Department of Neurosurgery, Fayazbakhsh Hospital, Tehran, Iran.
Department of Pathology, Fayazbakhsh Hospital, Tehran, Iran.
Global Spine J. 2011 Dec;1(1):19-22. doi: 10.1055/s-0031-1296052.
Chiari malformations are a congenital heterogeneous group of disorders characterized by anatomic anomalies of the cerebellum, brain stem, and craniocervical junction associated with downward displacement of the cerebellum, alone or with lower medulla, into the cervical spine canal. The patient was a 23-year-old woman, a known case of Arnold-Chiari malformation with peripheral neuropathy and muscular atrophy, who presented with headache, drowsiness, decreased vision, and severe gait dysfunction lasting for several years. Brain magnetic resonance imaging confirmed a hypointense signal mass in the left hemisphere of the cerebellum causing mass effects on the fourth ventricle, which shifted it, accompanied with dilation of third and lateral ventricles.
Chiari 畸形是一组先天性异质性疾病,其特征为小脑、脑干和颅颈交界区的解剖异常,伴有小脑单独或连同下部延髓向下移位至颈椎椎管。该患者为一名 23 岁女性,已知患有 Arnold-Chiari 畸形合并周围神经病和肌肉萎缩,表现为头痛、嗜睡、视力下降和严重步态功能障碍,持续数年。脑部磁共振成像证实左侧小脑半球存在低信号肿块,对第四脑室产生占位效应,导致其移位,同时伴有第三脑室和侧脑室扩张。