Chronic CSF leak causing syringomyelia and pseudo-Arnold-Chiari malformation

A 45-year-old woman developed orthostatic headaches after lifting weights. A CT myelogram localized a CSF leak at T5-T7. Multiple blood patches provided only transient relief. Over 5 years, the patient developed paresthesias, worsened headaches, and decreased hand coordination. MRI showed progressive cerebellar tonsillar descent and hydrosyringomyelia (figure). Repeat myelography was unrevealing, but subsequent multilevel epidural blood patches with fibrin glue led to headache resolution. Cerebellar tonsillar descent and hydrosyringomyelia can occur in chronic spontaneous CSF leaks.1 A CSF leak should be considered in patients with orthostatic headache and presumed Chiari malformation.


Figure Sagittal T2 MRI

Images demonstrate progressive cerebellar tonsillar descent and development of syrinx within the intramedullary cavity from C1 to C3, with T2 signal suggestive of intramedullary hydrostatic edema extending caudally to T4-5.

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