Case 28

Question 1

What are key history findings in an 18 mo with cerebral palsy?

Answer 1

Premature birth, Gross motor delay, Language delay, Normal social and fine-motor development, Positive family history of infant death.

Question 2

What are key findings in the physical exam of an 18 mo with cerebral palsy?

Answer 2

Increased tone and reflexes in lower extremities.

Question 3

What is on the differential diagnosis with cerebral palsy?

Answer 3

Genetic abnormality, Metabolic disorder, Normal variant, Neurocognitive disorder, Cerebral palsy, Myopathy, Autism/Pervasive developmental disorder, Reaction to psychosocial stress

Question 4

What is seen on an MRI of a toddler with cerebral palsy?

Answer 4

MRI of the brain shows irregularly shaped ventricles and increased FLAIR signal (indicating periventricular leukomalacia) and thinned corpus callosum.

Question 5

Prematurity and development:

Answer 5

Infants born prematurely (less than 37 weeks) are at risk for many difficulties that may affect their development, including those listed below. The number and severity of problems generally correlates with decreasing gestational age. These problems include:

• Bronchopulmonary dysplasia
• Retinopathy of prematurity
• Hyperbilirubinemia
• Periventricular leukomalacia (PVL)

Question 6

What is bronchopulmonary dysplasia?

Answer 6

• May cause poor growth due to increased caloric requirements, pulmonary infections, or congestive heart failure
• May necessitate prolonged or repeated hospitalization

Question 7

What is retinopathy of prematurity?

Answer 7

Visual impairment may affect development, depending on the degree.

Question 8

Hyperbilirubinemia:

Answer 8

• Potential neurotoxin

- Kernicterus is characterized by abnormal motor development and sensorineural hearing loss

Question 9

Periventricular leukomalacia (PVL):

Answer 9

• Damage to white matter surrounding the ventricles resulting from hypoxia, ischemia, and inflammation
• PVL with cysts is highly correlated with cerebral palsy

Question 10

What is the definition of Cerebral palsy?

Answer 10

Cerebral palsy (CP) refers to a group of non-progressive motor control disorders. Historically, the different types of cerebral palsy have been grouped together because they all involve motor abnormalities and are treated somewhat similarly. These disorders often include spasticity, exaggerated tendon jerks, and clonus.

Question 11

Spastic quadriplegia cerebral palsy:

Answer 11

Involves entire body.
Etiology: Global brain abnormalities.
Various clinical scenarios.

Question 12

Spastic diplegia cerebral palsy:

Answer 12

Involves legs more than arms.
Etiology: Periventricular white matter abnormality.
Clinical scenarios: Premature infants.

Question 13

Dyskinetic cerebral palsy (athetoid, dystonic):

Answer 13

Variable pattern of involvement, often entire body.
Etiology:
Basal ganglia, cerebellar, and/or thalamus abnormalities.
Clinical scenarios: Perinatal asphyxia, kernicterus.

Question 14

(Spastic) hemiplegia cerebral palsy:

Answer 14

General pattern of involvement: Arm and leg on one side.
Etiology: Unilateral upper motor neuron abnormalities.
Clinical scenario: Stroke.

Question 15

Ataxic cerebral palsy:

Answer 15

Involves entire body.
Etiology: Cerebellar abnormalities.
Clinical scenario: Cerebellar hypoplasia, pontocerebellar hypoplasia.

Question 16

Spastic diplegia:

Answer 16

Characterized by increased tone, predominantly in the lower extremities. Its incidence is greatly increased in premature infants.

Question 17

What is the prevalence of CP in western countries?

Answer 17

2/1,000

Question 18

What is the Etiology CP?

Answer 18

• Most CP is NOT caused by birth asphyxia or other identifiable events. Birth asphyxia and kernicterus generally cause dyskinetic CP, not other types.
• An Australian study of 213 children diagnosed with CP illustrates the multifactorial etiology:
• -Major CP-associated pathologies other than acute intrapartum hypoxia were identified in 98 percent of cases.
• -Some children had more than one associated pathology. The risk of each of the following pathologies was:
• –Prematurity (78 percent)
• –IUGR (34 percent)
• –Intrauterine infection (28 percent)
• –Perinatal asphyxia (10 percent)

Question 19

Developmental Surveillance:

Answer 19

Includes the elements listed below - is recc. by the AAP at every preventative care visit:

• Maintenance of a developmental history
• Accurate and informed observations of the child
• Identification of risk and protective factors
• Documentation of the process and findings

Question 20

Developmental Screening:

Answer 20

The AAP recommends developmental screening using a validated screening tool for all children at least at 9, 18, and 30 months.

• Several screening tools including the Ages and stages questionnaires (ASQ) are available.
• The modified checklist for autism in toddlers (M-CHAT) is a parent-completed questionnaire validated as a screening tool to identify children between 16 and 30 months of age at risk for autism spectrum disorders.

Question 21

Corrected age:

Answer 21

• When assessing growth and development in former premature infants, use their “corrected age: (chronological age minus the weeks of prematurity)
• According to the Centers for Disease Control (CDC), using corrected age should be discontinued when child reaches two years.

Question 22

Measuring head circumference:

Answer 22

Measure the largest circumference by making sure that the tape wraps tightly around the most prominent parts of the occiput and forehead. Measure 2-3 times to ensure accuracy.

