Rare Diseases Symptoms Automatic Extraction
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A random Abstract
Our Project
Our Team
A survey on self-assessed well-being in a cohort of chronic locked-in syndrome patients: happy majority, miserable minority.
[locked-in syndrome]
Objectives
Locked-
in
syndrome
(
LIS
)
consists
of
anarthria
and
quadriplegia
while
consciousness
is
preserved
.
Classically
,
vertical
eye
movements
or
blinking
allow
coded
communication
.
Given
appropriate
medical
care
,
patients
can
survive
for
decades
.
We
studied
the
self-reported
quality
of
life
in
chronic
LIS
patients
.
Design
168
LIS
members
of
the
French
Association
for
LIS
were
invited
to
answer
a
questionnaire
on
medical
history
,
current
status
and
end-of-life
issues
.
They
self-assessed
their
global
subjective
well-being
with
the
Anamnestic
Comparative
Self-
Assessment
(
ACSA
)
scale
,
whose
+
5
and
-
5
anchors
were
their
memories
of
the
best
period
in
their
life
before
LIS
and
their
worst
period
ever
,
respectively
.
Results
91
patients
(
54
%
)
responded
and
26
were
excluded
because
of
missing
data
on
quality
of
life
.
47
patients
professed
happiness
(
median
ACSA
+
3
)
and
18
unhappiness
(
median
ACSA
-
4
)
.
Variables
associated
with
unhappiness
included
anxiety
and
dissatisfaction
with
mobility
in
the
community
,
recreational
activities
and
recovery
of
speech
production
.
A
longer
time
in
LIS
was
correlated
with
happiness
.
58
%
declared
they
did
not
wish
to
be
resuscitated
in
case
of
cardiac
arrest
and
7
%
expressed
a
wish
for
euthanasia
.
Conclusions
Our
data
stress
the
need
for
extra
palliative
efforts
directed
at
mobility
and
recreational
activities
in
LIS
and
the
importance
of
anxiolytic
therapy
.
Recently
affected
LIS
patients
who
wish
to
die
should
be
assured
that
there
is
a
high
chance
they
will
regain
a
happy
meaningful
life
.
End-of-life
decisions
,
including
euthanasia
,
should
not
be
avoided
,
but
a
moratorium
to
allow
a
steady
state
to
be
reached
should
be
proposed
.
Diseases
Validation
Diseases presenting
"anxiety"
symptom
22q11.2 deletion syndrome
achondroplasia
adrenal incidentaloma
adrenomyeloneuropathy
cadasil
child syndrome
classical phenylketonuria
cohen syndrome
congenital adrenal hyperplasia
congenital toxoplasmosis
cutaneous mastocytosis
fabry disease
familial hypocalciuric hypercalcemia
familial mediterranean fever
gm1 gangliosidosis
hereditary cerebral hemorrhage with amyloidosis
junctional epidermolysis bullosa
locked-in syndrome
monosomy 21
oligodontia
oral submucous fibrosis
phenylketonuria
von hippel-lindau disease
x-linked adrenoleukodystrophy
This symptom has already been validated