Spontaneous Intracranial Artery Dissection in a Patient with Turner Syndrome

Abstract

Background— Turner syndrome is a genetic disorder caused by partial or complete monosomy of the X chromosome.
Although a few cases of ischemic stroke have been reported in Turner syndrome, but isolated intracranial artery
dissection has not yet been reported. We report a case of a patient with Turner syndrome with middle cerebral artery
dissection and, present the clinical and radiological features.
Case Description— A 34-year-old woman with Turner syndrome and diabetes mellitus was referred to our
hospital with symptoms of left hemiparesis post appendectomy. Magnetic resonance imaging outside hospital
showed diffusion restriction in the right middle cerebral artery territory with magnetic resonance angiography (MRA)
identifying a right MCA occlusion. Digital subtraction angiography revealed a M1 segment occlusion. Mechanical
thrombectomy of the right M1 segment was performed but a triangular flow-limiting stenosis for an underlying
dissection. Intracranial stent placement was then performed with successful reperfusion.
Conclusions— Ischemic stroke in Turner syndrome can occur due to dissection of intracranial arteries and may
require additional stent placement during mechanical thrombectomy. Further studies are required to investigate the
mechanism for predisposition for dissection in Turner syndrome