Longridge N S, Mallinson A I
Laryngoscope. 1985 Mar;95(3):335-9.
The Arnold-Chiari malformation (ACM) is a rare congenital condition in which there is a downward displacement of the cerebellum through the foramen magnum. Patients with this condition frequently develop symptoms of ataxia, imbalance, or vertigo. As a result the patient is often referred to an otolaryngologist. Such a case is reported. A patient presented with imbalance, had downbeating nystagmus on down gaze, and had other eye movement abnormalities recorded on electronystagmography (ENG) which were strongly suggestive of central pathology. Although her computerized tomography (CT) scan was normal, the eye findings and neurological evaluation were strongly suggestive of an ACM. A repeat CT scan with intrathecal metrizamide was abnormal. A definite diagnosis of ACM was confirmed using the new investigative technique of magnetic resonance imaging (MRI).
阿诺德-奇阿利畸形(ACM)是一种罕见的先天性疾病,小脑通过枕骨大孔向下移位。患有这种疾病的患者经常出现共济失调、平衡失调或眩晕症状。因此,患者常被转诊至耳鼻喉科医生处。本文报告了这样一个病例。一名患者出现平衡失调,向下注视时出现下跳性眼球震颤,眼震电图(ENG)记录到其他眼球运动异常,强烈提示中枢性病变。尽管她的计算机断层扫描(CT)结果正常,但眼部检查结果和神经学评估强烈提示为ACM。鞘内注射甲泛葡胺后的重复CT扫描结果异常。使用磁共振成像(MRI)这项新的检查技术确诊为ACM。