1. John
Martinelli,
MSIII,
SGUSOM
DATE:
9/28/13
Pediatrics,
Case
2:
SLE/Pseudotumor
Cerebri
Identifying
Data:
M.M.
is
a
17
year
old,
English
speaking,
African-­‐American
female
who
presented
to
the
SBMC
ED
with
her
mother
on
the
afternoon
of
9/25/13.
She
was
admitted
to
the
SBMC
pediatric
floor
on
the
same
day
where
she
continues
to
be
treated.
DOB:
2/2/96
Chief
Complaint:
Upon
presentation
to
the
ED,
M.M.
stated
she
recently
began
experiencing
a
headache,
now
worsening
to
a
point
in
which
she
cannot
function.
She
described
the
headache
as
“being
all
over”.
History
of
Present
Illness:
At
admission,
M.M.’s
symptoms
were
consistent
with
chronic
severe
non-­‐localized
headache
which
she
rated
10/10
for
pain.
The
episode
had
been
variable
for
approximately
7
days,
with
increasing
intensity
beginning
3
days
prior
to
admission,
prompting
her
visit
to
the
ED.
She
described
the
headache
as
beginning
at
the
back
of
her
neck
then
progressively
radiating
to
her
entire
head.
The
initial
pain
was
characterized
as
stabbing
and
fluctuating
anywhere
from
5/10
–
10/10
until
it
became
steady
leading
her
to
seek
care.
She
also
reported
feeling
fatigue
and
dizziness
during
this
same
7-­‐day
period.
In
addition,
she
noticed
increased
sensitivity
to
both
light
and
sound
that
is
somewhat
relieved
in
a
dark
quiet
room.
She
has
occasionally
experienced
similar
prior
headaches,
however,
they
were
of
short
1-­‐2
hour
duration
and
resolved
without
treatment.
M.M.
also
had
nausea
and
vomiting
beginning
2
days
prior
to
her
presentation.
On
the
day
of
her
ED
visit
and
admission,
her
nephrologist
examined
her
as
an
outpatient.
He
found
her
blood
pressure
to
be
low
for
which
he
discontinued
her
anti-­‐hypertensive
medication
and
also
recommended
her
going
to
the
ED.
She
denies
any
recent
head
trauma,
altered
mental
status,
confusion,
infection,
fever,
numbness,
tingling,
or
coordination/balance
issues.
She
recently
returned
from
a
camping
trip
but
does
not
recall
any
insect
bites
or
unusual
rashes.
She
also
does
not
recall
any
sick
contacts
over
the
last
several
weeks.
Past
Medical
History:
M.M.
has
a
history
of
systemic
lupus
erythematosus
(SLE),
which
was
first
diagnosed
in
2008.
Her
initial
presentation
was
post-­‐generalized
tonic/clonic
seizure
(GTC),
which
eventually
led
to
the
diagnosis
of
SLE
with
central
nervous
system
(CNS)
involvement.
The
seizures
were
described
with
frothing,
incontinence,
and
abnormal
eye
movements
lasting
approximately
3
minutes,
with
postictal
confusion.
Soon
afterwards,
she
subsequently
had
two
additional
GTC
seizures.
At
that
time,
she
was
placed
on
SLE
therapy
along
with
an
anti-­‐epileptic.
She
has
not
experienced
any
seizure
activity
since
2008;
however,
she
does
notice
occasional
intermittent
blurred
vision.
In
2008,
she
was
also
diagnosed
with
SLE
membranous
glomerulonephritis
and
related
hypertension,
calcium
deficiency,
as
well
as
bicarbonate
depletion.
She
did
require
dialysis
at
that
time.
In
addition
to
her
primary
SLE
therapy,
she
has
been
well
controlled
on
oral
antihypertensives,
as
well
as
vitamin
and
bicarbonate
supplementation.
In
2012,
she
did
require
hospitalization
for
pneumonia
that
was
successfully
treated.
In
2002,
she
had
a
tonsillectomy
and
adenoidectomy
with
a
normal
post-­‐operative
course.
M.M.
was
born
premature
at
30
weeks
by
spontaneous
vaginal
delivery.
Her
mother
states
she
was
not
advised
as
to
the
reason
for
her
early
delivery.
M.M.
did
require
approximately
1
month
of
NICU
care
in
which
she
received
initial
O2
therapy
as
well
as
phototherapy
for
minimal
early
jaundice.

2. During
her
NICU
admission,
there
were
no
complications
and
she
was
subsequently
discharged
for
routine
care.
Her
mother
states
M.M.’s
immunizations
were
always
kept
current
and
are
up
to
date.
