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The neuroimaging features of the cardiolipin antibody syndrome
K Weingarten1, C Filippi, D Barbut
1Department of Radiology, New York Hospital-Cornell Medical Center, New York 10021, USA.
Insights
Cardiolipin antibody syndrome, a cause of stroke in young adults, often presents with infarction and atrophy on brain imaging. Early diagnosis is crucial for preventing future cerebrovascular events.
Area of Science:
- Neurology
- Immunology
- Radiology
Background:
- Anticardiolipin antibody syndrome is associated with cerebrovascular events.
- Neuroimaging findings in this syndrome are not well-defined.
Purpose of the Study:
- To define the neuroimaging features of cardiolipin antibody syndrome.
- To identify risk factors for cerebrovascular events associated with anticardiolipin antibodies.
Main Methods:
- 38 patients with elevated anticardiolipin antibody titers underwent MRI or CT scans.
- Cerebral angiography was performed in 2 patients.
- Clinical data including transient ischemic attacks, amaurosis, and strokes were analyzed.
Main Results:
- Focal cerebral infarcts (32 patients) and cerebral atrophy (26 patients) were common neuroimaging findings.
- One patient had normal imaging; 5 had atrophy as the sole finding.
- Angiography revealed stenosis, occlusions, and collateral networks in cerebral circulation.
Conclusions:
- Cardiolipin antibody syndrome should be suspected in young patients with stroke or TIA without typical risk factors.
- Elevated anticardiolipin antibody titers indicate a risk for future ischemic cerebrovascular events, even without overt autoimmune disease.
- Understanding the role of these antibodies in thrombosis may elucidate stroke mechanisms.
Abstract:
The purpose of our study was to define the neuroimaging features of the cardiolipin antibody syndrome. Thirty-eight patients with elevated anticardiolipin antibody titers were studied with magnetic resonance imaging or computed tomography or both. Two patients underwent cerebral angiography. All patients had recurrent transient ischemic attacks, amaurosis, or strokes. One patient had normal imaging findings. The remaining patients had a combination of infarction and atrophy. Focal infarcts, the most common finding, were seen in 32 patients. Cerebral atrophy was seen in 26 patients and was the only radiographic finding in 5. Angiography demonstrated dramatic abnormalities in the distal portions of the anterior and posterior circulations, with multiple stenosis and occlusions and extensive pial and transdural collateral networks. The cardiolipin antibody syndrome should be suspected in young patients with transient ischemic attacks or strokes in the absence of the usual risk factors for cerebrovascular disease. The presence of raised anticardiolipin antibody titers or the cardiolipin antibody syndrome in patients with lupus, in those with other connective tissue diseases, and in patients without overt manifestations of an autoimmune disorder should be viewed as a risk factor for future ischemic cerebrovascular events. Further understanding of the precise role of these antibodies in the pathogenesis of vascular thrombosis may lead to a better understanding of the mechanisms underlying certain forms of stroke.