Fibromuscular dysplasia is an uncommon condition of idiopathic, non-inflammatory and non-atherosclerotic disease of the musculature of arterial walls. The disease is rare, but it commonly affects young and middle aged women. Isolated intracranial cerebral fibromuscular dysplasia is extremely rare because cerebral fibromuscular dysplasia usually affects extracranial vessels. A 26-year-old woman was admitted with right hemiplegia and global aphasia. Brain MRI and MRA demonstrated acute left middle cerebral artery territory infarction with a multifocal stenosis and dilatation of the left middle cerebra artery and left internal carotid. The characteristic conventional cerebral angiographic findings demonstrated a typical string-of-beads appearance in the left distal internal carotid artery and proximal portion of the left middle cerebral artery, which suggested a medial type fibromuscular dysplasia. We report a case of isolated intracranial fibromuscular dysplasia with left middle cerebral artery territory infarction. Fibromuscular dysplasia should be considered as a stroke risk factors in children and young adults, especially in patients with no known cardiovascular risk factors.
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Cerebral Infarction and Recovery in a 12-Year-Old Child With Intracranial Fibromuscular Dysplasia Min Hwan Kim, Jae In Lee, Shin-seung Yang Brain & Neurorehabilitation.2023;[Epub] CrossRef
BACKGROUND Systemic embolism especially, cerebral infarction is one of the most important complications in patients with mitral stenosis. The authors analyse the some that could predict the development of cerebral infarction in cases of mitral stenosis and propose preventive therapeutic measures. METHODS: Retrospective study of 127 patients with rheumatic mitral stenosis was performed by analysis their medical records for transthoracic(TTE) or transesophageal echocardiography(TEE) over a 12months period. The patients were divided into two groups according to the presence (Group I: n=26, age: 55+/-13 years) or absence (Group II: n=101, age: 48.5+/-13 years) of cerebral infarction. No significant difference was observed between the two groups with respect to sex and functional class. RESULTS: Patients of group I were older (55.0+/-13 vs 48.5+/-13;p<0.05), had more dilated left atrial size(5.10+/-0.48 vs 4.81+/-0.70;p<0.05) and smaller mitral surface area(1.01+/-0.39 vs 1.21+/-0.45;p<0.05). In Group I, the incidence of atrial fibrillation(22 out of 26 vs 57 out of 101;p<0.05) and spontaneous left intra-atrial contrast phenomenon(22 out of 26 vs 44 out of 101;p<0.05) was more frequently observed. On multivariate analysis, atrial fibrillation and anticoagulant therapy were the independent predictive factors. CONCLUSION: Age, left atrial dilatation, the severity of mitral stenosis, the presence of spontaneous contrast and especially the presence of atrial fibrillation are the main predictive factors of the development of cerebral infarction in mitral stenosis. Patients presenting one or several of these factors may benefit from prophylactic anticoagulant treatment.
Antiphospholipid antibody syndrome(APS) is a well-known clinical syndrome characterized by recurrent arterial or venous thromboses, recurrent fetal loss, thrombocytopenia, together with high titers of sustained anticardiolipin antibody(aCL) or lupus anticoagulant(LA). Although systemic lupus erythematosus(SLE) and APS may coexist, a high proportion of patients manifesting the APS do not suffer from classical lupus or other connective tissue disease. The patient has been defined as having a primary antiphospholipid antibody syndrome. We experienced one case of primary APS with recurrent fetal loss, recurrent cerebral infarctions, positive anticardiolipin antibody IgG and fluttering vegetation on the mitral valve, without other connective tissue diseases including SLE. Forty-three old female had 2 out of 11 criteria for the diagnosis of SLE, such as thrombocytopenia and positive antinuclear antibody, but did not meet whole criteria. The patient was treated with ticlopidine, and anticoagulant therapy was recommended.