Orthostatic headaches caused by CSF leak but with normal CSF pressures

OBJECTIVE: To report that the syndrome of orthostatic headaches caused by CSF leak can be seen with persistently normal CSF pressures. BACKGROUND: CSF leak or shunt overdrainage is known to cause orthostatic headaches and diffuse pachymeningeal gadolinium enhancement (DPGE), typically associated with unmeasurable or very low CSF pressures. METHODS: Of 40 consecutive patients with orthostatic headaches and DPGE, all had low or unmeasurable CSF pressures, except seven patients who had consistently normal CSF pressures and are thus reported. All had undergone multiple CSF examinations. RESULTS: Two patients had overdraining shunts, and five had documented CSF leaks. One refused treatment, but the other six patients responded to surgical treatment or epidural blood patch with complete resolution of symptoms and related MRI abnormalities. CONCLUSIONS: Some patients with symptomatic CSF leaks may have CSF opening pressures that are consistently within normal limits. In the presence of convincing clinical features and imaging abnormalities, a normal CSF pressure should not discourage the clinician from searching for a source of CSF leak.