Abstract
Myastenia gravis, iskelet kaslarında güçsüzlük ve yorgunlukla karakterize otoimmün hastalıktır. Burada sadece bulbar semptomlarla başvuran çocuk hasta sunulmuştur.Onbeş yaşında erkek hasta bir aydır yutkunmada zorluk, konuşmada pelteklik şikayeti ile başvurdu. Konuşmanın akşama doğru bozulduğunu, yorgunluğunun arttığını, yutkunmadaki zorluk nedeni ile katı gıdaları alamadığını sıvı gıda ile beslendiğini ifade etti. Bu şikayetlerine onbeş gündür göğüs ve omuz ağrısının eklendiğini belirtti. Hastanın fizik muayenesi normal idi. Yapılan elektromiyografisinde: ardışık sinir uyarım testinde anlamlı decrement yanıt elde edildi. Asetilkolin reseptör antikoru: 29.65mmol/L bulundu. Hasta intravenöz immünglobülin ve plazmaferez ile tedavi edildi. Hasta pridostigmin ve oral steroid ile taburcu edildi. Ancak izleminde ataklarının sık olmasından dolayı tedavisine azotiopürin eklendi. Sonuç olarak; myastenia gravis olgularının sadece bulbar semptomlarla başvurabileceği akılda tutulmalıdır.
References
- McGrogan A, Sneddon S, de Vries CS. The incidence of myasthenia gravis: A systematic literature review. Neuroepidemiology 2010; 34:171-83.
- Saltık S, Ergüven M, Turgut T, Demirbilek V, Özümüztoprak N, Doğu A. Çocukluk çağında miyasteni gravis olgu sunumu. Türk Pediatr Arş 2004;39:135-8.
- Arslan Y, Tokuçoğlu F, Kaya A, Ünlü GD, Zorlu Y. Geç tanı konjenital miyastenik sendrom. DEÜ Tıp Fakültesi Dergisi 2016;30:85-8.
- Téllez-Zenteno JF, Hernández-Ronquillo L, Salinas V, Estanol B, da Silva O. Myasthenia gravis and pregnancy: Clinical implications and neonatal outcome. BMC Musculoskelet Disord 2004;5:42.
- Chiang LM, Darras BT, Kang PB. Juvenile myasthenia gravis. Muscle Nerve 2009;39:423-31.
- Ünsal Y, Yiş U. Jüvenil miyasteni gravis: Üç olgu sunumu ve literatürün gözden geçirilmesi. J Clin Anal Med 2015;6:473-8.
- Ekmekci O, Karasoy H. Prepubertal juvenile myasthenia gravis; A case with early onset and bulbar symptoms. J Neurol Sci 2013;30:583-6.
- Sarnat H. Disorders of neuromuscular transmission and of motor neurons. In: Kliegman RM, Stanton BF, Schor NF, St. Geme JW, Behrman RE (eds). Nelson Textbook of Pediatrics. 19th ed. Philadelphia: W.B. Saunders Company, 2011:2132-8.
- Değerliyurt A, Şenbil N, Anlar B. Jüvenil (otoimmün ) miyasteni: Klinik özellikler ve tedavi sonuçları. Çocuk Sağ Hast Derg 2005;48:25-9.
- Tanrısever Ö, Sarıoğlu B, Kanık A, Arslan CN, Baydan F. Farklı bir klinik başlangıç gösteren miyastenia gravis olgusu. Tepecik Eğit Hast Derg 2014;24:59-62.
- Raksadawan N, Kankirawatana P, Balankura K, Prateepratana P, Sangruchi T, Atchaneeyasakul LO. Childhood onset myasthenia gravis. J Med. Assoc Thai 2002;85:769-7.
- Meriggioli MN, Sanders DB. Autoimmune myasthenia gravis: Emerging clinical and biological heterogeneity. Lancet Neurol 2009; 8:475-90.
- Ashraf VV, Taly AB, Veerendrakumar M, Rao S. Myasthenia gravis in children: A longitudinal study. Acta Neurol Scand 2006;114:19–23.
- Salman E, Yetişir F, Yürekli B, Bozkurt A, Kılıç M. Myastenik kriz yönetiminde intravenöz immunoglobulin tedavisi: Olgu sunumu. J Clin Anal Med 2013;4:352-4.
- Long RR. Myasthenia gravis in the intensive care unit. In: Irwin RS, Cerra FB, Rıppe JM (eds). Intensive Care Medicine. Philadelphia: Lippincott-Raven Publishing, 1998:2122-7.
- Yosunkaya A, Çelik J.B, Tuncer S, Topal A, Ökesli S. Myastenia gravis ve immunoadsorbsiyon plazmaferez. T Klin J Medl Sci 2002;22:578-82.
Abstract
Myasthenia gravis is an autoimmune disease that can lead to weakness and fatigue of skeletal muscles. Here we introduce a child presenting with bulbar symptoms only.A fifteen year-old boy presented to the hospital with difficulty swallowing and speaking, and fatigue especially at evenings for one month. He could only take liquids. His neurological examination was normal. The sequential nerve stimulation test results were decreased on electromyography examination. The acetylcholine receptor antibody level was high. We considered the diagnosis of bulbar Myasthenia Gravis, and treated the patient with intravenous immunoglobulin and plasmapheresis. He was discharged with oral steroids and prydostigmin. He had frequent attacks during the follow-up and azathiprine was added to his treatment. In conclusion, pediatric patients with myasthenia gravis can apply only with bulbar symptoms
Keywords
References
- McGrogan A, Sneddon S, de Vries CS. The incidence of myasthenia gravis: A systematic literature review. Neuroepidemiology 2010; 34:171-83.
