Infectious endocarditis complicated by an ischemic stroke and revealing Marfan syndrome

Abstract

Marfan syndrome is a systematic genetic disease of the connective tissue. The cardiac affection would predict the prognosis and ischemic stroke might complicate it. The purpose of this work is to discuss the mechanisms of the ischemic stroke in Marfan syndrome which have to be considered in all young patients of ischemic strokes. We report the case of a 17-year-old male patient who presented with right hemiparesis with brachio-facial dominance, hemihypoesthesia and Broca's aphasia; these symptoms were followed by partial right somato-motor epileptic seizure. The cerebral computed tomographic (CT) scan demonstrated early signs of ischemic stroke in the left Sylvian artery. Cardiovascular examination revealed a systolic murmur in the mitral site. Marfan syndrome with infectious endocarditis complicated by ischemic stroke was diagnosed. However, the family pathological history was negative. The Marfan syndrome diagnosis was approved considering the following criteria: Ligamentous hyperlaxity, crystalline ectopia and mitral valve prolapsus with mitral insufficiency. The patient benefited of antibiotherapy for 4 weeks. The patient underwent sessions of motor physical therapy and orthophonic rehabilitation. Then, cardiac surgery was carried out and valvuloplasty was performed. The surgical treatment confirmed the presence of mitral insufficiency and prolapsus of the big mitral valve and multiple friable anterior-posterior vegetations with broken cordage of the small mitral valve. Replacement of the mitral valve was achieved, and the post-surgery follow-ups were simple. The diagnosis of Marfan syndrome was based on well-defined criteria. The evolution of the patient was marked by a complete improvement of the cardiac and motor deficits, and disappearance of the Broca's aphasia. A fibroelastic disease such Marfan syndrome has to be considered in unexplained ischemic stroke in all young patients. Hence, the diagnosis of Marfan syndrome involves an anticipation of the neurovascular complications by early cardiovascular care.

A Rare Case Report of Endovascular Therapy for Basilar Artery Occlusion in a Young Adult with Marfan Syndrome

“…5 Maski reported a rare spontaneous intracranial artery dissection with cerebral infarction in a young Marfan syndrome patient and suggested that the infarction was due to the cerebral vasculature itself. 6 In this case, the patient had no evidence of atherosclerosis, cerebral aneurysm, cerebral arterial dissection or other known risk factors. Transthoracic ultrasound showed an aortic root aneurysm and found no emboli of cardiac origin.…”

Acute ischemic stroke leading to a diagnosis of Marfan syndrome: Case report and systematic review

“…MFS is a systemic connective tissue disorder with a prevalence of 2-3/100 000 individuals (5). About 75% of individuals with MFS develop an autosomal dominant inherited disease that is caused by mutation of the FBN1 gene, and approximately 25% have a de novo FBN1 pathogenic variant or germline mosaicism (3,5). MFS has variable cardiovascular, musculoskeletal, ophthalmologic, and pulmonary symptoms.…”

“…In a retrospective study of 513 people with MFS, the incidence of IS was 3% and that of hemorrhagic stroke was 0.5% (8). IS may arise from the dissection of aortic aneurysm or extra-/intracranial carotid artery, postoperative aortic root, valvular abnormalities, or cardiac valve disease (3,7). Cardioembolism is secondary to arterial dissection in AIS in individuals with MFS (9).…”

“…Table 1 lists the main clinical features of IS in cases of MFS reported in recent years (3,5,18,19,(24)(25)(26)(27)(28)(29). A cardioembolic cause was identi ed in the majority of the cases (5 out of 10) and arterial dissection was second (3 out of 10), whereas 3 cases had hemorrhagic transformation with/without intravenous thrombolysis after IS.…”

“…Neurologic symptoms are relatively rare in MFS and generally involve ischemic or hemorrhagic stroke. Ischemic stroke (IS) revealed by an arterial dissection or cardiogenic embolism secondary to IE is the most common neurologic complication of MFS (3).…”