Abstract
Opsoclonus-myoclonus syndrome (OMS) is a rare neurological disorder. Chronic neurological symptoms in patients, improperly examined and treated, and diagnostic errors during their first visit to a doctor suggest that most physicians are unaware of this syndrome and criteria for its diagnosis. The pediatrician who follows up an infant with cerebellar ataxia must know that this condition may be a manifestation of OMS. 50 % of childhood-onset OMS cases are associated with neuroblastoma (NB). However, to date difficulties have emerged in the early diagnosis of this condition and in the determination of initial examination scope and therapy policy. This paper reviews the literature on different aspects of the pathogenesis, diagnosis, clinical presentation, treatment, and prognosis of OMS. The issues of NB-associated OMS are considered separately.
Highlights
Varfolomeeva Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Ministry of Health of Russia, Moscow
Chronic neurological symptoms in patients, improperly examined and treated, and diagnostic errors during their first visit to a doctor suggest that most physicians are unaware of this syndrome and criteria for its diag nosis
The pediatrician who follows up an infant with cerebellar ataxia must know that this condition may be a manifestation of Opsoclonus-myoclonus syndrome (OMS). 50 % of childhood-onset OMS cases are associated with neuroblastoma (NB)
Summary
Синдром опсоклонус-миоклонус (ОМС) является редким неврологическим синдромом. С нейробластомой (НБ) ассоциированы 50 % случаев ОМС, развившегося в детском возрасте. В данной статье приведен обзор литературы, посвященный различным аспектам патогенеза, диагностики, клиники, лечения и прогноза при ОМС. Opsoclonus-myoclonus syndrome (OMS) is a rare neurological disorder. The pediatrician who follows up an infant with cerebellar ataxia must know that this condition may be a manifestation of OMS. 50 % of childhood-onset OMS cases are associated with neuroblastoma (NB). The issues of NB-associated OMS are considered separately. 1), известный под синонимами «синдром танцующих глаз» и «энцефалопатия Кинсбурна», характеризуется 3 основными симптомами: опсоклонус (совместные быстрые, нерегулярные и неравномерные по амплитуде движения глазных яблок, обычно в горизонтальной плоскости, наиболее выраженные в начале фиксации взгляда), миоклонус (подергивания неэпилептического характера, которые захватывают туловище, конечности и лицо) и атаксия.
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