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Magnetic resonance findings in leucodystrophies and MS.
[alexander disease]
White
matter
diseases
are
a
frequent
diagnosis
problem
in
adult
patients
.
They
are
divided
into
leucodystrophy
,
defined
by
abnormal
white
matter
from
the
beginning
,
and
leucoencephalopathy
,
with
an
initial
normal
white
matter
.
In
addition
,
two
different
natures
have
to
be
considered
:
vascular
and
non-
vascular
.
Vascular
diseases
are
mainly
acquired
and
related
to
atherosclerosis
.
Genetic
vascular
disorders
are
mostly
secondary
to
Notch
3
mutations
,
defined
as
cerebral
autosomal
dominant
arteriopathy
with
subcortical
infarcts
and
leucoencephalopathy
(
CADASIL
)
.
Occurrence
of
leucoaraiosis
and
lacunae
on
T
2
sequences
,
and
microbleeds
on
Gradient
Echo
sequences
,
strongly
suggest
this
diagnosis
.
Some
magnetic
resonance
(
MR
)
patterns
can
help
to
identify
genetic
leucodystrophies
,
such
as
childhood
ataxia
with
central
nervous
system
hypomyelination
/
leucoencephalopathy
with
vanishing
white
matter
disease
(
progressive
rarefaction
and
cystic
degeneration
of
the
affected
white
matter
,
replaced
by
water
)
;
Alexander
disease
(
hypointense
signals
on
T
2
sequences
involving
grey
matter
,
brainstem
and
cervical
cord
,
with
marked
atrophy
)
;
megalencephalic
leucoencephalopathy
with
subcortical
cysts
(
diffuse
,
symmetrical
white
matter
lesions
,
with
constant
frontoparietal
and
anterotemporal
subcortical
cysts
)
;
leucoencephalopathy
with
brainstem
and
spinal
cord
involvement
and
high
lactates
syndrome
(
extensive
demyelination
,
involvement
of
the
brainstem
,
i
.
e
.
cerebellar
peduncles
,
intraparenchymal
and
mesencephalic
trigeminal
nerves
and
spinal
cord
,
mainly
in
the
lateral
corticospinal
tracts
and
dorsal
columns
)
.
Half
of
the
genetic
adult
leucodystrophies
remain
without
any
precise
diagnosis
.
This
review
describes
MR
in
the
adult
leucoencephalopathies
and
in
multiple
sclerosis
(
MS
)
.
The
first
part
will
focus
on
MR
patterns
of
vascular
and
nonvascular
adult
leucoencephalopathies
,
the
second
part
on
MR
findings
in
MS
and
MS
-related
diseases
.
Specific
MR
patterns
in
both
diseases
will
be
summarized
and
compared
.
Diseases
Validation
Diseases presenting
"symmetrical white matter lesions"
symptom
alexander disease
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