1.Rapid change of estrogen levels induce reversible cerebral vasoconstriction syndrome and cerebral venous sinus thrombosis: A report of two cases
Yu Shimizu ; Katsuhiro Tsuchiya ; Hironori Fujisawa
Neurology Asia 2020;25(2):197-201
Reversible cerebral vasoconstriction syndrome (RCVS) presents with characteristic clinical, brain
imaging, and angiographic findings. The most common clinical feature of RCVS is a severe acute
headache, which is often referred to as a thunderclap headache owing to the nature of its presentation.
It may occur spontaneously or may be provoked by various precipitating factors. We present two
cases of RCVS concomitant with cerebral venous sinus thrombosis (CVST). Patient 1 was a 42-yearold woman admitted to our hospital with severe headache radiating to the neck, with associated
vomitting. She had a history of ovarian cancer and underwent an operation for resection of the tumor
a month prior to presentation. After resection, her estradiol (E2) levels were reduced from 288 pg/
ml to 31 pg/ml (normal range, 0-49 pg/ml). Initial imaging upon admission to our hospital revealed
left posterior convexity subarachnoid hemorrhage. Magnetic resonance angiography (MRA) showed
findings consistent with RCVS affecting the left posterior cerebral artery. Magnetic resonance
venography (MRV) showed CVST of the left transverse and sigmoid sinuses. Single photon emission
computed tomography (SPECT) showed a left posterior ischemic lesion. These findings improved
following treatment with nimodipine and anticoagulant. Patient 2 was a 39-year-old woman presented
with holocranial headache associated with vomiting. She was diagnosed with an ovarian tumor.
She underwent an operation three months prior to presentation. After tumor resection, her E2 level
decrease from 193 pg/ml to 19 pg/ml (normal range, 0-49 pg/ml). MRA confirmed the presence of
a vasospasm involving the right anterior cerebral artery. MRV confirmed the presence of thrombosis
involving the superior sagittal sinus. She was discharged on postpartum day 31 without neurological
deficits after treatment with anticoagulants. At 3 month follow-up, both MRA and MRV were within
the normal limits. In conclusion, this is the first report of two women diagnosed with RCVS with
concomitant CVST following ovarian tumor resection. The rapid change of perioperative E2 levels
may have contributed to the development of CVST and RCVS.