Cerebral blood flow and glucose metabolism in multi-infarct-dementia related to primary antiphospholipid antibody syndrome

The primary antiphospholipid antibody syndrome (PAPS) has been described in patients with a history of fetal loss, thrombocytopenia and arterial or ve nous thrombosis. In PAPS, a prothrombotic state is mediated by antiphosphol ipid antibodies (aPLs) leading to disseminated thromboembolic vascular occl usion. Today, the presence of aPLs in the serum is considered as a distinct risk factor for recurrent stroke in young adults. Some PAPS patients devel op a multi-infarct-syndrome with a stepwise decline of higher cortical func tions. We report on a 55-year-old man suffering from progressive dementia a nd PAPS, in whom cerebral glucose metabolism and blood flow were examined b y positron emission tomograpy (PET). Cerebral atrophy and moderate signs of leukaraiosis were detected in magnetic resonance imaging (MRI), whereas th e PET scans showed a considerable diffuse impairment of cortical glucose me tabolism combined with a reduced cerebral perfusion in the arterial border zones. These findings indicate that PAPS-associated vascular dementia is ac companied by a cortical neuronal loss, presumably caused by a small-vessel disease with immune-mediated intravascular thrombosis. This case shows that pathological findings in PAPS are congruent to cerebral changes of metabol ism and blood flow in systemic lupus erythematosus (SLE).