Syringomyelia with Chiari malformation; 3 unusual cases with implications for pathogenesis

Authors: Owler, B. K.; Halmagyi, G. M.; Brennan, J.; Besser, M.

Source: Acta Neurochirurgica, Volume 146, Number 10, October 2004 , pp. 1137-1143(7)

Publisher: Springer

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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.

Keywords: Dandy-Walker malformation; Syringomyelia; idiopathic intracranial hypotension; pseudotumour cerebri

Document Type: Research Article


Affiliations: Departments of Neurosurgery and Neurology, Institute of Clinical Neurosciences, Royal Prince Alfred Hospital, Sydney, Australia,

Publication date: October 1, 2004

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