Sage Journals: Discover world-class research

Abstract

The primary antiphospholipid antibody syndrome (PAPS) has beendescribed in patients with a history of fetal loss,thrombocytopenia and arterial or venous thrombosis. In PAPS, a prothrombotic state is mediated by antiphospholipid antibodies (aPLs) leading to disseminated thromboembolic vascular occlusion. Today, the presence of aPLs in the serumis considered as a distinct risk factor for recurrent stroke in young adults. Some PAPS patients develop a multi-infarct-syndrome with a stepwise decline of higher cortical functions. We report on a 55-year-old man suffering from progressive dementia and PAPS, in whom cerebral glucose metabolism and blood flow were examined by positron emission tomograpy (PET). Cerebral atrophy and moderate signs of leukaraiosis were detected in magnetic resonance imaging (MRI), whereas the PET scans showed a considerable diffuse impairment of cortical glucose metabolism combined with a reduced cerebral perfusion in the arterial border zones. These findings indicate that PAPS-associated vascular dementia is accompanied 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 metabolism and blood flow in systemic lupus erythematosus (SLE).

Keywords

primary antiphospholipid antibody syndrome (PAPS)cerebral glucose metabolism regional cerebral blood flow multi-infarct-dementia (MID)positron emission tomography (PET)

Get full access to this article

View all access options for this article.

References

Hughes GRV . Thrombosis, abortion, cerebral disease and the lupus anticoagulant . BMJ 1983; 287: 1088 ‐ 1091 .

Hughes GRV . The anticardiolipin syndrome . Clin Exp Rheumatol 1985; 3: 285 ‐ 286 .

Harris EN , Hughes GRV , Gharavi AE . Antiphospholipid antibodies: an elderly statesmen Dons new garments . J Rheumatol 1987; 14 (Suppl 13): 208 ‐ 213 .

The Antiphospholipid Antibodies in Stroke Study Group (APASS) . Recurrent thromboembolic stroke risk in patients with neurological events and antiphospholipid antibodies (abstract) . Ann Neurol 1990; 28: 226–226 .

Briley DP , Coull BM , Goodnight SH . Neurological disease associated with antiphospholipid antibodies . Ann Neurol 1989; 25: 221 ‐ 227 .

Brey RL , Hart RG , Sherman DG et al. Antiphospholipid antibodies and cerebral ischemia in young people . Neurology 1990; 40: 1190 ‐ 1196 .

Kittner SJ , Gorelick PB . Antiphospholipid antibodies andstroke: an epidemiological perspective . Stroke 1992; 23 (Suppl 1): 119 ‐ 122 .

Levine SR , Welch KMA . The spectrum of neurologic diseaseassociated with antiphospholipid antibodies . Arch Neurol 1987; 44: 876 ‐ 883 .

The Antiphospholipid Antibodies in Stroke Study Group (APASS) . Clinical, radiological and pathological aspects of cerebrovascular disease associated with antiphospho-lipid antibodies . Stroke 1993; 24 (Suppl 1): 120 ‐ 123 .

10.

Hess DC . Stroke associated with antiphospholipid antibodies .Stroke 1992; 23 (Suppl 1): 123 ‐8 .

11.

Levine SR , Brey RL . Neurological aspects of antiphospholipid antibody syndrome . Lupus 1996; 5: 347 ‐353 .

12.

Kao CH , Ho YJ , Lan JL et al. Discrepancy between regional cerebral blood flow and glucose metabolism ofthe brain in systemic lupus erythematosus patients with normal brain magnetic resonance findings . ArthritisRheum 1999; 42: 61 ‐ 68 .

13.

Kao CH , Lan JL , ChangLai SP et al. The role of FDG-PET,HMPAOSPECT and MRI in the detection of brain involvement in patients with systemic lupus erythematosus . Eur J Nucl Med 1999; 26: 129 ‐ 134 .

14.

Maeshima E , Maeshima S , Yamada Y et al. Antiphospholipid antibodies and regional cerebral blood flow insystemic lupus erythematosus . Ryumachi 1993; 33: 125 ‐ 130 .

15.

Otte A , Weiner SM , Peter HH et al. Brain glucose utilization in systemic lupus erythematosus with neuropsychiatric symptoms: a controlled positron emission tomography study . Eur J Nucl Med 1997; 24: 787 ‐ 791 .

16.

