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Efferent and afferent evoked potentials in patients with adrenomyeloneuropathy.
[adrenomyeloneuropathy]
This
paper
investigates
efferent
and
afferent
conductions
of
the
central
nervous
system
by
various
evoked
potentials
in
patients
with
adrenomyeloneuropathy
(
AMN
)
.
Ten
pure
AMN
patients
without
cerebral
involvement
were
studied
.
Motor
evoked
potentials
(
MEPs
)
,
somatosensory
evoked
potentials
(
SEPs
)
,
auditory
brainstem
response
(
ABR
)
,
and
pattern
reversal
full-field
visual
evoked
potentials
(
VEPs
)
were
recorded
.
For
MEP
recording
,
single
-pulse
or
double
-pulse
magnetic
brainstem
stimulation
(
BST
)
was
also
performed
.
Abnormal
MEP
was
observed
in
all
ten
patients
,
abnormal
SEP
in
all
ten
,
abnormal
ABR
in
nine
,
and
abnormal
VEP
in
only
one
.
Brainstem
latency
was
measured
in
three
of
the
seven
patients
with
central
motor
conduction
time
(
CMCT
)
prolongation
.
The
cortical
-brainstem
conduction
time
was
severely
prolonged
along
the
normal
or
mildly
delayed
brainstem-
cervical
conduction
time
in
those
three
patients
.
The
pattern
of
normal
VEP
and
abnormal
MEP
,
SEP
,
ABR
is
a
clinically
useful
electrophysiological
feature
for
the
diagnosis
.
BST
techniques
are
helpful
to
detect
,
functionally
,
intracranial
corticospinal
tract
involvement
,
probably
demyelination
,
in
pure
AMN
patients
.
Diseases
Validation
Diseases presenting
"central nervous system"
symptom
22q11.2 deletion syndrome
adrenomyeloneuropathy
alexander disease
aniridia
aromatase deficiency
canavan disease
child syndrome
classical phenylketonuria
congenital toxoplasmosis
cowden syndrome
cushing syndrome
cystinuria
dracunculiasis
erdheim-chester disease
fabry disease
gm1 gangliosidosis
hereditary cerebral hemorrhage with amyloidosis
hirschsprung disease
hodgkin lymphoma, classical
kabuki syndrome
kallmann syndrome
kindler syndrome
krabbe disease
lamellar ichthyosis
legionellosis
liposarcoma
malignant atrophic papulosis
monosomy 21
neonatal adrenoleukodystrophy
phenylketonuria
proteus syndrome
scrub typhus
severe combined immunodeficiency
sneddon syndrome
triple a syndrome
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
wiskott-aldrich syndrome
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
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