Medications:
Current:
Cellcept
(mycophenolate
mofetil):
300mg
suspension
PO
BID
Cozaar
(Losartan):
100mg
tab
PO
QD
Keppra
(Levetiracetam):
250mg
tab
PO
BID
Prozac
(Fluoxetine):
10mg
cap
PO
HS
Plaquinil
(Hydroxycholoroquine):
200mg
tab
PO
Q12H
Rocaltrol
(Calcitriol):
0.25mcg
liquid
solution
PO
HS
Vitamin
D2:
50,000
IU
cap
PO/week
Sodium
Bicarbonate:
650mg,
3
tabs,
PO
Q12H
Upon
Admission:
½
NS
1000ml:
75ml/hr,
IV.
Current
medications
as
IV
equivalents
except
plaquinil
and
sodium
bicarbonate
remain
PO.
Procardia
XL
(nifedipine):
60mg
tab
PO
QD.
Famotidine:
20mg,
2ml,
IV
push
Q12H.
Ondansetron:
4mg,
2ml,
IV
push
Q8H.
Allergies:
Norvasc
(amlodipine)
Family
History:
M.M.’s
family
history
is
significant
for
hypertension
and
asthma
for
her
mother
and
maternal
grandparents.
Her
mother
also
has
a
history
of
anemia
and
back
pain.
Her
maternal
grandparents
have
an
additional
history
of
diabetes
and
thyroid
disorders.
There
is
no
known
history
of
systemic
lupus
erythematosus
or
autoimmune
disorders.
Social
History:
M.M.
is
an
only
child
and
lives
at
home
with
her
mother
and
father.
There
are
no
pets
and
no
smoking
in
the
home.
She
is
currently
a
high
school
senior,
normally
active,
socializes
with
friends,
and
does
well
in
school.
She
does
not
smoke,
drink
alcohol,
or
do
illicit
drugs.
Her
appetite
is
usually
good
and
she
tries
to
eat
regular
healthy
meals,
however,
she
requires
a
low
sodium
and
potassium
diet.
Review
of
Systems
(upon
admission):
General:
Mild
obesity.
Skin:
Intermittent
facial
rash.
Eye:
Occasional
blurred
vision
in
both
eyes.
HENT:
Unremarkable.
Normocephalic.
Cardiovascular:
Unremarkable.
Pulmonary:
Unremarkable.
Lymphatics:
Unremarkable.
Gastrointestinal:
Unremarkable.
Genitourinary:
SLE
Membranous
Glomerulonephritis
in
2008.
No
recent
urgency,
frequency,
or
pain.
Musculoskeletal:
Pain
(3/10)
when
turning
head
to
right.
Neurologic:
SLE
related
seizures
in
2008.
2

3. Hematologic:
SLE
diagnosed
in
2008.
Endocrine:
Unremarkable.
Psychiatric:
Alert
and
oriented
to
time
and
place.
Appropriate
affect.
Physical
Exam
(upon
admission):
Vitals:
BP:
125/91
PR:
102
RR:
18
Pulse
Ox:
98%
RA
T:
98.8
General:
Mild
obesity,
supine,
in
moderate
distress
due
to
severe
non-­‐focal
headache,
10/10
pain.
Skin:
Facial
malar
rash
present.
Eye:
PERRL.
EOM’s
full
without
restriction.
Transient
scintillating
scotoma
with
intermittent
bursts
of
color
OU
as
headache
progressed.
HENT:
Normocephalic.
Cardiovascular:
Regular
rate
and
rhythm.
S1,
S2.
No
murmurs,
gallops,
or
clicks
heard.
Pulmonary:
Clear
to
auscultation
with
equal
breath
sounds
bilaterally.
Gastrointestinal:
Abdomen
non-­‐distended,
soft,
and
non-­‐tender
in
all
quadrants.
Genitourinary:
Deferred
physical
exam.
UA
300+
protein.
Musculoskeletal:
Normal
gait.
Neck
range
normal
except
pain
on
lateral
rotation
to
right
side.
Normal
strength
and
tone
of
upper
and
lower
extremities.
Neurologic:
CN
II
–
XII
intact.
Speech
and
language
intact.
Normal
sensation
upper
and
lower
extremities.
DTR
normal
response
bilaterally.
Hematologic:
Left
shift
with
thrombocytopenia.
Elevated
creatinine,
ESR,
and
CRP.
Lymphatics:
No
lymphadenopathy
at
neck,
axilla,
groin.
Endocrine:
No
evidence
of
goiter,
myxedema,
tremor,
exophthalmos,
or
hirsutism.
Psychiatric:
Alert
and
oriented
to
time
and
place.
Appropriate
affect.
Labs
(upon
admission):
WBC:
6.1
(S72,
B6,
L15)
Na:
139
K:
4.6
Gl:
84
CRP:
0.83
Hgb:
12.3
Cl:
106
HCO3:
20
Ca:
9.6
ESR:
40
Platelets:
110
BUN:
22
Cr:
1.09
ALT:
28
Tbili:
0.2
Amylase:
102
AST:
28
Tprotein:
7.8
ALP:
95
Albumin:
4.2
UA:
Protein
300+,
(-­‐)
glucose,
RBC,
WBC,
casts,
bilirubin,
ketones,
nitrites,
leukocyte
esterase.
