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Case 15

A 12-month-old boy whom you have followed since birth arrives


for a well-child visit. The mother is concerned that the baby’s
manner of crawling, where he drags his legs rather than using a
four-limbed move- ment, is abnormal. She says that the child only
recently began crawling and he does not pull to a stand. You noted
at his 6-month visit that he was not yet rolling over nor sitting;
previous visits were unremarkable as was the mother’s pregnancy
and vaginal delivery. On examination today, you note that he
positions his legs in a “scissoring” posture when held by the axillae.

➤ What is the initial step in the evaluation of this child?

➤ What is the most likely diagnosis?

➤ What is the next step in the evaluation?


130 CASE FILES: Pediatrics

ANSWERS TO CASE 15:


Cerebral Palsy
Summary: A 12-month-old boy crawls using primarily his upper
extremities, and holds his legs in a “scissoring” posture when
suspended.
➤ Initial step: Gather detailed history, focusing on developmental questions;
obtain a thorough pregnancy, birth, social, and family histories; and
perform a detailed neurologic examination.
➤ Most likely diagnosis: Cerebral palsy (CP).
➤ Next step: Vision and hearing testing, consider a brain magnetic
resonance imaging (MRI) scan, and arrange for therapy with a
developmental specialist.

ANALYSIS

Objectives
1. Know the definition of CP.
2. Recognize the classifications of CP.
3. Know the basic therapeutic approach to CP.

Considerations
The spasticity of the baby’s lower extremities described is abnormal and
is sug- gestive of CP. He has gross motor delay. A complete
developmental and neu- rologic assessment is crucial for initiating
therapies that will help him achieve maximal functional outcome.
Although often of low yield, an attempt should be made to identify the
etiology of the child’s CP. Knowing the etiology can aid in developing a
treatment plan, in family planning (especially if the eti- ology is
inherited), and in assuaging parental guilt for this child’s condition.

APPROACH TO
Cerebral Palsy

DEFINITIONS
CEREBRAL PALSY (CP): A disorder of movement and posture that
results from an insult to or anomaly of the immature central nervous
system (CNS). This definition recognizes the central origin of the
CLINICAL 13
dysfunction, thus distin- guishing it from neuropathies and myopathies.
132 CASE FILES: Pediatrics

DEVELOPMENTAL DELAY: Failure of a child to reach developmental


mile- stones of gross motor, fine motor, language, and social-adaptive
skills at antic- ipated ages.
NEUROLOGIC DEFICIT: Abnormal functioning or lack of function of a
part of the nervous system.

CLINICAL APPROACH
With a prevalence of at least 1 to 2 cases per 1000 live births, CP is
the most common childhood movement disorder. Approximately one-
third of CP patients also have seizures, and approximately 60% are
mentally retarded. Deafness, visual impairments, swallowing difficulty
with concomitant aspira- tion, limb sensory impairments, and
behavioral disturbances are common comorbidities. The effect of
aggressive neonatal medical therapies on CP prevalence is unclear;
improved premature infant outcomes may mitigate the impact of
increased survival of very low-birth-weight infants.
Most children with CP have no identifiable risk factors. Current
research indicates that CP most likely is the result of antenatal insults.
Difficulties during the pregnancy, delivery, and the perinatal period are
thought to reflect these insults and are probably not the primary cause
of CP.
Cerebral palsy, or “static” encephalopathy, is the result of a one-time
CNS insult. In contrast, progressive encephalopathies destroy brain function
with time. The term static is misleading, however, because the
manifestations of CP change with age. Contractures and postural
deformities may become more severe with time or may improve with
therapy. Also, a child’s changing developmental stages early in life can
alter the expression of his or her neurologic deficits.
Immaturity of the CNS at birth makes diagnosis of CP nearly
impossible in a neonate. If a CNS insult is suspected, head imaging (by
ultrasound or MRI) can be helpful in recognizing CP early. Possible
imaging findings include periventricular leukomalacia, atrophy, or focal
infarctions. Beyond infancy, CP is suspected when a child fails to meet
anticipated developmental milestones.
Examples of concerning findings are
• A stepping response after age 3 months
• A Moro reflex beyond 6 months
• An asymmetrical tonic neck reflex beyond 6 months
Cerebral palsy can be classified in terms of physiologic,
topographic, or functional categories. Physiologic descriptors identify the
major motor abnor- mality and are divided into pyramidal (spastic) and
extrapyramidal (nonspastic) categories. Extrapyramidal types can be
subdivided further into choreoathetoid, ataxic, dystonic, or rigid types.
The topographic classification categorizes CP types according to
limb involvement. Hemiplegia refers to involvement of a single lateral
side of the body, with greater impairment of the upper extremities than
CLINICAL 13
the lower extrem- ities. Diplegia describes four-limb involvement, with
greater impairment of the
134 CASE FILES: Pediatrics

