Opsoclonus Myoclonus Ataxia Syndrome

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Gök V., Per H.

Rare Diseases in Children, Focusing on Pediatric Immunology & Neurology, Ekrem Ünal,Hüseyin Per,Ayşenur Paç Kısaarslan,Musa Karakükcü, Editör, Nobel Yayınevi, Ankara, ss.276-281, 2020

  • Yayın Türü: Kitapta Bölüm / Mesleki Kitap
  • Basım Tarihi: 2020
  • Yayınevi: Nobel Yayınevi
  • Basıldığı Şehir: Ankara
  • Sayfa Sayıları: ss.276-281
  • Editörler: Ekrem Ünal,Hüseyin Per,Ayşenur Paç Kısaarslan,Musa Karakükcü, Editör
  • Erciyes Üniversitesi Adresli: Evet

Özet

Opsoclonus myoclonus ataxia syndrome (OMAS), also known as dancing eye syndrome, is a rare neurological disorder, characterized by rapid, chaotic, and synchronous eye movements (opsoclonus), spontaneous muscle jerking (myoclonus), ataxia, and irritabil- ity.1,2 Ganglioneuroma/blastoma or neuroblastoma is detected in almost half of pediatric OMAS patients. Similarly, 1-2% of neuroblastomas are associated with OMAS. The other half develops due to parainfectious and idiopathic causes.3,4 There is an autoinflammatory process on the background of this disease. Although no single pathogenic autoantibody has been identified in individuals with OMAS, increased B cell function and the presence of oligoclonal bands in cerebrospinal fluid (CSF) support underlying autoimmunity and the importance of B cells in the pathophysiology of OMAS.5,6 Therefore, the recovery of neurological symptoms responds to immunotherapy. There are many treatment options as immunotherapy, including corticosteroids, adrenocorticotrophic hormone (ACTH), in- travenous immunoglobulin (IVIG), cyclophosphamide, plasmapheresis, rituximab, and mycophenolate mofetil.7-9