Full text of DOI:10.1177/088307380101600617

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Brusilow
Batshaw
Waber L: Neonatal
SW,
coma. Adv
ML,
hyperammonemic
and
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composition,
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Pediatr
1982;29:69.
nial
is not
intracranial
commonly
pressure.
Benign
hypertension
11.
12.
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ProblPediatr
Curr
Hyperammonemia.
1984;14:1-69.
associatedwith
include
corticosteroid
and
therapy. Symptoms
transientvisual
signs
Batshaw
ML,
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of
inborn
Waber
et al: Treatment
L,
errors
S,
blurred
obscurations,
headache, vomiting,
vision,
ofurea
Activation ofalternative
ofwaste
nitro-
synthesis.
pathways
1982;306:1387.
is
diplopia, andbilateral
Abducens
paresis
and
J Med
excretion.
Bock
dizziness,
a
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papilledema.
gen
N Engl
GH, et
comparison
13.
less commonbut
stud-
al: The
oflife
threat-
modal-
therapeutic
E,
Wiegand ThompsonT,
management
finding.’ Neuroimaging
lesion, hydrocephalus,
well-recognized
evidence of
A
ofseveral
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ening
ies show no
a
mass
or
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ities. JPediatr
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heliumas
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cause for the elevated
intracranial
pressure.3-5
14.
15.
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Med
a
new
ProcSoc
therapeuticgas. Exp
Biol
intracranial
hasbeendescribedinCrohn’s
Benign
disease but
hypertension
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Moller
in
and
in
isolated
association with stan-
only
reports
Budesonide is
low
systemic
NadsenJ: Protective effect of
of
and
O,
Hyldegaard
heliox
M,
oxygen
sick-
a
dard corticosteroid
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spinal decompression
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new-generation
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ness. Undersea
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Hyperb
corticosteroidthathas
alocal
potent
effect,
decreasedcorticosteroid-related
16.
Elleau
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et al:
H,
mixture in
C, Galperine RI,
distress
Helium-oxygen
and
This
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A
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double-blind
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122:132-136.
syndrome:
1993;
would
to
lead
besafer andcould to
in
increased use
chil-
appear
Oral
dren.
budesonidewasintroducedinIsraelin 1997.
17.
18.
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Kass
CastriottaRJ: Heliox
in acute severe
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early
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therapy
1999, all pediatric
contacted. Weidentified
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A
Madison
JM,
1995;107:597-598.
trial balloon. Chest
anddescribe three
with
patients
benign
intracranial
50 children with
estimated
amongthe
hypertension
Manthous
Hall
al: Heliox
et
improves
CA,
and
JB, Caputo MA,
pulsus
with
Crohn’sdiseasewhohadreceivedoral
modified-releasebudes-
pH
flow in nonintubated
paradoxus
severeasthma.Am
peak expiratory
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onide
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A,
disease.
respiratory
1995;332:192-193.
Theuse of
J Med
Case
Reports
lung
Shiue
N
Engl
Gluck EH:
with
mixtures in the
respiratory
ST,
of
helium-oxygen
sup-
acidosis. J
status
and
asthmaticus
Case 1
Case
Crohn’s disease who
port
Asthma
patients
1989;26:177-180.
1
is
a
with
disease
lb and
14-year-old
type
of
retro-orbital
girl
glycogen storage
22.
Gluck
bated
Onorato
with
with
a
2-week
CastriottaR.
mixturesinintu-
EH,
patients
presented
history
and oral budesonide
pain.
mg/dayfor
to admission
DJ,
Helium-oxygen
respiratory
statusasthmaticusand
Chest
acidosis.
She had
1
received mesalamine
9
daily
g
1990;98:693-698.
2
over
months. The latter was discontinued
1
week
prior
because of headache. On
stunted and
she was afebrile and
physical examination,
and her
and
werebelow the
height
appeared
third
wasted,
weight
for
mass
Herblood
was105/63
percentile
age(body
index, 15).
pressure
Intracranial
AssociatedWith
Benign
Hypertension
mm
The
liver was
10cmbelowthe costal
Hg.
palpated
Neurologic
margin.
Budesonide Treatment in
Disease
ChildrenWithCrohn’s
examinationdemonstrated
weaknessandbilateral
showednostructural
generalized
papilledema.
