Paradoxical presentation of orthostatic headache associated with increased intracranial pressure in patients with cerebral venous thrombosis
A previously healthy 19-year-old man admitted with a 2-day history of headache. The headache was localized to the occipital area, severe in intensity and throbbing in nature. Typically, the headache in this patient was position-dependent, and developed with orthostatic posture, leading to severe intensity in the upright position (visual analogue scale [VAS] 9–10/10), and was relieved when the patient was in the recumbent position. Routine neurologic examination and laboratory findings were normal. Initial computerized tomography (CT) of the brain showed no abnormalities. Cerebrospinal fluid (CSF) tapping was done under the suspicion of SIH, but the opening pressure was over 40 cmH2O (normal range: 8-18 cmH2O), indicating increased intracranial pressure (IICP). After admission, the patient developed left upper extremity monoparesis with intermittent clonic movement, followed by generalized tonic movement. Diffusion-weighted magnetic resonance imaging (MRI) (DWI) of the brain was performed immediately after the episode. The b-value of 1000 s/mm2 and apparent diffusion coefficient (ADC) image of the DWI showed high signal intensity in the right parietal cortex, suggesting vasogenic edema [Figure -]. Gradient-echo imaging (GRE) identified superior sagittal sinus (SSS) and cortical vein dilatation [Figure and ]. Subsequent MRI revealed right parietal, left frontal cortex high-signal intensity on T2-weighted and fluid-attenuated inversion recovery images, with an empty delta sign on gadolinium-enhanced T1-weighted images [Figure -]. There was no evidence to suggest SIH on MRI, such as pachymeningeal enhancement or downward brain displacement. Brain MR venography (MRV) revealed the absence of the SSS, straight sinus and right transverse sinus [Figure and ]. We made a diagnosis of CVT with venous infarction according to the established criteria[] to which the patient's headache and seizure were attributed. Further laboratory tests for causative medical conditions showed deficiencies in protein C (16%, normal range: 70- 130%) and protein S (33%, normal range: 74- 146%). The headache and seizure completely subsided after intravenous anticoagulation treatment. He remained asymptomatic without any neurological sequelae after 10 months of follow-up.