3

4.
Imaging:
CT
w/o
contrast
revealed
no
acute
intracranial
abnormality.
Prominence
of
ventricles
and
sulci
was
apparent
and
equal
to
2008
CT
studies.
There
was
some
opacification
of
the
left
maxillary
sinus.
MRI
w/o
contrast
demonstrated
no
evidence
of
acute
or
subacute
infarction.
However,
there
is
a
focal
small
zone
of
left
parietal
lobe
encephalomalacia
that
was
also
noted
in
2008,
probably
secondary
to
a
small
infarction
at
the
time.
MRA/MRV
was
within
normal
limits.
Lumbar
Puncture:
An
elevated
opening
pressure
of
40cm
H2O
(Normal:
1cm
–
10cm
H2O)
was
discovered
with
a
subsequent
closing
pressure
of
10cm.
CSF
analysis
did
not
suggest
viral,
fungal,
or
bacterial
CNS
involvement.
Ophthalmologic
Consult:
Papilledema
OU.
Nephrology
Consult:
Increasing
creatinine
combined
with
proteinuria,
therefore,
renal
biopsy
scheduled.
Differential
Diagnosis/Discussion/Assessment:
Considering
M.M.’s
history
of
systemic
lupus
erythematosus,
along
with
her
associated
renal
and
previous
CNS
impairment,
creates
a
high
degree
of
suspicion
with
respect
to
SLE
cerebritis
as
the
etiologic
basis
for
her
current
symptoms.
However,
her
current
imaging
studies
are
unchanged
from
2008
and
also
reveal
no
acute
findings.
In
addition,
her
LP
analysis
did
not
point
to
an
inflammatory
process.
Finally,
she
did
not
present
with
fever
or
leukocytosis,
which
may
also
be
indicative
of
CNS
inflammation.
With
decreased
suspicion
of
SLE
cerebritis,
M.M.’s
presentation
is
quite
typical
of
“benign”
intracranial
hypertension/pseudotumor
cerebri.
Pseudotumor
cerebri
is
more
commonly
found
in
overweight
females,
usually
under
the
age
30,
who
present
with
transient
or
increasing
visual
obscurations
combined
with
intermittent
or
chronic
non-­‐focal
global
headache.
It
can
be
an
insidious
process,
and
oftentimes
misdiagnosed
as
migraine
due
to
negative
imaging
findings
with
normal
laboratory
blood
work.
It
can
be
confirmed
with
an
elevated
LP
opening
pressure
with
or
without
papilledema.
Regarding
SLE
and
M.M.’s
history
of
membranous
glomerulonephritis,
increasing
creatinine
levels
and
proteinuria
were
noted.
Assessment:
Pseudotumor
Cerebri
with
associated
Papilledema
and
chronic
headache.
SLE
Membranous
Glomerulonephritis
exacerbation
per
laboratory
findings.
Previous
SLE
Cerebritis
with
associated
epileptic
episodes
(2008).
Systemic
Lupus
Erythematosus
(Dx:
2008).
4

5. Plan:
Without
treatment,
various
permanent
neurologic
deficits
can
ensue.
In
particular,
long-­‐standing
advanced
pseudotumor
cerebri
can
produce
chronic
papilledema,
compressive/ischemic
optic
neuropathy,
leading
to
secondary
unilateral
or
bilateral
optic
atrophy.
Optic
atrophy
can
manifest
with
permanent
central
scotoma,
visual
field
defect(s),
possibly
leading
to
non-­‐functional
vision.
Therefore,
based
on
the
above
findings,
M.M.’s
treatment
protocol
involves
the
following:
Lumbar
puncture
as
both
diagnostic
and
therapeutic
strategies.
In-­‐patient
pain
management
as
needed.
VEEG
monitoring
for
evidence
of
possible
recurrent
seizure
activity.
Keppra
level
monitoring
for
seizure
prophylaxis.
IV
bolus
solumedrol
for
possible
SLE
nephritic
flare.
C3,
C4
to
support
SLE
membranous
glomerulonephritis
diagnosis.
Renal
biopsy.
Anti-­‐cardiolipin-­‐antibody
to
support
SLE
diagnosis.
Blood
and
urine
cultures
to
rule-­‐out
possible
septic
meningitis.
Enterovirus
PCR
to
rule
out
enteroviral
meningitis.
Lyme
titer
IgG,
IgM
to
rule
out
possible
lyme
CNS
involvement.
M.M.
reported
significant
headache
relief
post
LP,
supporting
the
diagnosis
of
pseudotumor
cerebri.
Currently,
she
is
continuing
in-­‐patient
treatment
pending
the
above
studies
and
biopsy
results.
5