lower extremities. Spastic quadriplegia is four-limb involvement with


signifi- cant impairment of all extremities, although the upper limbs
may be less impaired than lower limbs. (The term paraplegia is reserved
for spinal and lower motor neuron disorders.)
The functional classification of CP relies on the “motor quotient” to
place patients into minimal, mild, moderate, and severe (profound)
categories. The motor quotient is derived by dividing the child’s “motor
age” (ie, motor skills developmental age) with the chronologic age. A
motor quotient of 75 to 100 represents minimal impairment, 55 to 70 mild
impairment, 40 to 55 moderate impairment, and lesser quotients severe
impairment. These categories help clinicians identify children with less
obvious impairments so that early treat- ment can be provided.
The evaluation of CP is based on the history and physical
examination. The yield of diagnostic findings with brain imaging and
metabolic or genetic testing is low but can be helpful in managing the
patient, in future family planning, and in reassuring the parents.
Identification of comorbid conditions includes cognitive testing for mental
retardation and electroencephalography (EEG) for seizures.
Treatment goals include maximizing motor function and preventing sec-
ondary handicaps. During the preschool years, the child’s
communication ability is important. School performance and peer
acceptance become impor- tant issues for older children. Physical therapy
for motor deficits may be sup- plemented with pharmacologic and
surgical interventions. Occupational therapy improves positioning and
allows for better interaction with the envi- ronment and eases care as the
child grows. The family’s psychological and social needs should not be
overlooked; children may require extensive physi- cal and emotional
support.
CLINICAL 13

Comprehension Questions

15.1 A term infant requires resuscitation after a spontaneous vaginal


deliv- ery. The Apgar scores at 1, 5, and 10 minutes were 2, 7,
and 9, respec- tively. The mother’s medical records show that
she received routine prenatal care with normal prenatal
ultrasonogram, triple screen, and glucose tolerance tests. The nurse
tells you that the father seemed very agitated and mentioned
“suing the obstetrician if the baby does not turn out normal.” Your
examination of the baby reveals no abnormalities. In counseling the
family, which of the following is most appropriate?
A. Tell them that your examination findings indicate that
everything is fine.
B. Tell them that the low Apgar scores at 1 and 5 minutes
indicate that the baby suffered perinatal asphyxia.
C. Tell them that because the pregnancy was uncomplicated,
any neurologic deficit that the baby may develop likely can be
attrib- uted to events occurring at delivery.
D. Tell them that your examination findings are reassuring, and
that you will perform a careful developmental assessment at
every well- child visit.
E. Avoid speaking to the parents until you have had a chance to
speak with the obstetrician and to see the cord blood gas
results.
15.2 A 4-year-old child with CP comes to your clinic for the first
time for a routine visit. He walks with the help of leg braces
and a walker, and his speech is slurred and limited to short
phrases. He has never been hospitalized and he does not have
swallowing problems. He began walking at age 2.5 years, and
he is unable to take off his clothes and use the toilet without
help. On examination you find that the boy has only minimally
increased tone in the upper extremities but good fine motor
coordination; he has significantly increased tone and deep ten- don
reflexes in the lower extremities. How would you categorize this
child’s CP?
A. Mild, diplegic
B. Mild, hemiplegic
C. Moderate, diplegic
D. Moderate, quadriplegic
E. Severe, diplegic
136 CASE FILES: Pediatrics

