Owler B K, Halmagyi G M, Brennan J, Besser M
Departments of Neurosurgery and Neurology, Institute of Clinical Neurosciences, Royal Prince Alfred Hospital, Sydney, Australia.
Acta Neurochir (Wien). 2004 Oct;146(10):1137-43; discussion 1143. doi: 10.1007/s00701-004-0323-3.
Syringomyelia is an important cause of neurological deficit. Most cases of non-traumatic syringomyelia occur in association with a Chiari malformation. We present three unusual examples of syringomyelia with such an association. The first case is that of syringomyelia in a young woman with Marfan's syndrome, a spontaneous CSF leak and intractable intracranial hypotension. The second is a woman with long-standing lumbo-peritoneal shunt for pseudotumour cerebri who developed an acquired Chiari malformation. A young woman with a Dandy-Walker cyst that herniated into the upper cervical canal is the third case. These cases provide a basis for discussion of the pathogenesis and management of syringomyelia and the Chiari malformation in such cases.
脊髓空洞症是神经功能缺损的重要原因。大多数非创伤性脊髓空洞症病例与Chiari畸形相关。我们展示了三例伴有这种关联的不寻常脊髓空洞症病例。第一例是一名患有马凡综合征、自发性脑脊液漏和顽固性颅内低压的年轻女性的脊髓空洞症。第二例是一名长期因假性脑瘤行腰-腹腔分流术的女性，她出现了后天性Chiari畸形。第三例是一名患有Dandy-Walker囊肿并疝入上颈椎管的年轻女性。这些病例为讨论此类病例中脊髓空洞症和Chiari畸形的发病机制及治疗提供了依据。