Contrasthead
mass
computedtomography(CT)
or
lesion,
brain
fluid
effect,
edema,
hydrocephalus. Cerebrospinal
openingpres-
surewas37cm
fluid analysisshowed
3
Cerebrospinal
monocytes/~,L,
was7.2
H 20-
ABSTRACT
of28
and
of94
Plasma
protein
mg/dL,
glucose
mg/dL.
hemoglobin
and mean
volume was 76 fL. White blood cells were
corpuscular
g/dL,
Oral
in
budesonide
adult studies is
a
corticosteroid with
potent
bioavailability andan
totalserumironwas17
andtotaliron
was
capacity
3300/~,L,
193
mg/dL,
binding
decreased
adverse
effect
improved
Serum
bloodureanitro-
systemic
mg/dL.
aminotransferases, bilirubin, ammonia,
were
within normal
range.
and vitamin
D
in
with
It has
been intro-
creatinine,
gen, electrolytes,
Albumin was 2.4
profile
comparison
prednisone.
recently
with furosemide was started because of
was administered
g/dL. Therapy
ducedforthe treatment of
boweldisease in
has
inflammatory
intracranial
Europe,
been
increased
intracranial
pressure. Methylprednisolone
andIsrael.
Canada,
Benign
corticosteroid
hypertension
rarely
Therewas
clinical
Theheadacheand
intravenously.
improvement.
withinweeks. Twomonthsafter
gradual
disappeared
neurologic complaints.
witha course of
with
associated
buthasnotbeen
in
therapy
reported
allbut
papilledema
admission,
Shehassincebeentreatedunevent-
associationwithbudesonide
adolescents
Three
with
Crohn’s
there
wereno
therapy.
nutritional status
disease and
intracranial
fully
prednisone.
poor
while
developed
benign
oral budesonide. All three
hypertension
patients
the
receiving
received
Case
2
had
coursesof
previously
multiple
without
prednisone during
disease
a2-week
history
a
with
boy
ofCrohn’s
Case
2
was
a
4-yearhistory
with
15-year-old
courseoftheir
intracranial
disease,
developing
resolved after
hypertension.
who
colon
the small boweland
presented
involving
of
intracranial
medication with-
hypertension
Benign
drawalanddidnotrecurwith
ear-
himfromsleep. Six weeks
awakened
severe frontal headache that
diar-
and
useof
Eval-
he
abdominal
hadsuffered fromintermittent
anorexia,
subsequent
prednisone.
lier,
pain,
a
and
formula
3
rhea and was started on budesonide
mg/tid
polymeric
uationfor
intracranial
shouldbe
in
considered
benign
with
hypertension
all ofwhich
andhe
and
inadditionto mesalamine
supplement,
6-mercaptopurine,
bowel
who
develop
be
effectmay
disease
headache
inflammatory
patients
while
had
he
Pain and
hadreceivedthe
diarrhea
resolved,
year.
previous
oralbudesonide. Thisside
associated
receiving
becauseofheadache.
had
His
to admission
discontinuedbudesonide
3
daysprior
with
poornutritional
status.
ChildNeurol
(J
2001;16:458-461).
the 3rd
below
were at the 25th and
and
percentiles,
examinations
height
weight
mass
and
Physical
index, 15.3).
neurologic
His blood
respectively (body
were normal
was
of
for
bilateral
pressure
except
papilledema.
examination
revealed
and
a
mm
113/70
12.0
Pertinent
hemoglobin
Hg.
albumin
laboratory
of3.3
Corticosteroids are
used for exacerbations of
Crohn’s
frequently
wereboth
D
A
levels
vitamin
Serum
and
g/dL
g/dL.
corticotropin
disease.1,2
increased
intracranial
is
characterized
hypertension
normal
Benign
cerebrospinal
adrenal
ruled out
by
An
test
and sinuses
the brain
normal.
intravenous
sup-
the
rapid
CT
scan of
was
fluid
A
contrast
fluid
normal;
pressure,
cerebrospinal
pression.
Downloaded from jcn.sagepub.com at Freie Universitaet Berlin on May 4, 2015

459
Theheadache
With
refused
a
lumbar
1.
Table
ConditionsAssociated
Intracranial
patient
Onemonth
puncture.
heconsentedto an
gradually disappeared.
after
elective lumbar
admission,
puncture;
Benign
Hypertension
was 14 cm
with 2
his
14
and
openingpressure
72
cells/ILL, protein
resolved overthenext
g/dL,
H20
2
months.