15.3 An infant girl is born via spontaneous vaginal delivery at 28-week


ges- tation because of an incompetent cervix. Which of the
following fea- tures of her clinical course in the neonatal intensive
care unit (ICU) is most likely to correlate with her clinical outcome
5 years from now?
A. Administration of surfactant
B. Apnea of prematurity
C. Grade IV intraventricular hemorrhage
D. Retinopathy of prematurity stage 1 on initial ophthalmologic
examination
E. Umbilical artery catheterization
15.4 The parents of a 2-year-old girl, recent immigrants from Guatemala,
bring their child to you for the first time. The child was born at term
after an uncomplicated pregnancy and delivery, and her neonatal
course was uneventful. She sat without support at 6 months of age,
pulled to a stand at 10 months, and walked at 14 months. She has a
10-word vocabulary, is able to drink from a cup, and feeds herself
with a spoon. A previous child in the family died at age 5 years from
“heart trouble.” On physical examination, you note lower extremity
contractures, hand stiffness, somewhat coarse facial features, and
hepatosplenomegaly. The child’s growth is within normal limits, and her
examination is other- wise normal. Which of the following is the most
appropriate next step to diagnose this child’s condition?
A. Abdominal computerized tomography (CT)
B. Brain magnetic resonance imaging (MRI)
C. Chromosomal analysis
D. Tests for a storage disorder
E. Thyroid function studies

ANSWERS
15.1 D. The Apgar score at 1 minute reflects the neonatal environment
immediately prior to birth; the 5-minute score correlates the
infant’s response to resuscitation. The Apgar scores are not an
accurate reflection of morbidity. An examination is a better
indicator of the child’s outcome, but CP cannot be ruled out on the
basis of a normal neonatal physical examination. A discussion of
the events of deliv- ery is best left to the obstetrician; the majority
of difficult deliveries are the result of a previously unidentified
antenatal insult. However, avoidance of the parents will likely only
further their anxiety and may impede your efforts to provide care
for the child.
CLINICAL 13

15.2 C. In diplegia all four extremities are affected, with greater


impairment of the lower extremities. As most children walk by age
14 months, this child’s motor quotient is 14 months/30 months =
0.47, which classifies him as moderately impaired.
15.3 C. Intraventricular hemorrhage is a complication in preterm
infants. It is associated with seizures, hydrocephalus, and
periventricular leukomalacia. A grade IV bleed involves the brain
parenchyma, put- ting this child at higher risk for
neurodevelopmental handicap.
15.4 D. The enlarged liver and spleen, the coarse facies, and the history
of death in a previous child from “heart trouble” point to a storage
disorder. Her joint contractures and hand stiffness may be
explained by an abnormal metabolism rather than a CNS deficit as
in CP.

Clinical Pearls
Cerebral palsy is a disorder of movement or posture resulting from an insult to, or an anomaly of, the ce
Most children with cerebral palsy have no identifiable risk factors for the disorder.
Optimal treatment plans for cerebral palsy use a multidisciplinary approach.

REFERENCES
American Academy of Pediatrics. Use and abuse of the Apgar score. Available at:
http://www.aap.org.
Johnson MV. Cerebral palsy. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF,
eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, PA: WB Saunders;
2007:2494-2495.
Shapiro BK, Capute AJ. Cerebral palsy. In: McMillan JA, Feigin RD, DeAngelis CD,
Jones MD, eds. Oski’s Pediatrics: Principles and Practice. 4th ed. Philadelphia,
PA: Lippincott Williams & Wilkins; 2006:2251-2258.
Wollack JB, Nichter CA. Cerebral palsy. In: Rudolph CD, Rudolph AM, Hostetter
MK, Lister G, Siegel NJ, eds. Rudolph’s Pediatrics. 21st ed. New York, NY:
McGraw- Hill; 2003:2197-2202.

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