Week 4
11:00-12:00
PEDIATRIC NEUROLOGY
Case 1: On a busy
afternoon you see the following children in subsequent
visits:
1) a 4-month old girl born at 34 weeks gestation
without major complications. Her parents arrive with
concerns that she has reduced tone and difficulty
feeding. On exam she is alert and smiling, but drooling
freely. She does have low resting tone and a head lag,
but otherwise seems strong. Her reflexes are brisk and
she has 6 beats of clonus at the ankles bilaterally.
2) a 12-year-old who comes for an update on his
seizure medications. He had normal development until age
3 years, when he was thrown through a window during a
car accident. Following 12 hours of coma, he emerged
with a seizure disorder, learning difficulties in the
mildly mentally retarded range, and spastic
quadriplegia.
3) a 5-year-old arrives for advice regarding his
school planning. He is a bright child who knows his
alphabet and is already reading well. However, he is in
a wheelchair because of significant athetosis and
chorea, which prevent him from speaking clearly or using
writing utensils. He was born full term, complicated by
placental abruption requiring a blood transfusion and a
four day Neonatal ICU stay.
1. Is it appropriate to use the diagnosis of
cerebral
palsy for any or all of these children?
2. Which ones and why/ why not?
3. How is CP classified usually?
4. Are all children with CP mentally impaired?
Case 2: A 6 year
old presents with a history of an increased activity
level and a short attention span. His parents report
that he is unable to sit quietly at school and has
frequent temper tantrums when he does not get his way.
At home he interrupts conversations, runs into the
street without looking for cars, disappears in a store,
does not sit still at the dinner table, and easily gets
distracted by stimuli in the environment. His family
states that these behaviors have been happening for
several years.
1. What are typical clinical features of
attention
deficit-hyperactivity disorder?
2. What clinical features are most prominent at
different ages?
3. What advice should be given to the family regarding
management?
4. What is the differential diagnosis of a child with
this presentation?
Case 3: You are
asked to evaluate a three month old baby because he is
floppy. He was the product of an uncomplicated
pregnancy, labor and delivery. He was noted in the
nursery to be quite floppy, and had a poor suck. His
mother states that he has been in the hospital three
times since birth with pneumonia. Over the last month he
has had increasing difficulty with feeding. On
examination, you find a very alert baby who has
decreased spontaneous movements except of his hands and
feet. He lies supine in the "frog-leg" position. His
cranial nerve examination is normal. Motor exam reveals
markedly reduced proximal muscle strength with a
moderately weak hand grasp bilaterally. There are no
deep tendon reflexes.
1. What is the ‘anatomic' differential diagnosis of a
floppy infant? (i.e., what locations from brain to
muscle can cause hypotonia?).
2. What other ‘systemic' problems should be considered?
3. Are all hypotonic infants weak?
4. What is the localizing value of the following
findings?
a. physical dysmorphic
features
b. seizures and/or drowsiness
c. fasciculations of tongue and other muscles
d. increasing weakness with effort
e. hyperreflexia
f. normal or hypoactive reflexes
g. absent reflexes
5. What is your diagnosis of this baby? What tests
would you do to confirm this diagnosis?
6. How would you counsel the parents?
Case 4:
A 10
month old baby boy is brought to your office because of
his “big head”. His mother states when he was born his
head was “medium” sized, but his pediatrician is
concerned that over the last four months his head has
been growing too fast. His head circumference is now 52
cm, well above the 98th percentile. His examination is
basically normal except for three hypopigmented birth
marks on his chest.
1. What is the differential diagnosis of macrocephaly
in infants?
2. What would you examine on the parents that might help
you with your diagnosis?
3. What is the significance of the hypopigmented birth
marks?
4. What tests/laboratory studies would you recommend for
this child ( if any)?
Extra Cases if Time Permits
Case 5: A
two-year-old child previously well is seen in the ER
with the chief complaint of "she is walking like she is
drunk.” Her mother states that she woke up this morning
a little unsteady, but has gotten much worse as the day
has progressed. She has been well, but her five-year-old
sibling presently has chicken pox. On examination she is
afebrile and vital signs are stable. She is somewhat
irritable but will calm down when consoled by her
mother. Her examination shows intact cranial nerves.
Muscle strength and tone is normal. Deep tendon reflexes
are 2+ and symmetrical. She had a broad-based ataxic
gait. She has dysmetria on finger to nose testing.
1. What is the differential diagnosis of acute
ataxia
in childhood?
2. What is the significance of the chicken pox exposure
in this child?
3. What if her examination revealed absence of deep
tendon reflexes, how would this change your suspected
diagnosis?
4. Would you admit this child? What studies/labs would
you order?
Case 6:
A
Caucasian baby girl was born to a primigravida 17
year-old woman who had no prenatal care until the one
month prior to delivery. The mother presented to the
obstetrical service with a fever and foul smelling
amniotic fluid, in active labor on the day of delivery.
Fetal bradycardia was noted during intrapartum
monitoring between 80 and 100 beats per minute. The
child was delivered with an Apgar at one minute of four
and at five minutes of eight with a birth weight of 1100
grams. Physical examination included an estimated
maturity between 28 and 30 weeks gestation. Grunting and
retractions were noted at one hour of life and the
infant was intubated, requiring ventilatory assistance
for six days. On day two of life, a sudden desaturation
and mottled appearance of the skin was noted and a right
pneumothorax was diagnosed. After placement of a chest
tube, the child was noted to be acidotic with a pH of
7.15. Sodium bicarbonate was administered. A cranial
ultrasound within 12 hours of the occurrence of the
pneumothorax documented an intraventricular hemorrhage
that filled the right ventricle without dilatation of
the ventricular outline. Following extubation on day
seven, the child's head circumference increased by 2 cm
from birth and a repeat sonogram documented significant
ventriculomegaly, with an echodensity in the right
periventricular region extending from the frontal to the
parietal/occipital areas. The infant remained
hospitalized for one month. Upon discharge her
neurologic examination revealed hypertonia in all limbs
and a left hemiparesis.
1. What are some possible explanations for this
infant's premature birth?
2. What is the significance of the maternal fever and
foul smelling amniotic fluid?
3. Why do premature infants develop intraventricular
hemorrhage? What is the significance of the echodensity
in the right periventricular region on the repeat
sonogram?
4. What is the mechanism for the increasing head size?
What is your next step in dealing with the
"ventriculomegaly?”
5. What is the neurologic sequela of extreme premature
birth? Of intraventricular hemorrhage?
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