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Clinical Features of Myasthenia Gravis Patients’: The assessment of 138 Patients

Year 2021, , 694 - 698, 17.09.2021
https://doi.org/10.16899/jcm.946728

Abstract

Aim: The aim of this study was to evaluate the demographic characteristics, clinical presentation, antibody, and electrophysiological characteristics of patients with the diagnosis of Myasthenia Gravis (MG).
Materials and Methods: The demographic characteristics, clinical presentation, antibody, electrophysiological and radiological (thoracic tomography) characteristics of the patients who were followed in the Neuromuscular and Muscular Diseases Polyclinic of the hospital between 2014-2019 were analyzed.
Results: The mean age of the patients was 53.8±16.67 years. The mean disease duration was 4.8±5.05 years. Ptosis was the most common complaint in 79% (n=109) of our patients. The number of patients followed as pure ocular MG was 61.6% (n=85). There were 26 patients who presented with ptosis or diplopia at the first presentation and follow-up generalized MG with bulbar findings and weakness. Acetylcholine receptor antibody (AntiAChRAb) positivity was found in 78.3% (n=108) of the patients and thymus pathology was detected in thorax tomography in 37.7% (n=52).
Conclusions: The initial complaint provides little insight into the course of the disease. The important factor is clinical follow-up. Although MG treatment is known, the pathophysiology of MG remains to be elucidated, and other autoimmune conditions cause changes in treatment choice. Clarifying the pathophysiology of MG will enable the development of more effective diagnostic and treatment methods.

Supporting Institution

Yok

Project Number

Yok

Thanks

This paper was not funded by anyone and the author declared that it has received no financial support. The author declares no conflict of interest related to this work.

References

  • 1. Matthew N. Meriggioli. Myasthenia Gravis: Immunopathogenesis, Diagnosis, And Management. Contınuum (Myasthenic Disorders and ALS) 2009;15(1):35-62.
  • 2. Phillips LH. The epidemiology of myasthenia gravis. Ann N Y Acad Sci 2003;998:407–412.
  • 3. Pakzad Z, Aziz T, Oger J. Increasing incidence of myasthenia gravis among elderly in British Columbia, Canada. Neurology. 2011;76(17):1526-8.
  • 4. Binks S, Vincent A, Palace J. Myasthenia gravis: a clinical-immunological update. J Neurol 2016;263(4):826j834.
  • 5. Carr AS, Cardwell CR, McCarron PO, McConville J. A systematic review of population based epidemiological studies in Myasthenia Gravis. BMC Neurol 2010;10:46.
  • 6. Renton AE, Pliner HA, Provenzano C, et al. A genome-wide association study of myasthenia gravis. JAMA Neurol 2015;72(4):396Y404.
  • 7. Drachman DB, Adams RN, Stanley EF, Pestronk A. Mechanisms of acetylcholine receptor loss in myasthenia gravis. J Neurol Neurosurg Psychiatry 1980;43(7):601-610.
  • 8. Nicolle MW. Myasthenia gravis. Neurologist 2002;8(1):2-21.
  • 9. Koneczny I, Cossins J, Vincent A. The role of muscle-specific tyrosine kinase (MuSK) and mystery of MuSK myasthenia gravis. J Anat 2014;224(1):29-35.
  • 10. Berrih-Aknin S. Cortactin: a new target in autoimmune myositis and Myasthenia Gravis. Autoimmun Rev 2014;13(10):1001-1002.
  • 11. Berrih-Aknin S, Le Panse R. Myasthenia gravis: a comprehensive review of immune dysregulation and etiological mechanisms. J Autoimmun 2014;52:90-100.
  • 12. Binks S, Vincent A, Palace J. Myasthenia gravis: a clinical-immunological update. J Neurol 2016;263(4):826-834.
  • 13. Hohlfeld R, Wekerle H. The thymus in myasthenia gravis. Neurol Clin 1994;12(2):331-342.
  • 14. Nacu A, Andersen JB, Lisnic V, Owe JF, Gilhus NE. Complicating autoimmune diseases in myasthenia gravis: a review. Autoimmunity 2015;48(6):362-368.
  • 15. Gilhus NE, Nacu A, Andersen JB, Owe JF. Myasthenia gravis and risks for comorbidity. Eur J Neurol 2015;22(1):17-23.
  • 16. Jaretski A 3rd, Barohn RJ, Ernstoff RM, et al. Myasthenia gravis: recommendations for clinical research standards. Task force of the Medical Scientific Advisory Board of the Myasthenia Gravis Foundation of America. Neurology 2000;55(1):16-23.
  • 17. Wang L, Zhang Y, He M. Clinical predictors for the prognosis of myasthenia gravis. BMC Neurol 2017;17 (1), 77
  • 18. Maarika Liik M, Punga AR. Repetitive Nerve Stimulation Often Fails to Detect Abnormal Decrement in Acute Severe Generalized Myasthenia Gravis. Clin Neurophysiol 2016;127 (11),3480-3484.
  • 19. Baysal Aİ, Kuruoğlu HR, Odabaşı Z. Olgu Çalışmalarıyla Klinik Elektromyografi İlkeleri, Ankara: Güneş Tıp Kitabevleri, 2012.
  • 20. Karaahmet OZ, Bal A, Dulgeroglu D, Bahceci HK, Cakcı A. Methotrexate treatment in myasthenia gravis. J Clin Neuromuscul Dis. 2014;16(2):106-107.
  • 21. Sawa N, Kataoka H, Eura N, Ueno S. Dropped head with positive intravenous edrophonium, progressing to myasthenia gravis. BMJ Case Rep. 2013;2013:bcr2012007616. doi: 10.1136/bcr-2012-007616.
  • 22. D'Amelio M, Di Benedetto N, Ragonese P, et al. Dropped head as an unusual presenting sign of myasthenia gravis. Neurol Sci. 2007;28(2):104-106.

