一名儿童中Lhermitte-Duclos病与脊髓纵裂畸形的关联。

Association of Lhermitte-Duclos disease and split cord malformation in a child.

作者信息

Abi Lahoud Georges, Chalouhi Nohra, Zanaty Mario, Rizk Tony, Jabbour Pascal

机构信息

Department of Neurosurgery, Division of Spine Surgery, Paris Descartes University and Sainte-Anne University Hospital, Paris, France.

Department of Neurosurgery, Thomas Jefferson University and Jefferson Hospital for Neuroscience, 901 Walnut Street, 3rd Floor, Philadelphia, PA 19107, USA.

出版信息

J Clin Neurosci. 2014 Nov;21(11):1999-2002. doi: 10.1016/j.jocn.2014.05.005. Epub 2014 Jun 15.

DOI:10.1016/j.jocn.2014.05.005

PMID:24938384

Abstract

Lhermitte-Duclos disease (LDD) or cerebellar gangliocytoma is a rare pathological entity, the etiology of which remains controversial. Numerous developmental anomalies are known to be associated with LDD, but the association between LDD and split cord malformation (SCM) has not been reported to our knowledge. We report LDD in a 3-year-old girl in whom repetitive urinary tract infections led to the diagnosis of a neurogenic bladder. Thorough screening revealed a SCM at the thoracic level which was surgically repaired. The pathogenesis of this entity and management strategies are discussed in this paper.

摘要

Lhermitte-Duclos病（LDD）或小脑神经节细胞瘤是一种罕见的病理实体，其病因仍存在争议。已知许多发育异常与LDD相关，但据我们所知，LDD与脊髓纵裂畸形（SCM）之间的关联尚未见报道。我们报告了一名3岁女孩患有LDD，其反复尿路感染导致神经源性膀胱的诊断。全面筛查发现胸段存在SCM，并进行了手术修复。本文讨论了该实体的发病机制和治疗策略。