Papilledemagradually
mg/dL.
glucose
Hehas sincebeentreated
withseveralcourses
uneventfully
of prednisone.
Case 3
Case3,
bowelCrohn’s
a
a
with
of
predominantly
small
13-year-old
boy
6-yearhistory
wasadmittedfor
and
severeheadache
vomiting. He
disease,
course
hadfinished
a
for an
2
months
to
exacerbation
of prednisone
prior
at whichtime hehad been
admission,
additionto mesalamineand
on
9
budesonide
in
placed
mg/day,
whichhewas
azathioprine,
werebothwellbelowthe3rd
alreadyreceiving.
mass
and
(body
index,
Blood
papilledema.
Weight
18.5).
height
percentile
for
examination was normal
Physical
except
was 13.4
mm
andalbuminwas
was 100/60
pressure
g/dL,
Hg. Hemoglobin
3.8
Serum iron andvitamin and Dlevels were normal.
A
Computed
g/dL.
tomographyof
Cerebrospinal
glucose
open-
thebrainand
H20,
sinuseswasnormal.
fluid
was28cm
was52
fluid
and
ingpressure
was 23
cerebrospinal
mg/dL,
with
1
He received acetazolamide with
protein
mg/dL,
cell/ILL.
Papilledema
was slow to resolve.
symptomatic improvement.
Magnetic
resonance
ofthe brain wasnormal.
imaging
Discussion
with
factors have been associated
Many
benign
predisposing
in
Acute and
intracranial
the
pediatric population.
hypertension
endocrine
and
chronic
obesity
diseases,
disorders, medications,
intracranial
haveall beenlinkedto
benign
inmostcases,
hypertension3-13(Table
factorcannotbedis-
a
Nevertheless,
1).
predisposing
cerned.5,8 Corticosteroid
hasalso beendescribedin asso-
therapy
ciation with
which
occur
intracranial
hypertension,
may
benign
active treatment or
medicationwithdrawal
during
during
with
diseaseand
bowel
inflammatory
We
three
present
intracranial
patients
that
oralbudes-
hypertension
lumbar
benign
onide
developedduring
not
on
a
was
thrombosisandvasculitis. 17
intracranial
casesof
bowel
therapy. Although
the
puncture
onsetofacute
the
performed
However,previouslyreported
in
2
severe
case
acute
persistent
during
episode,
resolvingpapilledema,
normalcontrastCTscanmakesthis
inflammatory
benign
disease
hypertensionoccurring
headachewith
normalblood
and
Both
beenrelatedtocorticosteroid
Liuet alps
have
pressure,
therapy.
cases
a
andNewtonand
described
dur-
diagnosis likely.
factors couldbe relatedtothe
Cooper’
multiple
occurring
in
with
or
Multiple
of
appear-
inflam-
inflammatory
of
confounding
intracranial
ing prednisone therapy
bowel
taper
one
patients
with
with
ance
in
benign
hypertension
patients
multiple
episodes
disease, including
patient
bowel
suchas iron
anemiaorvitamin
on corticosteroid withdrawal.
intracranial
disease,
Tvvoofour
matory
D
deficiency
werenot
hypertension
were
benign
at
at the
time.
anemic
Noneofthese
mesalamine
deficiency.
patients
levels were
presentation,
patients
receiving
andvitamin
D
in
Wecannotexclude
factorfor
normal
all three.
it is
that our
Hence,
very likely
patients developed benign
iron
anemiaas
a
the
intracranial
due to
corticosteroid
deficiency
contributingetiologic
in
benign
therapy they
hypertension
at the
1.
intracranial
case
were
budesonide. Oral budesonide
corticosteroidwithalow of10%.
potent
hypertension
receiving
time, namely
A
can occur dueto vitamin
is
a
Hypervitaminosis
the
supplementa-
systemic
bioavailability
during first-pass metabolism,
had
tion.
a
It is
its
and
metabolized
only patient
However,
receiving supplementation
efficiently
A
level. Adrenal
due
vitamin
to
normal serum
are
it is often considered
These
characteristics allow
a
a
insufficiency
corticotropin
pitu-
stimulation
by-products
form of
nearlyinactive; therefore,
wasexcluded
a
intestinal
by
itary-axis depression
topical
side-effect
therapy,.’
in
this an
in two of the
test
with standard
cortico-
unlikely
patients, making
mimicCrohn’sdiseaseandcause
can
superior
steroid
performed
mechanism.
comparison
profile
disease
Thishasbeenvalidated in several
studies,. 18,19
Behqet’s
therapy.
