Background and Purpose-The aim of our study was to characterize the pa
tient profile and prognostic value associated with high positive IgG (
>100 GPL) anticardiolipin antibodies (aCL). Methods-We studied the cli
nical, laboratory, radiological, and prospective historical features o
f ischemic cerebrovascular disease in patients with >100 GPL titers. F
rom our neurology department, 27 consecutive patients were prospective
ly identified and followed up (mean follow-up time, 34 months). Result
s-The mean age of our cohort was 41 years. Lupuslike illness occurred
in 3; 23 had primary antiphospholipid syndrome, including 3 who met cr
iteria for Sneddon's syndrome; 1 patient had progressive systemic scle
rosis. Cerebral infarcts occurred in 74% and were recurrent in 37%. Sy
stemic ischemic events, most commonly deep vein thrombosis, occurred i
n 37%. Tobacco use was documented in 85%, hyperlipidemia in 74%, hyper
tension in 44%, and diabetes mellitus in 7% of patients. A prominent h
eadache history was present in 67%. Lupus anticoagulant (LA) was prese
nt in 72%, approximately one half had positive antinuclear antibodies
and thrombocytopenia, and one quarter had a false-positive VDRL. We co
mpared mean GPL levels in patients testing positive for specific labor
atory features of antiphospholipid syndrome with those testing negativ
e for these parameters. Only the LA(+) group had a significantly highe
r mean GPL than the LA(-) group (P=0.006). Brain imaging showed nonlac
unar infarcts in 73% and lacunes in 12%. Of 19 cerebral angiograms, 5
(26%) showed large-vessel occlusive disease and 6 (32%) branch obstruc
tion. Echocardiograms were abnormal in 75%: thickened left-sided valve
s in 33% and vegetations in 12%. Recurrent cerebrovascular ischemic ev
ents were observed in 96%, with transient events (mean rate, 25%/y) oc
curring 5 times more frequently than strokes (mean rate, 5%/y). Using
a standardized disability scale blinded to aCL titer, neurological imp
airment was severe in 7%, moderate in 30%, and mild or nonexistent in
63%, and unrelated to mean GPL value (P=0.567). Titers fluctuated grea
tly for individual patients, and most did not consistently test as hig
hly positive. An analysis of fluctuation in symptom severity with conc
urrent GPL values did not show a statistically significant correlation
. Compared with historical controls having a wide range of positive ti
ters, the presence of high IgG aCL titers did not confer a worse progn
osis for disability and recurrent ischemic events. Conclusions-Our dat
a suggest that cerebrovascular events associated with high positive GP
L are frequently multiple and minor (with no disability-titer correlat
ion), present in relatively young patients, and often associated with
tobacco abuse, hyperlipidemia, LA, systemic ischemic events, and occul
t cardiac disease.