Milhorat Thomas H, Bolognese Paolo A, Black Karen S, Woldenberg Rona F
Department of Neurosurgery, The Chiari Institute, North Shore-Long Island Jewish Health System, Manhasset, New York 11030, USA.
Neurosurgery. 2003 Nov;53(5):1220-1; discussion 1221-2. doi: 10.1227/01.neu.0000088809.14965.00.
Syringomyelia is generally regarded as a chronic, slowly progressive disorder. We describe a case of acute dilation of the central canal of the spinal cord that presented with rapidly progressive segmental signs.
A 29-year-old female patient who had previously undergone surgical treatment for a Chiari I malformation, syringomyelia, and hydrocephalus presented with an 8-day history of headaches, progressive paraparesis, and urinary retention. Magnetic resonance imaging scans demonstrated panventricular hydrocephalus in association with a holocord syrinx that extended to the obex. Magnetic resonance imaging scans that had been coincidentally obtained just 3 days before the onset of symptoms had revealed no evidence of hydrocephalus or syringomyelia.
The patient underwent emergency revision of a failed ventriculoperitoneal shunt. Postoperatively, there was prompt resolution of the syringomyelia, hydrocephalus, and associated neurological deficits.
Among patients with communicating syringomyelia, the central canal of the spinal cord participates as a "fifth ventricle" and can undergo rapid dilation in association with acute hydrocephalus. Appropriate treatment in such cases involves placement of a ventriculoperitoneal shunt.
脊髓空洞症通常被视为一种慢性、进展缓慢的疾病。我们描述了一例脊髓中央管急性扩张的病例,该病例表现为快速进展的节段性体征。
一名29岁女性患者,既往曾接受过针对Chiari I畸形、脊髓空洞症和脑积水的手术治疗,此次出现头痛、进行性双下肢轻瘫和尿潴留8天。磁共振成像扫描显示全脑室脑积水,伴有延伸至延髓闩部的全脊髓空洞。症状出现前3天偶然获得的磁共振成像扫描未显示脑积水或脊髓空洞症的迹象。
患者接受了对失败的脑室腹腔分流术的紧急修复。术后,脊髓空洞症、脑积水及相关神经功能缺损迅速得到缓解。
在交通性脊髓空洞症患者中,脊髓中央管作为“第五脑室”参与其中,并可与急性脑积水相关联而迅速扩张。此类病例的适当治疗包括放置脑室腹腔分流管。