Absent pachymeningeal gadolinium enhancement on cranial MRI despite symptomatic CSF leak

CSF leak is recognized to cause orthostatic headaches and diffuse pachymeningeal gadolinium enhancement (DPMGE) on MRI. We report six patients with typical symptoms and documented CSF leaks without DPMGE. Two had normal meninges from the onset; one initially had normal meninges, but subsequently DPMGE developed. In three, the initially noted DPMGE resolved while they were still symptomatic with documented continued CSF leaks. Absent DPMGE does not rule out CSF leak. When clinical manifestations suggest this disorder, additional diagnostic studies should be pursued.