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Adrenal disorders and the paediatric brain: pathophysiological considerations and clinical implications.
[cushing syndrome]
Various
neurological
and
psychiatric
manifestations
have
been
recorded
in
children
with
adrenal
disorders
.
Based
on
literature
review
and
on
personal
case-studies
and
case-series
we
focused
on
the
pathophysiological
and
clinical
implications
of
glucocorticoid-related
,
mineralcorticoid-related
,
and
catecholamine-related
paediatric
nervous
system
involvement
.
Childhood
Cushing
syndrome
can
be
associated
with
long
-lasting
cognitive
deficits
and
abnormal
behaviour
,
even
after
resolution
of
the
hypercortisolism
.
Exposure
to
excessive
replacement
of
exogenous
glucocorticoids
in
the
paediatric
age
group
(
e
.
g
.
,
during
treatments
for
adrenal
insufficiency
)
has
been
reported
with
neurological
and
magnetic
resonance
imaging
(
MRI
)
abnormalities
(
e
.
g
.
,
delayed
myelination
and
brain
atrophy
)
due
to
potential
corticosteroid-related
myelin
damage
in
the
developing
brain
and
the
possible
impairment
of
limbic
system
ontogenesis
.
Idiopathic
intracranial
hypertension
(
IIH
)
,
a
disorder
of
unclear
pathophysiology
characterised
by
increased
cerebrospinal
fluid
(
CSF
)
pressure
,
has
been
described
in
children
with
hypercortisolism
,
adrenal
insufficiency
,
and
hyperaldosteronism
,
reflecting
the
potential
underlying
involvement
of
the
adrenal
-
brain
axis
in
the
regulation
of
CSF
pressure
homeostasis
.
Arterial
hypertension
caused
by
paediatric
adenomas
or
tumours
of
the
adrenal
cortex
or
medulla
has
been
associated
with
various
hypertension
-related
neurological
manifestations
.
The
development
and
maturation
of
the
central
nervous
system
(
CNS
)
through
childhood
is
tightly
regulated
by
intrinsic
,
paracrine
,
endocrine
,
and
external
modulators
,
and
perturbations
in
any
of
these
factors
,
including
those
related
to
adrenal
hormone
imbalance
,
could
result
in
consequences
that
affect
the
structure
and
function
of
the
paediatric
brain
.
Animal
experiments
and
clinical
studies
demonstrated
that
the
developing
(
i
.
e
.
,
paediatric
)
CNS
seems
to
be
particularly
vulnerable
to
alterations
induced
by
adrenal
disorders
and
/
or
supraphysiological
doses
of
corticosteroids
.
Physicians
should
be
aware
of
potential
neurological
manifestations
in
children
with
adrenal
dysfunction
to
achieve
better
prevention
and
timely
diagnosis
and
treatment
of
these
disorders
.
Further
studies
are
needed
to
explore
the
potential
neurological
,
cognitive
,
and
psychiatric
long
-term
consequences
of
high
doses
of
prolonged
corticosteroid
administration
in
childhood
.
Diseases
Validation
Diseases presenting
"excessive replacement"
symptom
cushing syndrome
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