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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
"progressive abnormalities in leg"
symptom
adrenomyeloneuropathy
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