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Cerebral Disease in the Antiphospholipid Syndrome

  • Chapter
Hughes Syndrome

Abstract

Antiphospholipid antibody syndrome (APS) has been associated with central nervous system (CNS) involvement in a large number of patients. However, not all studies have found a significant correlation between antiocardiolipin (aCL) levels and neurological/neuropsychiatric manifestations of APS [1]. Clinical manifestations of APS associated with the CNS include arterial thrombotic events, psychiatric manifestations and a variety of other non-thrombotic neurological syndromes [2] (see Table 5.1). In fact, some of the most debilitating clinical aspects of APS are the neurological and neuropsychiatric manifestations. Nevertheless, the specific role of antiphospholipid antibodies (aPL) in the CNS remains one of the least understood aspects of this syndrome, which is partly due to the heterogeneity of its symptomatology. Patients may present with diffuse or global dysfunction, including coma, grand mal seizures, and cognitive dysfunction, psychosis, affective disorders, as well as focal symptoms attributable to lesions to a specific area of the brain. A majority of cognitive disorders associated with aPL area are probably the result of thrombotic events; however, there may be other aPL-mediated mechanisms, as well [3].

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Navarrete, M.G., Brey, R.L., Levine, S.R. (2000). Cerebral Disease in the Antiphospholipid Syndrome. In: Khamashta, M.A. (eds) Hughes Syndrome. Springer, London. https://doi.org/10.1007/978-1-4471-3666-8_5

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