title: Syringomyelia with Chiari malformation; 3 unusual cases with implications for pathogenesis.
abstract: 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.
authors.affiliation: Departments of Neurosurgery and Neurology, Institute of Clinical Neurosciences, Royal Prince Alfred Hospital, Sydney, Australia. brianowl@bigpond.com
authors.forename: B K
authors.initials: BK
authors.lastname: Owler
authors.forename: G M
authors.initials: GM
authors.lastname: Halmagyi
authors.forename: J
authors.initials: J
authors.lastname: Brennan
authors.forename: M
authors.initials: M
authors.lastname: Besser
mesh_terms: D000328:Adult; D016080:Arachnoid Cysts / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D001139:Arnold-Chiari Malformation / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D001933:Brain Stem / Q000002:abnormalities* / Q000473:pathology / Q000503:physiopathology; D002531:Cerebellum / Q000002:abnormalities / Q000473:pathology / Q000503:physiopathology; D002557:Cerebrospinal Fluid Shunts; D003388:Cranial Fossa, Posterior / Q000002:abnormalities* / Q000473:pathology / Q000503:physiopathology; D003616:Dandy-Walker Syndrome / Q000150:complications / Q000473:pathology / Q000503:physiopathology; D005260:Female; D006801:Humans; D019585:Intracranial Hypotension / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D008382:Marfan Syndrome / Q000150:complications; D011559:Pseudotumor Cerebri / Q000150:complications / Q000601:surgery; D013116:Spinal Cord / Q000473:pathology* / Q000503:physiopathology; D013595:Syringomyelia / Q000150:complications* / Q000473:pathology / Q000503:physiopathology