352
CAS PubMed Web of Science® Google Scholar
15Guidetti D, Sabadini R, Bondavalli M, Cavalletti S, Lodesani M, Mantegazza R, et al. Epidemiological study of myasthenia gravis in the province of Reggio Emilia, Italy. Eur J Epidemiol 1998; 14: 381–387.
10.1023/A:1007449221638
CAS PubMed Web of Science® Google Scholar
16Phillips LH 2nd, Torner JC. Epidemiologic evidence for a changing natural history of myasthenia gravis. Neurology 1996; 47: 1233–1238.
10.1212/WNL.47.5.1233
PubMed Web of Science® Google Scholar
17Carr AS, Cardwell CR, McCarron PO, McConville J. A systematic review of population based epidemiological studies in myasthenia gravis. BMC Neurol 2010; 18: 1–9.
Google Scholar
18Robertson NP, Deans J, Compston DA. Myasthenia gravis: a population based epidemiological study in Cambridgeshire, England. J Neurol Neurosurg Psychiatry 1998; 65: 492–496.
10.1136/jnnp.65.4.492
CAS PubMed Web of Science® Google Scholar
19Vincent A, Clover L, Buckley C, Grimley Evans J, Rothwell PM; UK Myasthenia Gravis Survey. Evidence of underdiagnosis of myasthenia gravis in older people. J Neurol Neurosurg Psychiatry 2003; 74: 1105–1108.
10.1136/jnnp.74.8.1105
CAS PubMed Web of Science® Google Scholar
20Aragonès JM, Bolíbar I, Bonfill X, Bufill E, Mummany A, Alonso F, et al. Myasthenia gravis: a higher than expected incidence in the elderly. Neurology 2003; 60: 1024–1026.
10.1212/01.WNL.0000050461.05432.C5
CAS PubMed Web of Science® Google Scholar
21Alkhawajah NM, Oger J. Late-onset myasthenia gravis: a review when incidence in older adults keeps increasing. Muscle Nerve 2013; 48: 705–710.
10.1002/mus.23964
PubMed Web of Science® Google Scholar
22Vincent A, Leite MI, Farrugia ME, Jacob S, Viegas S, Shiraishi H, et al. Myasthenia gravis seronegative for acetylcholine receptor antibodies. Ann NY Acad Sci 2008; 1132: 84–92.
10.1196/annals.1405.020
CAS PubMed Web of Science® Google Scholar
23Guptill JT, Sanders DB. Update on muscle-specific tyrosine kinase antibody positive myasthenia gravis. Curr Opin Neurol 2010; 23: 530–535.
10.1097/WCO.0b013e32833c0982
CAS PubMed Web of Science® Google Scholar
24Tsiamalos P, Kordas G, Kokla A, Poulas K, Tzartos SJ. Epidemiological and immunological profile of muscle-specific kinase myasthenia gravis in Greece. Eur J Neurol 2009; 16: 925–930.
10.1111/j.1468-1331.2009.02624.x
CAS PubMed Web of Science® Google Scholar
25Oopik M, Kaasik AE, Jakobsen J. A population based epidemiological study on myasthenia gravis in Estonia. J Neurol Neurosurg Psychiatry 2003; 74: 1638–1643.
10.1136/jnnp.74.12.1638
CAS PubMed Web of Science® Google Scholar
26Cetin H, Fülöp G, Zach H, Auff E, Zimprich F. Epidemiology of myasthenia gravis in Austria: rising prevalence in an ageing society. Wien Klin Wochenschr 2012; 124: 763–768.
10.1007/s00508-012-0258-2
PubMed Web of Science® Google Scholar
27Pedersen EG, Hallas J, Hnasen K, Jensen PEH, Gaist D. Identifying patients with myastenia for epidemiological research of automated research linkage of automated registers. Neuroepidemiology 2011; 27: 120–128.
10.1159/000331481
Web of Science® Google Scholar
28Gattellari M, Goumas C, Worthington JM. A national epidemiological study of myasthenia gravis in Australia. Eur J Neurol 2012; 19: 1413–1420.