Myastenia Gravis Hastalarının Klinik Özellikleri: 138 Hastanın Değerlendirilmesi

Year 2021, , 694 - 698, 17.09.2021
https://doi.org/10.16899/jcm.946728

Abstract

Amaç: Bu çalışmanın amacı, Myastenia Gravis (MG) tanısı ile izlenen hastaların demografik özellikleri, ilk başvuru şikayeti ile klinik seyir, antikor ve elektrofizyolojik özelliklerini değerlendirmektir.
Gereç ve Yöntem: 2014-2019 yılları arasında, hastanemiz Nöromusküler ve Kas Hastalıkları Polikliniği’nde takip edilen yüz otuz sekiz MG hastasının demografik özellikleri, ilk başvuru şikayeti ile klinik seyri, antikor, elektrofizyolojik ve radyolojik (toraks tomografi) özellikleri incelendi.
Bulgular: Hastaların yaş ortalaması 53.8±16.67 idi. Ortalama hastalık süresi 4.8±5.05 yıl idi. Hastalarımızın en sık, ilk başvuru şikayeti %79 (n=109)’unda ptozis idi. Saf oküler MG olarak takip edilen hasta sayısı %61.6 (n=85) kişiydi. İlk başvurusunda ptozis veya diplopi şikayetiyle gelip, daha sonrasında bulber bulgular ve güçsüzlük eklenerek jeneralize MG düşünülen 26 hasta bulunuyordu. Hastaların %78.3 (n=108)’ünde asetil kolin reseptör antikoru (AntiAChRAb) pozitifliği ve %37.7 (n=52)’sinde toraks tomografide timüs patolojisi bulundu.
Sonuç: İlk başvuru şikayeti hastalığın seyri hakkında çok az fikir vermektedir. Asıl önemli olan, klinik yakın takiptir. Her ne kadar MG tedavisi bilinse de MG’in patofizyolojisi halen tam olarak aydınlatılamamıştır ve diğer otoimmun durumlar tedavi seçiminde değişikliğe neden olmaktadır. MG patofizyolojisinin aydınlatılması, daha etkin tanı ve tedavi yöntemlerinin geliştirilmesini sağlayacaktır.

