Multiple Vascular Accidents Including Rupture of a Sinus of Valsalva Aneurysm, a Minor Ischemic Stroke and Intracranial Arterial Anomaly in a Patient with Systemic Congenital Abnormalities: A Case Report
A 39-year-old man was admitted to our hospital because of a sudden onset of hemiparesis on his left side with dysarthria. He had a history of a SVA rupture that was surgically treated at the age of 24 (fig. ). During the surgery, there were no specific findings of cystic medial necrosis or crystalline lens subluxation due to the Marfan syndrome. In his infancy, there were no developmental abnormalities. In his school records, the subject had poor grades during childhood and adolescence. His family history over 3 generations was unremarkable in terms of neurological, psychological, and cardiac diseases. During the patient's examination, his body temperature was 37.0°C, blood pressure was 184/104 mm Hg, pulse was 83 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 97% (while he was breathing ambient air). At the time of hospitalization, the patient presented with an acute stroke and the symptoms included left-sided weakness and dysarthria; these neurological symptoms lasted for a week after admission. We assessed the risk of juvenile ischemic stroke in a laboratory study, but high-risk factors that are linked to juvenile cerebral ischemia, such as collagen diseases and vasculitides, were not found. An electrocardiogram revealed a sinus rhythm within the normal range. Although we performed magnetic resonance imaging (MRI) with a 1.5-tesla unit on the first day and with a 3-tesla unit 2 weeks later, acute cerebral infarction was not detected (fig. ). Routine sonographic evaluation of the carotid arteries demonstrated a diffuse narrowing of the left ICA and a low-lying carotid bifurcation. Thereby, cerebral angiography was conducted, and it showed hypoplasty of the left ICA and a low-lying carotid bifurcation at the level of the C6 vertebra (fig. ). The A1 segment of the bilateral anterior cerebral arteries (ACAs) was united and formed an azygos type (fig. ). The common ACA duct and left posterior cerebral artery were mainly supplied by the right ICA. Aortography and 4 vessel studies (bilateral common carotid arteries and vertebral arteries) were conducted, and there was no embolic source, such as ulceration of the arterial surface or arterial dissection, or significant partial arterial stenosis. No right-to-left shunt was found in transesophageal echocardiography.123 I iofetamine single-photon emission computed tomography brain imaging was performed, and no regional reductions or abnormal accumulations of tracer uptake into the brain were observed. The patient's intelligence quotient was evaluated with the Wechsler Adult Intelligence Scale-III []; his mental faculties were found to be at a significantly low level of functioning (table ). Facial manifestations were unremarkable, but he showed symptoms of nasal speech and nasal air emission while talking. In the otorhinolaryngological examinations, velopharyngeal insufficiency was revealed by laryngoscopy. The patient was discharged after 3 weeks; MRI could not detect any significant abnormal findings such as infarction, hemorrhage, or malformation. We decided that the patient should continue with an antithrombotic therapy after assessing normal treatment for cerebral ischemia. The patient is now taking warfarin; the target range for the prothrombin time/international normalized ratio is 2.0–3.0.