- Saltık S, Ergüven M, Turgut T, Demirbilek V, Özümüztoprak N, Doğu A. Çocukluk çağında miyasteni gravis olgu sunumu. Türk Pediatr Arş 2004;39:135-8.
- Arslan Y, Tokuçoğlu F, Kaya A, Ünlü GD, Zorlu Y. Geç tanı konjenital miyastenik sendrom. DEÜ Tıp Fakültesi Dergisi 2016;30:85-8.
- Téllez-Zenteno JF, Hernández-Ronquillo L, Salinas V, Estanol B, da Silva O. Myasthenia gravis and pregnancy: Clinical implications and neonatal outcome. BMC Musculoskelet Disord 2004;5:42.
- Chiang LM, Darras BT, Kang PB. Juvenile myasthenia gravis. Muscle Nerve 2009;39:423-31.
- Ünsal Y, Yiş U. Jüvenil miyasteni gravis: Üç olgu sunumu ve literatürün gözden geçirilmesi. J Clin Anal Med 2015;6:473-8.
- Ekmekci O, Karasoy H. Prepubertal juvenile myasthenia gravis; A case with early onset and bulbar symptoms. J Neurol Sci 2013;30:583-6.
- Sarnat H. Disorders of neuromuscular transmission and of motor neurons. In: Kliegman RM, Stanton BF, Schor NF, St. Geme JW, Behrman RE (eds). Nelson Textbook of Pediatrics. 19th ed. Philadelphia: W.B. Saunders Company, 2011:2132-8.
- Değerliyurt A, Şenbil N, Anlar B. Jüvenil (otoimmün ) miyasteni: Klinik özellikler ve tedavi sonuçları. Çocuk Sağ Hast Derg 2005;48:25-9.
- Tanrısever Ö, Sarıoğlu B, Kanık A, Arslan CN, Baydan F. Farklı bir klinik başlangıç gösteren miyastenia gravis olgusu. Tepecik Eğit Hast Derg 2014;24:59-62.
- Raksadawan N, Kankirawatana P, Balankura K, Prateepratana P, Sangruchi T, Atchaneeyasakul LO. Childhood onset myasthenia gravis. J Med. Assoc Thai 2002;85:769-7.
- Meriggioli MN, Sanders DB. Autoimmune myasthenia gravis: Emerging clinical and biological heterogeneity. Lancet Neurol 2009; 8:475-90.
- Ashraf VV, Taly AB, Veerendrakumar M, Rao S. Myasthenia gravis in children: A longitudinal study. Acta Neurol Scand 2006;114:19–23.
- Salman E, Yetişir F, Yürekli B, Bozkurt A, Kılıç M. Myastenik kriz yönetiminde intravenöz immunoglobulin tedavisi: Olgu sunumu. J Clin Anal Med 2013;4:352-4.
- Long RR. Myasthenia gravis in the intensive care unit. In: Irwin RS, Cerra FB, Rıppe JM (eds). Intensive Care Medicine. Philadelphia: Lippincott-Raven Publishing, 1998:2122-7.
- Yosunkaya A, Çelik J.B, Tuncer S, Topal A, Ökesli S. Myastenia gravis ve immunoadsorbsiyon plazmaferez. T Klin J Medl Sci 2002;22:578-82.
Details
| Other ID | JA35KD93KT |
|---|---|
| Journal Section | Case Report |
| Authors | |
| Publication Date | April 1, 2018 |
| Submission Date | April 1, 2018 |
| Published in Issue | Year 2018 Volume: 12 Issue: 1 |
The publication language of Turkish Journal of Pediatric Disease is English.
Manuscripts submitted to the Turkish Journal of Pediatric Disease will go through a double-blind peer-review process. Each submission will be reviewed by at least two external, independent peer reviewers who are experts in the field, in order to ensure an unbiased evaluation process. The editorial board will invite an external and independent editor to manage the evaluation processes of manuscripts submitted by editors or by the editorial board members of the journal. The Editor in Chief is the final authority in the decision-making process for all submissions. Articles accepted for publication in the Turkish Journal of Pediatrics are put in the order of publication, with at least 10 original articles in each issue, taking into account the acceptance dates. If the articles sent to the reviewers for evaluation are assessed as a senior for publication by the reviewers, the section editor and the editor considering all aspects (originality, high scientific quality and citation potential), it receives publication priority in addition to the articles assigned for the next issue.
The aim of the Turkish Journal of Pediatrics is to publish high-quality original research articles that will contribute to the international literature in the field of general pediatric health and diseases and its sub-branches. It also publishes editorial opinions, letters to the editor, reviews, case reports, book reviews, comments on previously published articles, meeting and conference proceedings, announcements, and biography. In addition to the field of child health and diseases, the journal also includes articles prepared in fields such as surgery, dentistry, public health, nutrition and dietetics, social services, human genetics, basic sciences, psychology, psychiatry, educational sciences, sociology and nursing, provided that they are related to this field. can be published.