Primary antiphospholipid antibody syndrome: neuroradiologic findings in 11 patients

Abstract

Objective: To describe the neuroradiologic findings of primary antiphospholipid antibody syndrome (PAPS).

Materials and methods: During a recent two-year period, abnormally elevated antiphospholipid antibodies were detected in a total of 751 patients. In any cases in which risk factors for stroke were detected-hypertension, diabetes mellitus, hyperlipidemia, smoking, and the presence of SLE or other connective tissue diseases-PAPS was not diagnosed. Neuroradiologic studies were performed in 11 of 32 patients with PAPS. We retrospectively reviewed brain CT (n = 7), MR (n = 8), and cerebral angiography (n = 8) in 11 patients with special attention to the presence of brain parenchymal lesions and cerebral arterial or venous abnormalities.

Results: CT or MR findings of PAPS included nonspecific multiple hyper-intensity foci in deep white matter on T2-weighted images (5/11), a large infarct in the territory of the middle cerebral artery (4/11), diffuse cortical atrophy (2/11), focal hemorrhage (2/11), and dural sinus thrombosis (1/11). Angiographic findings were normal (5/8) or reflected either occlusion of a large cerebral artery (2/8) or dural sinus thrombosis (1/8).

Conclusion: Neuroradiologic findings of PAPS are nonspecific but in young or middle-aged adults who show the above mentioned CT or MR findings, and in whom risk factors for stroke are not present, the condition should be suspected.

Fig. 1

A 47-year-old man who at the time of examination had suffered headache and general weakness for one month (case 4). Axial T2-weighted MR image shows multiple high signal intensities(arrows) in the white matters at the level of the centrum semiovale.

Fig. 2

A 32-year-old man who reported the onset of hemiplegia three hours prior to examination (case 2). A. Contrast-enhanced CT scan shows subtle low density in the right temporal lobe and insular cortex(arrows), suggesting a hyper-acute infarct in the territory of the right middle cerebral artery. B. Frontal view of right internal carotid angiogram shows complete occlusion of the right middle cerebral artery at M1 portion(arrow).

Fig. 3

A 34-year-old woman with progressive dementia (case 8). Axial T1-weighed MR image shows diffuse cerebral atrophy with prominent sulci and dilatation of the lateral ventricles.

Fig. 4

A 25-year-old woman who had suffered right hemiplegia for 2 days (case 6). A. Enhanced CT scan shows a filling defect in the superior sagittal sinus (empty delta sign, arrow). B. FLAIR axial T2-weighted MR image shows abnormally high signal intensity in the right transverse sinus, suggesting thrombosis (arrows). C. Oblique view of right internal carotid angiogram during the venous phase shows multiple filling defects in the superior sagittal sinus (arrows).