Spontaneous intracranial hypotension complicated with cerebral venous thrombosis and intracerebral hemorrhage in a patient with protein S deficiency: a case report

Cerebral venous thrombosis (CVT) has been reported as an uncommon complication of spontaneous intracranial hypotension (SIH). Since this is a rare presentation, we report a patient with SIH complicated by CVT and intracerebral hemorrhage (ICH). A 43-year-old woman presented with a 2-day history of orthostatic headache and neck stiffness. Magnetic resonance imaging (MRI) of the brain demonstrated typical findings of SIH with pituitary hyperemia and engorgement of the venous structures, as well as flattening of the pre-pontine space. Lumbar puncture confirmed low opening cerebrospinal pressure (10 mm H2O). Neurological examination on admission was normal, except for left optic nerve atrophy with associated blindness that was due to an old central retinal artery occlusion. However, on day 3 of admission, the patient developed acute left-sided hypesthesia and hemiparesis. Computed tomography (CT) scan of the brain revealed CVT related ICH. The headache failed to respond to conservative treatment, but resolved within an hour of applying an epidural blood patch to the lower cervical region. Thrombophilia screening was performed and showed protein S dysfunction (<10 %). Warfarin was initiated as maintenance therapy and the patient did not experience any recurrent CVT event or orthostatic headache during the 1.5-year follow-up period. This report highlights that CVT with ICH is a potential complication of SIH. Any change of neurological status in a patient with SIH should raise the index of suspicion for CVT. A thorough investigation of the underlying cause is warranted and should include a thrombophilia screen. * Corresponding author. Department of Neurology, Changhua Christian Hospital 135 Nanhsiao Street, Changhua, 500 Taiwan E-mail address: manchi @cch.org.tw (M.-C. Lo) Copyright © 2013, Changhua Christian Hospital. The Changhua Journal of Medicine journal homepage: http://www2.cch.org.tw/7477