Pediatric Examination and Board Review (57 page)

2.
(B)
The use of stimulants in Tourette syndrome can cause worsening of tics and should be treated with a different medication.

3.
(D)
The ratio of boys to girls with Tourette syndrome is 4 to 1.

4.
(A)
Guanfacine is a centrally acting alpha-2 adrenergic agonist with antihypertensive activity. The mechanism for decreasing tics is not fully understood but is considered a first-line therapy for Tourette syndrome.

S
UGGESTED
R
EADING

Jankovic J. Tourette’s syndrome.
N Engl J Med.
2001;345:1184-1192.

Leckman JF, Cohen DJ, Goetz CG, Jankovic J. Tourette syndrome: pieces of the puzzle.
Adv Neurol.
2001;85:369-90.

Silay YS, Jankovic J. Emerging drugs in Tourette syndrome.
Expert Opin Emerg Drugs.
2005; 10:365-80.

CASE 36: AN 8-YEAR-OLD GIRL WHO IS BOSSY

You are seeing an 8-year-old girl in your office with her mother who explains that her daughter is having trouble in school. She is having a hard time making friends and is constantly worried about what other children think of her. She does not think she has any friends, but her mother says she does. In school, she is constantly tattling on other children who break rules. She often misinterprets or follows rules literally. When there is a substitute teacher, she gets frustrated and acts out constantly because the teacher is not on the usual schedule. She likes being around the other children but has trouble keeping a conversation back and forth with them. She relates better to adults or small children than to peers. Her sleep is also a concern. Getting to bed takes at least 2 hours each night because she needs to take a shower, dress in the same pajamas, listen to a CD, read particular books, turn the fan on, line up her stuffed animals, and kiss a picture of her deceased aunt that she keeps under her pillow (who died when she was 3 years old). If any part of the routine is off, she gets upset and tantrums. The visit with you was scheduled at the last minute, and the change in her routine caused her so much distress that she gagged and vomited because she expected to take a math test instead. Her developmental history is normal for language development and cognitive abilities. She gets good grades in school. Her mother looks to you for help in explaining her behavior and for management.

SELECT THE ONE BEST ANSWER

1.
Which of the following would best explain her behavior problems?

(A) sleep disorder

(B) ADHD

(C) oppositional defiant disorder (ODD)

(D) bipolar disorder

(E) obsession with routine and preoccupation with rules

2.
In your office, she answers questions appropriately with direct, succinct answers. She has difficulty telling you about her day or explaining the plot to a story that she read, instead focusing on minute details that do not give a good overall summary. Based on this, what is your diagnosis?

(A) obsessive compulsive disorder (OCD)

(B) Asperger syndrome

(C) learning disability

(D) ADHD

(E) schizophrenia

3.
Which of the following would you expect to be most difficult for her?

(A) playing on the playground with her class

(B) keeping track of a calendar

(C) arts and crafts projects

(D) being introduced to adults at a party

(E) playing a board game with a group of her classmates

4.
What intervention would give her the most benefit?

(A) one-on-one tutor during group times

(B) special education classes

(C) social skills playgroup with social stories training

(D) stimulant medication

(E) none of the above

5.
How should her mother best handle changes in routine?

(A) use of advanced warning, with frequent reminders

(B) use a transitional object, such as a favorite toy

(C) wait until the change in routine happens; then prepare for emotional support

(D) never change routine

(E) none of the above

6.
How do you advise her mother to deal with her daughter’s emotional breakdowns?

(A) letting her hit a pillow

(B) taking away her toys

(C) use of journaling or artwork to express her feelings

(D) putting her in her bedroom by herself

(E) spanking

ANSWERS

1.
(E)
Being preoccupied with rules and routines seem to be the source of her negative dysfunctional behaviors.

2.
(B)
Asperger syndrome is characterized by impairments in social skills, restricted patterns of interest or behavior, and dysfunctional impairments without delays in language or cognition.

3.
(E)
Being in her peer group and playing a game where there are rules to follow would be a difficult task for a child with Asperger. The likelihood for misinterpreting or over interpreting rules is high, and there is likely the need for reciprocal social interaction with her peers.

4.
(C)
Learning scripted social stories and practicing social interactions with a peer group would be most beneficial for this child.

5.
(A)
Planning for transitions well in advance is important for children with Asperger syndrome so they can anticipate events in their day. This will lessen the emotional response to changes in routine.

6.
(C)
Exploring feelings and emotions through alternative means, such as journaling or artwork can be beneficial to children with Asperger.

S
UGGESTED
R
EADING

Attwood T.
The Complete Guide to Asperger’s Syndrome
. London, United Kingdom: Jessica Kingsley; 2006.

Myles BS, Adreon D.
Asperger syndrome and adolescence: Practical strategies for school success
. Shawnee Mission, KS: Autism Asperger Publishing; 2001.

Chapter 5

ENDOCRINOLOGY

CASE 37: A 10-YEAR-OLD BOY WITH SHORT STATURE

A 10-year-old boy comes into your office for a routine physical examination. His only complaint is that he is shorter than all of his friends and he can’t ride the mega roller coaster at the local amusement park because he is shorter than the requirement. You have followed this child for many years, but most visits have been for illness, and his height has not been measured for the last few years. He is not on any medications. He reports occasional fatigue and occasional constipation. On physical examination, his height is less than the 3rd percentile and he is prepubertal.