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Rheumatology Advance Access published online on September 14, 2004
Rheumatology, doi:10.1093/rheumatology/keh408
Rheumatology © British Society for Rheumatology 2004; all rights reserved
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Received July 8, 2004
Accepted August 10, 2004

Concise Report

Dementia associated with the antiphospholipid syndrome: clinical and radiological characteristics of 30 patients

J. A. Gómez-Puerta 1, R. Cervera 1*, L. M. Calvo 1, B. Gómez-Ansón 2, G. Espinosa 1, G. Claver 1, S. Bucciarelli 1, A. Bové 1, M. Ramos-Casals 1, M. Ingelmo 1, and J. Font 1

1 Department of Autoimmune Diseases, Institut Clínic de Medicina i Dermatologia, Barcelona, Catalonia, Spain
2 Department of Neuroradiology, Centre Clínic de Diagnòstic per la Imatge, Hospital Clínic, Barcelona, Catalonia, Spain

* To whom correspondence should be addressed. E-mail: rcervera{at}clinic.ub.es.


  Abstract

Objective. To analyse the clinical and radiological characteristics of patients with dementia associated with the antiphospholipid syndrome (APS).

Methods. Twenty-five patients were identified by a computer-assisted (MEDLINE, National Library of Medicine, Bethesda, MD) search of the literature to locate all cases of dementia associated with APS published in English, Spanish and French from 1983 to 2003. Additionally, we included five patients from our clinics.

Results. There were 21 (70%) females and 9 (30%) males. The mean age of patients was 49±15 yr (range 16-79 yr). Fourteen (47%) of the patients suffered from primary APS, 9 (30%) had systemic lupus erythematosus and 7 (23%) had ‘lupus-like’ syndrome. Ten (33%) patients had Sneddon's syndrome and 2 (7%) had cerebral lesions described as Binswanger's disease. Other APS-related manifestations included thrombocytopenia in 12 (40%) patients, cerebrovascular accidents in 11 (37%), heart valve lesions in 8 (27%), deep vein thrombosis in 7 (28%), migraine in 7 (23%), seizures in 4 (13%); five of the 21 (24%) female patients had nine spontaneous abortions. Lupus anticoagulant was present in 21/29 (72%) patients and anticardiolipin antibodies were present in 24/29 (83%) patients. Cortical infarcts were found in 19 (63%) patients, subcortical infarcts in 9 (30%), basal ganglia infarcts in 7 (23%) and signs of cerebral atrophy in 11 (37%). Anticoagulation was used in 14/25 (56%) patients, steroids in 12/25 (48%), aspirin in 6/25 (24%) and dypiridamole in 5/25 (20%).

Conclusions. Dementia is an unusual manifestation of APS but one which has a high disability impact in a patient's daily life. In order to prevent these consequences, an echocardiographic and cerebral CT or MRI evaluation are recommended in all patients with APS. Furthermore, ruling out APS should be recommended in the clinical approach to dementia, especially in young patients.

Keywords: Antiphospholipid syndrome; Dementia; Vascular dementia; Multi-infarct dementia; Sneddon's syndrome; Binswanger's disease.
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J. A. Gomez-Puerta, R. Cervera, L. M. Calvo, and J. Font
Dementia associated with antiphospholipid antibodies: reply
Rheumatology, February 1, 2006; 45(2): 242 - 243.
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 Rheumatology (Oxford)Home page
J. C. Adair
Dementia associated with antiphospholipid antibodies
Rheumatology, February 1, 2006; 45(2): 241 - 242.
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