OPSOСLONUS-MYOCLONUS SYNDROME

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Abstract

This article provides an overview of the Russian and foreign studies on paraneoplastic opsoсlonus-myoclonus syndrome. Opsoclonus is characterized by involuntary, arrhythmic, chaotic, multi-directional saccades with horizontal, vertical and torsional components, and it is commonly accompanied by cerebellar ataxia and myoclonic jerks in the trunk and limbs. It is a rare neurological disorder of unknown causes which appears to be the result of an autoimmune process involving the nervous system. Paraneoplastic opsoсlonus-myoclonus syndrome is most commonly associated with small-cell lung cancer, breast cancer, ovarian cancer, non-Hodgkin's lymphoma, renal adenocarcinoma. In children, a neuroblastoma is detected in approximately 50% of cases. Many autoantibodies have been detected in patients with paraneoplastic opsoсlonusmyoclonus
syndrome: this finding suggests the involvement of a humoral immune mechanism. However, most patients are seronegative for these autoantibodies. Paraneoplastic opsoсlonus-myoclonus syndrome is less responsive to immunotherapy (corticosteroids, intravenous immunoglobulin, adrenocorticotropic hormone, plasma exchange, cyclophosphamide, or rituximab) and improves only with tumor resection. Further studies are needed to further elucidate its immunopathogenesis and pathophysiology in order to develop novel and efficacious therapy.

About the authors

N. A. Shnayder

Prof. V.F. Voino-Yasenetsky Krasnoyarsk State Medical University, Ministry of Health of the Russian Federation

Clinical Hospital Fifty-One, Federal Biomedical Agency, Zheleznogorsk

Author for correspondence.
Email: NASchnaider@yandex.ru
Russian Federation

E. A. Kantimirova

Prof. V.F. Voino-Yasenetsky Krasnoyarsk State Medical University, Ministry of Health of the Russian Federation

Email: fake@neicon.ru
Russian Federation

V. A. Ezhikova

Prof. V.F. Voino-Yasenetsky Krasnoyarsk State Medical University, Ministry of Health of the Russian Federation

A.I. Kryzhanovsky Krasnoyarsk Territorial Cancer Center

Email: fake@neicon.ru
Russian Federation

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Copyright (c) 2013 Shnayder N.A., Kantimirova E.A., Ezhikova V.A.

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