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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
"psychosis"
symptom
22q11.2 deletion syndrome
child syndrome
homocystinuria without methylmalonic aciduria
hydrocephalus with stenosis of the aqueduct of sylvius
sneddon syndrome
This symptom has already been validated