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[Antiphospholipid syndrome in neurology].
[sneddon syndrome]
Antiphospholipid
syndrome
(
APS
)
is
an
autoimmune
disease
with
recurrent
thromboses
and
pregnancy
complications
(
90
%
are
female
patients
)
that
can
be
primary
and
secondary
(
with
concomitant
autoimmune
disease
)
.
Antiphospholipid
antibodies
are
prothrombotic
but
also
act
directly
with
brain
tissue
.
One
clinical
and
one
laboratory
criterion
is
necessary
for
the
diagnosis
of
APS
.
Positive
serological
tests
have
to
be
confirmed
after
at
least
12
weeks
.
Clinical
picture
consists
of
thromboses
in
many
organs
and
spontaneous
miscarriages
,
sometimes
thrombocytopaenia
and
haemolytic
anaemia
,
but
neurological
cases
are
the
most
frequent
:
headaches
,
stroke
,
encephalopathy
,
seizures
,
visual
disturbances
,
Sneddon
syndrome
,
dementia
,
vertigo
,
chorea
,
balism
,
transitory
global
amnesia
,
psychosis
,
transversal
myelopathy
and
Guillain-
Barre
syndrome
.
About
50
%
of
strokes
below
50
years
of
age
are
caused
by
APS
.
The
first
line
of
therapy
in
stroke
is
anticoagulation
:
intravenous
heparin
or
low
-weight
heparins
.
In
chronic
treatment
,
oral
anticoagulation
and
antiplatelet
therapy
are
used
,
warfarin
and
aspirin
,
mostly
for
life
.
In
resistant
cases
,
corticosteroids
,
intravenous
immunoglobulins
and
plasmapheresis
are
necessary
.
Prognosis
is
good
in
most
patients
but
some
are
treatment-resistant
with
recurrent
thrombotic
events
and
eventually
death
.
Diseases
Validation
Diseases presenting
"stroke"
symptom
acute rheumatic fever
adrenomyeloneuropathy
alexander disease
alpha-thalassemia
cadasil
cohen syndrome
dedifferentiated liposarcoma
fabry disease
heparin-induced thrombocytopenia
hereditary cerebral hemorrhage with amyloidosis
homocystinuria without methylmalonic aciduria
hydrocephalus with stenosis of the aqueduct of sylvius
kallmann syndrome
locked-in syndrome
malignant atrophic papulosis
neuralgic amyotrophy
sneddon syndrome
thoracic outlet syndrome
werner syndrome
zellweger syndrome
This symptom has already been validated