Question 23

Neurological Exam in a toddler:

Answer 23

The most important tool is observation

1. Mental status
2. Cranial nerve exam
3. Muscle tone
4. Muscle strength
5. Deep tendon reflexes
6. Babinski
7. Cerebellar

Question 24

Mental Status exam in toddler:

Answer 24

How does child interact with the caregiver? How does he interact with you (a less reliable measure)? Is he alert, sleepy, arousable, etc.?

Question 25

Cranial Nerve exam in toddler:

Answer 25

Cannot directly test most cranial nerves (except II), however, indirect methods include:

• Moving a flashlight or toy around and watching the actions of cranial nerves II, IV and VI
• Looking for facial symmetry as a marker for VII function (watch when they cry, too; is the face symmetric?)
• Testing hearing (VIII) with a mechanical watch or by rubbing your fingers together or by crumpling paper; does child turn toward the sound?
• Looking carefully at the palate and tongue during the oropharyngeal exam; does the palate go up symmetrically and the tongue protrude? Does the child drool?
• Listen to child’s voice (IX, X, XII)

Question 26

Muscle tone in a toddler:

Answer 26

While playing with the child, move his arms/legs through full range of movement. Observe the tone (the resting state of the muscle, which can be normal, increased or decreased). Toe-waling can be a sign of increased calf muscle tone.

• Muscle spasticity is defined as increased muscle resistance that is velocity-dependent (i.e., resistance is greater when passive movement is rapid and less when passive movement is slow). People often have both increased tone and spasticity.
• Hypotonia (low tone) is manifested as slumped posture, poor head control, soft muscles, and patient slipping through your hands when held under the armpits.

Question 27

Muscle strength in a toddler:

Answer 27

Watch the child for any obvious weakness as he plays. Also see how much the child resists your physical exam maneuvers (i.e., kicking). Look for asymmetry of muscle bulk.

Question 28

Deep tendon reflexes in a toddler:

Answer 28

Use your finger to tap the tendons. Note vigorousness of response. Observe for asymmetry; note if asymmetry correlates with asymmetry of tone or muscle bulk

Question 29

Babinski:

Answer 29

Use your thumbnail to stroke the bottom of the foot.

Question 30

Cerebellar:

Answer 30

Watch child’s balance as he plays. Does he have smooth, coordinated movements? Observe for asymmetry.

Question 31

What are more likely differential diagnosis with CP?

Answer 31

Normal variant, Metabolic disorder and genetic abnormality

Question 32

What are less likely differential diagnosis with CP?

Answer 32

Myopathy, Reaction to psychosocial stress, Neurodegenerative disorder, Autism/PDD

Question 33

Cerebral palsy (CP):

Answer 33

Children with CP have abnormal motor development, and other aspects of their development are variably impacted depending on the underlying illness. Neuromuscular exams are abnormal, often with increased tone and reflexes, again reflecting the extent of the underlying cause of the CP.

Question 34

Normal variant:

Answer 34

Childhood development is highly variable.

Question 35

Metabolic disorder:

Answer 35

A family history of miscarriages or infant deaths would raise this possibility. The newborn screen is helpful in ruling out many metabolic disorders.

Question 36

Genetic abnormality:

Answer 36

Specific findings vary with the genetic syndrome and the individual patient. A positive family history of similarly affected children or early infant deaths is also suggestive of a disorder such as chromosomal abnormalities or inborn errors of metabolism.

Question 37

Myopathy:

Answer 37

Children with myopathies have abnormal development in gross motor and perhaps fine motor skills. All other realms of development - including language development - should be normal.

Question 38

Reaction to psychosocial stress:

Answer 38

Behavioral changes or temporary loss of achievements - a normal adaptive response - may occur in times of stress. In toddlers, temper tantrums, sleep disturbances and refusal to eat are common adaptations. Language acquisition also may be slowed.

Question 39

Neurodegenerative disorder:

Answer 39

These usually cause regression in achieved milestones.

Question 40

Autism/PDD:

Answer 40

Children with autism or pervasive developmental disorder display impairment in communication (including delay in, or total lack of, development of spoken language). Motor development is typically within normal range.

Question 41

Neuroimaging for CP:

Answer 41

Recommended to both establish an etiology and for prognostic purposes. MRI is preferred to CT scan.
-Findings of periventricular leukomalacia and thinned corpus callosum support a diagnosis of CP

Question 42

Initial Evaluation of a child with CP:

Answer 42

The practice parameter from the American Academy of Neurology and Child Neurology Society recommends the following approach to the evaluation of the child with CP:

• Detailed history and PE
• -Imp. to determine whether condition is static or progressive or degenerative
• Classification of type of CP
• As part of the initial assessment, screening for developmental delays, ophthalmologic abnormalities, hearing impairment, speech and language disorders, and disorders of oral-motor function - problems that are commonly seen in CP.
• An EEG is rec. when there are features suggestive of epilepsy
• Neuroimaging rec. to bother est. an etiology and for prognostic purposes (MRI preferred to CT scan)
• Consider metabolic and genetic testing if clinical hx and findings on neuroimaging do not determine a specific structural abnormality or if there are additional and atypical features in teh history or clinical examination.