It
is unclear
criteria
such
a
intracranial
corticosteroid with low
intracranial
however, diagnostic
hypertension;
or
why topical
benign
as
systemic
An
wereabsentin our
cause
patients
pres-
ulcers,
pathergy
bioavailability
might
increase in
unexplained
or difference in
uveitis,
genital
benign
hypertension.
werethe
inthis
in all
Commondenominators
decrease
in
study
patients
absorption,
first-pass effect,
increased
withmesalamineandbudes-
andtreatment
enceof
disease
could
all
Crohn’s
patient population
explain
in the
had received
onide. All three
without
seem
patients
Althoughthis may
inconsistentwiththefact
prednisone
past
bioavailability.
that
in
none were
the withdrawal
and
side effects were
not
and the
present
headache,
glucocorticoid
developing
pitu-
they
axis
ofsteroid
was not
it is not
that
clear
itary-adrenal
phase
therapy.
suppressed,
be
arisk
factorfor
could
evident
itself
needto be
Crohn’s disease
corticosteroid-induced
intracra-
the-
theoretically
during
Case had
benign
It hasbeenassociatedwith
cen-
nial
1
intracranial
concurrentliver
hypertension.
manifestations
hypertension.
disease,
benign
which,
venous
andarterial
have
tral nervous
altered
metabolism.
including
oretically, may
system
first-pass
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460
One
the
haveto dowith
may
Lerman-Sagie, MD
possible
explanation
patientpop-
Tally
ulation
treated. Our
had
were adolescents and
had
Unit
Pediatric
patients
Two
signifi-
Neurology
Medical
low
for
status
Budesonide
than
Holon
poornutritional
Center,
E.
cantly
weight
age.
patients
Wolfson
at
is
theonset
oftreatmentandwere
TelAviv
hypoalbuminemic.
more
University
boundto
albuminand
is considerably
lipophilic
SacklerSchool
pred-
of Medicine
nisone. In the
the volume of dis-
of
Tel
Israel
presence
have
hypoalbuminemia,
thus to
Aviv,
tribution
may
decreased,
leading
higher availability
Received
lication
2000. Received revised
2000.
for
pub-
5,
20,
April
Sept
Sept 19,
Accepted
for
central
less
has
be
may
nervous
system
Although
budesonide
penetration.
2000.
absorbed
corticoid
a
into the
systemic circulation,
21 times
gluco-
methyl-
concentration is
Dr
Address
to
Nathan
Pediatric
Watemberg,
correspondence
E. WolfsonMedical
Neurology
972-3-502-8458;
than that of
receptor affinity
greater
receptor
glucocorticoid
POB
5
Israel. Tel:
Center,
Holon,
Unit,
fax:
e-mail:
andthe
972-3-502-8566;
nwatemberg@pol.net.
prednisolone,2°
inthe
ifany, betweencorticosteroid
brain.2’ The
high
doseand
relationship,
intracranial
is unclearat
present. 1,7
hypertension
benign
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The
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cerebri of
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no
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protein
measles,
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central nervous
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themid-
onance
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a
thebrainshowed
signal involving
hyperintense
imaging
andlenticular
anterior
caudate,
commissure, thalami,
brain, hypothalamus,
ini-
nuclei
onthe fluid-attenuated
inversion-recoverysequence,
bilaterally
as an infiltrative
glioma
tially interpreted
Thepatient
(Figure 1).
andintravenous
Acute
Basal
as
a
NecrotizingEncephalopathyPresenting
Ganglia Syndrome
antibiotics
with
wastreated
pend-
brain
acyclovir
A
stereotactic
results of
with noclinical
improvement.
ing
cultures,
cell and
showed
tissue with chronic
inflammatory
therapeutic
biopsy
hemorrhagic
were seen or
A
trial with
cultured.
