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儿童眼阵挛-肌阵挛综合征的最新进展。

Update on pediatric opsoclonus myoclonus syndrome.

作者信息

Hero Barbara, Schleiermacher Gudrun

机构信息

Department of Pediatric Oncology, Children's Hospital, University of Cologne, Köln, Germany.

出版信息

Neuropediatrics. 2013 Dec;44(6):324-9. doi: 10.1055/s-0033-1358604. Epub 2013 Nov 7.

Abstract

Opsoclonus myoclonus syndrome (dancing eye syndrome) is a rare paraneoplastic syndrome characterized by opsoclonus, myoclonus, and ataxia, usually accompanied by behavioral abnormalities. In adults, opsoclonus myoclonus syndrome has been reported in association with different types of cancer; whereas in children, the syndrome may be associated with neuroblastic tumors. Although a direct proof is lacking, the syndrome is assumed to be of autoimmune origin. The treatment is corticosteroid based with the addition of other immunosuppressive or immunomodulating drugs if intensification seems necessary. Because of the rarity of the disease, international collaborations as well on research as on therapeutic strategies are urgently needed. A European consortium just started a trial for this rare condition.

摘要

眼阵挛-肌阵挛综合征(舞动眼球综合征)是一种罕见的副肿瘤综合征,其特征为眼阵挛、肌阵挛和共济失调,通常伴有行为异常。在成人中,眼阵挛-肌阵挛综合征已被报道与不同类型的癌症相关;而在儿童中,该综合征可能与神经母细胞瘤有关。尽管缺乏直接证据,但该综合征被认为是自身免疫性起源。治疗以皮质类固醇为基础,如有必要强化治疗,则加用其他免疫抑制或免疫调节药物。由于该疾病罕见,迫切需要在研究和治疗策略方面开展国际合作。一个欧洲财团刚刚启动了针对这种罕见病症的试验。

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