Sabbadini MG , Manfredi AA , Bozzolo E et al. Central nervous system involvement in systemic lupus erythematosus patients without overt neuropsychiatric manifestations . Lupus 1999; 8: 11 ‐ 19 .

17.

Brandt JT , Triplett DA , Alving B et al. Criteria for the diagnosis of Lupus Anticoagulants: An update . Thromb Haemost 1995; 74: 1185 ‐ 1190 .

18.

Wienhard K , Dahlbohm M , Eriksson L et al. The ECAT EXACT HR:Performance of a new high resolution PET scanner . J Comp Ass Tom 1994; 18: 110 ‐ 118 .

19.

Wienhard K , Pawlik G , Herholz K et al. Estimation of local cerebral glucose utilization by positron emission tomography of 18F-2-fluoro-2-deoxy-D-glucose: A critical appraisal of optimization procedures . JCBFM 1985; 5: 115 ‐ 125 .

20.

Raichle ME , Martin WR , Herscovitch P et al. Brain bloodflow measurement with intravenous H215O. II. Implementation and validation . J Nucl Med 1983; 24: 790 ‐ 798 .

21.

Pietrzyk U , Herholz K , Fink G et al. An interactive technique for the three-dimensional image registration: validation for PET, SPECT, MRI and CT brain studies . J Nucl Med 1994; 35: 2011 ‐ 2018 .

22.

Brey RL , Coull BM . Antiphospholipid antibodies: origin,specifity, and mechanism of action . Stroke 1992; 23 (Suppl 1): I15 ‐ I18 .

23.

Petri M . Pathogenesis and treatment of the antiphospholipid antibody syndrome . Med Clin North Am 1997; 81: 151 ‐ 177 .

24.

Vargas-Alarcon , Granados J , Bekker C et al. Associationof HLA-DR5(possibly DDRB*1201g) with the primaryantiphosopholipid anti-body syndrome in Mexican patients .Arthritis Rheum 1995; 38: 1340 ‐ 1343 .

25.

Goldberg SN , Conti-Kelly AM , Greco TP . A family study of anticardiolipin antibodies and associated clinical conditions . Amer J Med 1995; 99: 473 ‐ 479 .

26.

Harmer IJ , Loizou S , Thompson KM et al. A human monoclonal antiphosopholipid antibody that is representative of serum antibodies and is germline encoded . Arthritis Rheum 1995; 38: 1068 ‐ 1076 .

27.

Cariou R , Tobelem G , Soria C et al. Inhibition of protein C activation by endothelial cells in the presence of lupus anticoagulant . N Engl J Med 1986; 314: 1193 ‐ 1194 .

28.

Feldmann E , Levine SR . Cerebrovascular disease with antiphospholipid antibodies: immune mechanisms, significance and therapeutic options . Ann Neurol 1995; 37 (Suppl 1): S114 ‐ S130 .

29.

Arfors L , Vestergvist O , Johnsson H et al. Increased thromboxane formation in patients with antiphospholipid syndrome . Eur J Clin Invest 1990, 20: 607 ‐ 612 .

30.

Lellouche F , Martinuzzo M , Said P et al. Imbalance of thromboxane/prostacyclin biosynthesis in patients with lupus anticoagulant . Blood 1991; 78: 2894 ‐ 2899 .

31.

Lin YL , Wang CT . Activation of human platelets by the rabbit anticardiolipin antibodies . Blood 1992; 80: 3135 ‐ 3143 .

32.

Lockshin MD . Pathogenesis of the antiphospholipid antibody syndrome . Lupus 1996; 5: 404 ‐ 408 .

33.

Simantov R , LaSala JM , Lo SK et al. Activation of cultured vascular endothelial cells by antiphospholipid antibodies . J Clin Invest 1995; 96: 2211 ‐ 2219 .

34.

Amengual O , Atsumi T , Khamashta MA et al. Antibodies against oxidized low-densitiy lipoprotein in antiphospholipid syndrome . Br J Rheumatol 1997; 36: 964 ‐ 968 .

35.

Kent M , Alvarez F , Vogt E et al. Monoclonal antiphosphatidylserine antibodies react directly with feline and murine central nervous system . J Rheumatol 1997; 24: 1725 ‐ 1733 .

36.

Turhim S , Rand JH , Goldbold JH et al. Elevated antiphosphatidylserine antibodies are a risk factor for ischemic stroke . Neurology 1998; 50: A246–A246 .

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

Abstract

Keywords

Get full access to this article

References