No
microglia.
organisms
ABSTRACT
improvement in mental
with
a
corticosteroids was associated
significant
ability
to
forherself. Herclin-
care
levelof
andthe
status,
ical
arousal,
patient’s
mild
left
Acute
is
a
newdisease. The
necrotizing
encephalopathy
relatively
choreiform
coursewas
marked
a
hemiparesis,
movements,
by
characteristic clinical
areoffebrile
illness followed
findings
byrapid
and
motions
morethanright),
restlessness,
jerky
and
(left
difficultyconcentrating
with
deteriorationinmentalstatusandseizures. Thehallmarkofthe
butno
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commands
processingsequential
difficulty
computations.
and
featureswere
pres-
Echolalia, preservativespeech,
obsessive-compulsive
ease is
mus,
multifocal bilateral
lesions
the thala-
symmetric
brainstem
affecting
ent. Shewasdischargedhomeon
a
withinstructions
prednisone day
40-mg
cerebralwhite
hypothalamus,
matter,
tegmentum,
for
ofthemedication. Ondiscontinuationof
corticosteroids,
the MRI
findings
gradualtaper
andcerebellum. The
is
butimmune-mediated
etiology
unknown,
her behavior
showed
deteriorated
even
dramatically,
resolution. She
though
mechanism
was
We
a
present
suggested.
12-year-old previously
tostu-
in
an increase
developed
involuntary
progressive
who
increased
choreiform
severe
healthy
developed
sleepiness
and jerkymovements,
auditoryhallucinations,
girl
agitation,
judgment
psychiatric
progressing
out
behavior,
striking
and
and
aggressive
poor
hyper-
insight,
and coma.
resonance
of the brain
por
Magnetic
imaging (MRI)
in
a
hospitalization
sexuality
requiring
facility.
showedthe characteristic
describedin acute
findings previously
Hermental status
Atherapeutic
trial withintravenous
IgG
She was treated with
for
5
showedno
days
change
and
dramat-
necrotizing encephalopathy.
with steroid
improved
in
risperidone. Clonazepam
symptomatology.
andthe MRIfindingsresolved com-
ically
treatment,
not
control
did
her
the
guanfacinehydrochloride
fully
symptoms,
discontinuedafter months.
prompting
within
6
months.
theacuteillness, she
restartofcorticosteroids, whichwere
6
pletely
a
Following
disorderconsistent
developed
eventually
withbasal
complexneuropsychiatric
ChildNeurol
ganglia
Discussion
syndrome.
(J
2001;16:461-462).
Acute
ofchildhoodis
a
necrotizing encephalopathy
newlyproposed
distributed brain
disease
characterized
entity
by symmetrically
Wedescribe
sented with
in
a
a
ofa
case
whopre-
culminat-
ofthe
previouslyhealthy
of
12-year-oldgirl
3-week
history
progressive sleepiness
resonance
andcoma.
stupor
ing
Magnetic
imaging(MRI)
the
brainshowed
a
hyperintense
signal involving
midbrain, hypo-
and
lenticularnuclei
This
thalamus, thalamus,
was
caudate,
describedas
bilaterally.
characteristicofacutenecro-
picture
previously
a
newdisease
encephalopathy,
tizing
et al in 1995.’ Stereotactic
entity
proposedbyMizuguchi
caudate inflam-
ofthe
showed
biopsy
Theclinical outcomewasconsistentwith
a
neu-
matorychanges.
manifested
transient
ropsychiatric syndrome
by
hemiparesis,
involuntary movements,
chorea,
preservative speech,
atten-
jerky
obsessive-compulsive symptoms, poor insight,
echolalia,
and
tion deficit andhyperactivity,
Such
impulsiveness, poor
judgment,
described as basal
were
recently
the acute illness
symptoms
hypersexuality.
with
improved
Although
ganglia syndrome.2
MRI
and the
the behavioral
steroid
treatment
resolved,
findings
ofbasal
characteristic
syndrome
ganglia
persist.
problems
Case
A12-year-old
Report
withno
medical
African-Americangirl
past
significant
progressively
history
withmildfeverand
diarrhea,
duration, prompting
increasingsleepiness,
presented
and
heradmission
to the
of
3
weeks’
was
tiredness
tal. On
hospi-
stim-
but
arousable to
brisk
she
afebrile, sleepy,
painful
Axial fluid-attenuated
confluent
from
admission,
brain
within the
midbrain,
Figure 1.
inversion-recovery
image
bilateral
MRI
revealed
and
few
uli. The examination
ptosis,
stupor
reflexes, ataxia,
revealing
hyperintense
signal
and
thalami, caudate,
lentiform nuclei
to
andcoma
overthenext
deteriorated
rapidly
She
hypothalamus,
bilaterally.
retropulsion.
Downloaded from jcn.sagepub.com at Freie Universitaet Berlin on May 4, 2015