Repeated embolism and multiple aneurysms: central nervous system manifestations of cardiac myxoma

Acute cerebral embolism of left atrial myxomas has been well documented, but cerebral aneurysm formation resulting from atrial myxoma is a rare neurological manifestation. We describe here a patient whose left atrial myxoma and multiple cerebral aneurysms were identified after multiple cerebral embolisms during a 5year period. A 27-year-old woman was admitted to our hospital with a sudden onset of vertigo, dysarthria and right-sided weakness. The patient reported a history of a sudden fall with loss of consciousness and left-sided hemiplegia at the age of 22. At that time, she was diagnosed at a local hospital with multiple cerebral infarctions because of magnetic resonance imaging (MRI) evidence of multiple ischaemic lesions in the cerebellum, brain stem and basal ganglia (Fig. 1a and b). Echocardiography was not performed at the local hospital because of the patient s absence of any history or signs of cardiac problems. The patient fully recovered within 6 months and had no residual symptoms. However, the patient experienced two episodes of vertigo and right-sided weakness in the past 5 years. Each episode lasted 5–10 min and resolved spontaneously. No conventional vascular risk factors, stroke family history or contraceptive drug administration were reported. In our hospital, abnormal neurological signs in the patient included pathological laughing, scanning speech, muscle strength of right limbs: 3 (strength: 3/6 grade), hyperreflexia of the right deep tendon reflex and increased muscle tone in the right limbs presenting as spasticity and an ataxic gait. A cardiology consult revealed that the patient had a regular heart rate and rhythm, with no audible murmurs on auscultation. An electrocardiography demonstrated no abnormality as well. Laboratory tests were remarkable only for increased erythrocyte sedimentation (28.0 mm/h, reference: 0–20 mm/h) and C-reactive protein (5.63 mg/l, reference 0–5 mg/l). Clinical immunology studies, examination of the serum for syphilis and lumbar puncture revealed no abnormalities. A MRI scan disclosed an additional infracted lesion in the left cerebellar hemisphere in addition to the previous lesions (Fig. 1c). A T1-weighted MRI with contrast showed marked dilated arteries enhanced with gadolinium–DTPA in the posterior cerebral artery territories (Fig. 1d). Echocardiography revealed a pedunculated mass originating from the left atrium through the mitral valve into the left ventricle. Angiography disclosed multiple typical distal fusiform and saccular aneurysms or aneurysmal dilations in the bilateral internal carotid artery territories (Fig. 2a and b), as well as a marked enlargement of the proximal portion of the bilateral branch of the posterior cerebral arteries (Fig. 2c). Clinical and laboratory investigations including abdominal ultrasonography, chest radiography and vascular ultrasonography of the cervical and extremities did not reveal an aneurysm of the extracerebral arteries. The patient was then referred to the cardiosurgery department for a thoracotomy. Pathological analysis disclosed that the resected atrial mass, measuring 4 · 3 · 3 cm, was a typical myxoma. After surgery, the patient was discharged from the hospital without any specific treatment. No neurological symptoms reoccurred during 2 years of follow-up study. No myxoma was revealed during repeat echocardiographical investigation. Careful follow-up including MRI and angiography will still be continued for both recurrence and progression or regression of cerebral aneurysms.