10.1111/j.1468-1331.2012.03698.x
CAS PubMed Web of Science® Google Scholar
29 Censos—Resultados definitivos. Região Norte—2011. Madrid: Instituto Nacional de Estatística, I.P, Ano de Edição 2012. p. 1–390.
Google Scholar
30 Estatísticas Demográficas 2013. Madrid: Instituto Nacional de Estatística, Ano de Edição 2014. p. 1–155.
Google Scholar
31 Emilia-Romagna Study Group on Clinical and Epidemiological Problems in Neurology. Incidence of myasthenia gravis in the Emilia-Romagna region: a prospective multicenter study. Emilia-Romagna Study Group on Clinical and Epidemiological Problems in Neurology. Neurology 1998; 51: 255–258.
10.1212/WNL.51.1.255
PubMed Web of Science® Google Scholar
32Jaretzki A 3rd, Barohn RJ, Ernstoff RM, Kaminski HJ, Keesey JC, Penn AS, et al. Myasthenia gravis: recommendations for clinical research standards. Task Force of the Medical Scientific Advisory Board of the Myasthenia Gravis Foundation of America. Ann Thorac Surg 2000; 70: 327–334.
10.1016/S0003-4975(00)01595-2
PubMed Web of Science® Google Scholar
33Leite MI, Jacob S, Viegas S, Cossins J, Clover L, Morgan BP, et al. IgG1 antibodies to acetylcholine receptors in ‘seronegative’ myasthenia gravis. Brain 2008; 131: 1940–1952.
10.1093/brain/awn092
PubMed Web of Science® Google Scholar
34Leite MI, Waters P, Vincent A. Diagnostic use of autoantibodies in myasthenia gravis. Autoimmunity 2010; 43: 371–379.
10.3109/08916930903541208
PubMed Web of Science® Google Scholar
35McGrogan A, Sneddon S, de Vries CS. The incidence of myasthenia gravis: a systematic review. Neuroepidemiology 2010; 34: 171–183.
10.1159/000279334
PubMed Web of Science® Google Scholar
36Andersen JB, Engeland A, Owe JF, Gilhus NE. Myasthenia gravis requiring pyridostigmine treatment in a national population cohort. Eur J Neurol 2010; 17: 1445–1450.
10.1111/j.1468-1331.2010.03089.x
CAS PubMed Web of Science® Google Scholar
37Pakzad Z, Aziz T, Oger J. Increasing incidence of myasthenia gravis among elderly in British Columbia, Canada. Neurology 2011 26;76: 1526–1528.
10.1212/WNL.0b013e318217e735
Google Scholar
38Murai H, Yamashita N, Watanabe M, Nomura Y, Motomura M, Yoshikawa H. Characteristics of myasthenia gravis according to onset-age: Japanese nationwide survey. J Neurol Sci 2011; 305: 97–102.
10.1016/j.jns.2011.03.004
PubMed Web of Science® Google Scholar
39Kuks J. In: H Kaminski, editor. Clinical presentation and epidemiology in myasthenia gravis and related disorders, 2nd ed. New York: Humana Press; 2009. p. 90.
Google Scholar
40Evoli A. Clinical aspects of neuromuscular transmission disorders. Acta Neurol Scand Suppl 2006; 183: 8–11.
10.1111/j.1600-0404.2006.00606.x
PubMed Web of Science® Google Scholar

Citing Literature

Volume54, Issue3

September 2016

Pages 413-421

Epidemiology of myasthenia gravis in Northern Portugal: Frequency estimates and clinical epidemiological distribution of cases

ABSTRACT

REFERENCES

Citing Literature

References

Information

About Wiley Online Library

Help & Support

Opportunities

Connect with Wiley

Epidemiology of myasthenia gravis in Northern Portugal: Frequency estimates and clinical epidemiological distribution of cases

ABSTRACT

REFERENCES

Citing Literature

References

Related

Information