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Sensorimotor function and axonal integrity in adrenomyeloneuropathy.
[adrenomyeloneuropathy]
Gait
abnormalities
and
sensorimotor
disturbances
are
principal
defects
in
adrenomyeloneuropathy
(
AMN
)
.
However
,
to
our
knowledge
,
their
association
with
overall
impairment
and
neuroanatomical
changes
has
not
been
defined
.
To
understand
how
sensorimotor
impairments
create
mobility
deficits
and
to
analyze
how
these
impairments
are
related
to
specific
metrics
of
axonal
integrity
.
Cross-sectional
study
assessing
impairments
,
including
vibration
sensation
,
strength
,
spasticity
,
and
global
measures
of
walking
and
balance
.
Fractional
anisotropy
was
measured
to
evaluate
the
integrity
of
the
corresponding
brainstem
tracts
.
Men
with
AMN
and
healthy
control
subjects
.
Individuals
with
sensory
loss
only
showed
minimal
walking
deficits
.
Concomitant
strength
and
sensory
loss
resulted
in
slower
walking
,
with
abnormal
knee
control
;
increased
spasticity
led
to
an
exaggerated
trunk
motion
and
a
knee
-flexed
(
crouched
)
posture
.
Hip
strength
was
an
independent
predictor
of
walking
velocity
in
subjects
with
AMN
.
Subjects
with
sensory
loss
only
had
greater
sway
amplitudes
during
standing
balance
testing
,
which
did
not
worsen
with
additional
impairments
.
There
were
significant
associations
among
sway
amplitude
,
great
toe
vibration
sense
,
and
dorsal
column
fractional
anisotropy
.
Brainstem
fractional
anisotropy
in
AMN
was
significantly
negatively
correlated
with
impairment
,
indicating
that
overall
tract
integrity
is
associated
with
sensorimotor
abnormalities
in
AMN
.
Impairment
measures
capture
specific
abnormalities
in
walking
and
balance
that
can
be
used
to
direct
rehabilitation
therapy
in
AMN
.
Tract-
specific
magnetic
resonance
imaging
metrics
,
such
as
fractional
anisotropy
(
used
herein
to
evaluate
structure-function
relationships
)
,
significantly
reflect
disease
severity
in
AMN
.
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