Project Number

Yok

References

  • 1. Matthew N. Meriggioli. Myasthenia Gravis: Immunopathogenesis, Diagnosis, And Management. Contınuum (Myasthenic Disorders and ALS) 2009;15(1):35-62.
  • 2. Phillips LH. The epidemiology of myasthenia gravis. Ann N Y Acad Sci 2003;998:407–412.
  • 3. Pakzad Z, Aziz T, Oger J. Increasing incidence of myasthenia gravis among elderly in British Columbia, Canada. Neurology. 2011;76(17):1526-8.
  • 4. Binks S, Vincent A, Palace J. Myasthenia gravis: a clinical-immunological update. J Neurol 2016;263(4):826j834.
  • 5. Carr AS, Cardwell CR, McCarron PO, McConville J. A systematic review of population based epidemiological studies in Myasthenia Gravis. BMC Neurol 2010;10:46.
  • 6. Renton AE, Pliner HA, Provenzano C, et al. A genome-wide association study of myasthenia gravis. JAMA Neurol 2015;72(4):396Y404.
  • 7. Drachman DB, Adams RN, Stanley EF, Pestronk A. Mechanisms of acetylcholine receptor loss in myasthenia gravis. J Neurol Neurosurg Psychiatry 1980;43(7):601-610.
  • 8. Nicolle MW. Myasthenia gravis. Neurologist 2002;8(1):2-21.
  • 9. Koneczny I, Cossins J, Vincent A. The role of muscle-specific tyrosine kinase (MuSK) and mystery of MuSK myasthenia gravis. J Anat 2014;224(1):29-35.
  • 10. Berrih-Aknin S. Cortactin: a new target in autoimmune myositis and Myasthenia Gravis. Autoimmun Rev 2014;13(10):1001-1002.
  • 11. Berrih-Aknin S, Le Panse R. Myasthenia gravis: a comprehensive review of immune dysregulation and etiological mechanisms. J Autoimmun 2014;52:90-100.
  • 12. Binks S, Vincent A, Palace J. Myasthenia gravis: a clinical-immunological update. J Neurol 2016;263(4):826-834.
  • 13. Hohlfeld R, Wekerle H. The thymus in myasthenia gravis. Neurol Clin 1994;12(2):331-342.
  • 14. Nacu A, Andersen JB, Lisnic V, Owe JF, Gilhus NE. Complicating autoimmune diseases in myasthenia gravis: a review. Autoimmunity 2015;48(6):362-368.
  • 15. Gilhus NE, Nacu A, Andersen JB, Owe JF. Myasthenia gravis and risks for comorbidity. Eur J Neurol 2015;22(1):17-23.
  • 16. Jaretski A 3rd, Barohn RJ, Ernstoff RM, et al. Myasthenia gravis: recommendations for clinical research standards. Task force of the Medical Scientific Advisory Board of the Myasthenia Gravis Foundation of America. Neurology 2000;55(1):16-23.
  • 17. Wang L, Zhang Y, He M. Clinical predictors for the prognosis of myasthenia gravis. BMC Neurol 2017;17 (1), 77
  • 18. Maarika Liik M, Punga AR. Repetitive Nerve Stimulation Often Fails to Detect Abnormal Decrement in Acute Severe Generalized Myasthenia Gravis. Clin Neurophysiol 2016;127 (11),3480-3484.
  • 19. Baysal Aİ, Kuruoğlu HR, Odabaşı Z. Olgu Çalışmalarıyla Klinik Elektromyografi İlkeleri, Ankara: Güneş Tıp Kitabevleri, 2012.
  • 20. Karaahmet OZ, Bal A, Dulgeroglu D, Bahceci HK, Cakcı A. Methotrexate treatment in myasthenia gravis. J Clin Neuromuscul Dis. 2014;16(2):106-107.
  • 21. Sawa N, Kataoka H, Eura N, Ueno S. Dropped head with positive intravenous edrophonium, progressing to myasthenia gravis. BMJ Case Rep. 2013;2013:bcr2012007616. doi: 10.1136/bcr-2012-007616.
  • 22. D'Amelio M, Di Benedetto N, Ragonese P, et al. Dropped head as an unusual presenting sign of myasthenia gravis. Neurol Sci. 2007;28(2):104-106.
There are 22 citations in total.

Details

Primary Language English
Subjects Health Care Administration
Journal Section Original Research
Authors

Mehlika Panpallı Ateş 0000-0002-9744-9255

Project Number Yok
Publication Date September 17, 2021
Acceptance Date June 29, 2021
Published in Issue Year 2021

Cite

AMA Panpallı Ateş M. Clinical Features of Myasthenia Gravis Patients’: The assessment of 138 Patients. J Contemp Med. September 2021;11(5):694-698. doi:10.16899/jcm.946728