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Strength: a relevant link to functional performance in the neurodegenerative disease of adrenomyeloneuropathy.
[adrenomyeloneuropathy]
With
progressive
abnormalities
in
leg
strength
,
tone
,
and
sensation
,
adrenomyeloneuropathy
(
AMN
)
is
a
differential
diagnosis
for
multiple
sclerosis
and
hereditary
spastic
paraparesis
.
AMN
pathology
has
been
linked
to
weakness
,
making
it
a
relevant
model
to
evaluate
the
relationship
between
neurodegeneration
and
disability
.
Quantifying
symptom
severity
in
AMN
is
essential
for
treatment
development
in
rehabilitative
management
.
To
identify
deficits
in
body
functions
,
activity
,
and
participation
of
people
with
AMN
and
provide
a
practical
framework
for
evaluating
the
severity
of
disability
.
Cohort
analysis
of
142
participants
with
AMN
.
of
body
functions
(
leg
strength
,
vibration
sensation
,
range
of
motion
,
and
spasticity
)
,
activity
(
walk
velocity
,
standing
balance
,
Timed
Up
and
Go
,
and
Sit-
to
-
Stand
Time
)
,
and
participation
(
6
-
Minute
Walk
)
are
evaluated
.
Regression
analyses
identify
relationships
between
the
measures
.
A
staging
framework
(
mild
,
moderate
,
and
severe
)
reflects
the
continuum
of
disability
.
Finally
,
an
analysis
of
variance
/
Kruskal-
Wallis
was
used
for
between-
stage
and
sex
differences
among
the
variables
.
Strength
is
the
strongest
correlate
for
the
5
measures
of
activity
and
participation
.
Staging
based
on
weakness
distinguishes
3
levels
of
severity
along
a
continuum
of
disability
.
Differences
between
the
sexes
are
more
prevalent
earlier
in
the
continuum
but
show
equally
severe
deficits
in
the
last
stage
.
In
AMN
,
staging
based
on
degrees
of
weakness
provides
a
practical
means
to
characterize
the
severity
of
common
deficits
in
body
functions
as
well
as
activity
and
participation
at
each
stage
,
to
direct
the
evaluation
.
Such
information
could
help
clinicians
develop
more
effective
rehabilitative
techniques
.
Diseases
Validation
Diseases presenting
"spasticity"
symptom
adrenomyeloneuropathy
alexander disease
congenital toxoplasmosis
gm1 gangliosidosis
homocystinuria without methylmalonic aciduria
hydrocephalus with stenosis of the aqueduct of sylvius
krabbe disease
lamellar ichthyosis
neonatal adrenoleukodystrophy
pyruvate dehydrogenase deficiency
x-linked adrenoleukodystrophy
zellweger syndrome
This symptom has already been validated