Bilateral internal carotid dissection and multiple embolic cerebral infarcts in a woman with Turner’s syndrome treated with exogenous hormones to induce pregnancy

Abstract

To the Editor: Turner’s syndrome (TS) is a relatively common chromosomal disorder affecting approximately 1 in 2500 live female births. It is associated with a range of comorbidities involving the cardiovascular system, which affects both pediatric and adult patients. Vascular abnormalities, including aortic dilatation, aortic dissection, aortic coarctation, aneurysm, hemangioma, and arteriovenous malformations, are common findings in TS and have been reported to be up to 45% of cases [1]. Aortic dissection, which can occur with or without coarctation, is a rare but potentially life-threatening complication of TS; however, dissection involving the cerebral arteries is rare. We report the case of a woman with TS, treated with high doses of estrogen progestin therapy to induce pregnancy, who developed multiple embolic cerebral infarcts caused by bilateral carotid dissection. The patient was a 37-year-old woman diagnosed with TS (karyotype: 45, X) at the age of 12 due to short stature. She had been treated for 20 days until the day before admission with hormone replacement therapy (estrogen and progesterone) for an assisted pregnancy. She was admitted to our neurology ward with lower left limb hyposthenia, left inferior facial paresis, and disequilibrium (NIHSS = 2). She also complained of a mild ovarian hyperstimulation syndrome (OHS) with feeling of fullness, headache, and vomit for 4 days. She denied any neck manipulation or trauma. During the hospitalization, brain computed tomography (CT) was performed which was unremarkable. Carotid ultrasound test and magnetic resonance imaging (MRI) with intracranial angiography were also performed. Neck arteries ultrasound test showed a progressive severe narrowing of both the internal carotid arteries (ICA) in the distal tract with a low amplitude sisto-diastolic flow (absent the diastolic flow in the left ICA). Cerebral MRI showed small lesions involving multiple vascular territories that were hyperintense with restricted diffusion in diffusion-weighted imaging (Fig. 1a) and magnetic resonance angiography (MRA) demonstrated lack of opacification of the left ICA in the intracranial tract with an endoluminal dissecting aspect and a very narrow right ICA at the origin of the intracranial tract (Fig. 1b). Then, the patient underwent an angiographic study which revealed bilateral extracranial carotid dissection (Fig. 1c–d). Transthoracic echocardiography revealed normal valvular structure and function with a normal left ventricular ejection fraction. Patent foramen ovale, atrial septal aneurysm, or any aortic pathologies including aortic atheroma were not detected in transesophageal echocardiography. Holter ECG monitoring and Transcranial doppler failed to find an embolic source. An anticoagulant (low molecular weight heparin and then warfarin) therapy was initiated following the diffuse practice of using it when the carotid dissection is extracranial and produces severe stenosis even if there is no evidence that proves the superiority of this therapy over the antiplatelet one. The patient’s symptoms disappeared after 5 days confirming the favorable outcome of embolic stroke in young adults described in the literature. On clinical follow-up, after 2 months of warfarin therapy, the woman had a normal neurological examination and CT angiography showed patency of the carotid arteries (Fig. 2). The anticoagulant therapy was consequently stopped. * Luigi Bartolomei [email protected]