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[Non-ischemic neurological manifestations in patients with primary antiphospholipid syndrome].
[sneddon syndrome]
Ischemic
disorders
of
brain
blood
circulation
caused
by
brain
artery
thrombosis
due
to
antiphospholipids-induced
anticoagulopathy
are
main
neurological
appearances
of
primary
antiphospholipid
syndrome
(
PAPS
)
.
A
number
of
neurological
disorders
in
patients
with
PAPS
are
the
result
of
primary
involvement
of
the
brain
and
peripheral
nervous
system
.
We
analyzed
the
spectrum
of
neurological
non-ischemic
PAPS
manifestations
in
125
patients
(
102
female
,
23
male
,
mean
age--
37
.
5
+
/
-
11
.
3
years
)
with
definite
PAPS
.
These
manifestations
included
headache
(
67
%
)
,
epileptic
seizures
(
23
%
)
,
chorea
(
15
%
)
,
optic
neuropathy
(
9
%
)
,
peripheral
neuropathy
(
6
%
)
,
multiple
sclerosis
like
syndrome
(
MSLS
)
(
8
%
)
,
acute
psychosis
(
2
%
)
,
myasthenic
syndrome
(
1
%
)
,
non-
vascular
parkinsonism
(
1
%
)
.
In
the
development
of
non-ischemic
PAPS
manifestations
,
antiphospholipids
as
well
as
other
antibodies
produced
as
a
result
of
immune
disregulation
(
antibodies
to
acetylcholine
receptors
in
myasthenic
syndrome
,
antineuronal
antibodies
in
MSLS
)
may
have
pathogenic
significance
.
In
some
cases
a
role
of
infection
involved
in
PAPS
manifestation
can
not
be
ruled
out
.
Diseases
Validation
Diseases presenting
"headache"
symptom
acute rheumatic fever
adrenal incidentaloma
allergic bronchopulmonary aspergillosis
cadasil
cushing syndrome
cutaneous mastocytosis
erdheim-chester disease
esophageal squamous cell carcinoma
familial mediterranean fever
hereditary cerebral hemorrhage with amyloidosis
hydrocephalus with stenosis of the aqueduct of sylvius
legionellosis
locked-in syndrome
malignant atrophic papulosis
scrub typhus
severe combined immunodeficiency
sneddon syndrome
systemic capillary leak syndrome
thoracic outlet syndrome
typhoid
von hippel-lindau disease
This symptom has already been validated
