CLIPP case 28. 18 month-old with motor delays (prematurity, CP)

Question 1

18-month-old boy who is not yet able to walk and has a possible language delay. Further history reveals normal social and fine-motor development. He was a premature baby delivered via emergency Caesarean section with low initial Apgars of 2 and 5. Exam findings reveal increased tone and hyperreflexia in his lower extremities.

Answer 1

Cerebral palsy

• MRI confirms diagnosis
• F/u care: hearing, vision, and developmental testing

Question 2

DDx for developmental delay

Answer 2

• Cerebral palsy
• Genetic abnormality: FHx
• Metabolic disorder: FHx
• Normal variant
• Neurodegenerative disorder: Regression in achieved milestones
• Myopathy: Gross motor +/- fine motor skills. All other (language) should be normal.
• Autism/pervasive developmental disorder: Impaired communication. Normal motor.
• Reaction to psychosocial stress: Behavioral changes or temporary loss of achievements (temper tantrums, sleep disturbances, refusal to eat). Language acquisition also may be slowed.

Question 3

MRI of CP

Answer 3

-Irregularly shaped ventricles and increased
FLAIR signal (periventricular leukomalacia) and thinned corpus callosum

Question 4

Associated disorders of prematurity

Answer 4

-BPD: May cause poor growth due to increased caloric requirements, pulmonary
infections, CHF, or prolonged hospitalization
-Retinopathy: Visual impairment may affect development, depending on the degree
-Hyperbilirubinemia: Neurotoxin. Kernicterus characterized by abnormal motor development and sensorineural hearing loss
-Periventricular leukomalacia: Damage to white matter surrounding the ventricles resulting from hypoxia, ischemia, and inflammation. PVL with cysts highly correlated with CP

Question 5

Cerebral palsy

Answer 5

• Group of nonprogressive motor control
  disorders. Often include spasticity, exaggerated tendon jerks, and clonus.
• Prevalence 2/1,000

Question 6

Cerebral palsy etiology

Answer 6

*Most are NOT caused by birth asphyxia or other identifiable events. Birth asphyxia and kernicterus generally cause dyskinetic CP.

• Prematurity (78%)
• IUGR (34%)
• Intrauterine infection (28%)
• Perinatal asphyxia (10%)

Question 7

Cerebral palsy types

Answer 7

1. Spastic quadriplegia: whole body, global brain
2. Spastic diplegia: Legs > arms. Periventricular white matter abnormality, prematurity
3. Dyskinetic (athetoid) , dystonic: variable, different brain parts, perinatal asphyxia and kernicterus
4. Spastic hemiplegia: arm and leg on one side; unilateral UMN; stroke
5. Ataxic: whole body, cerebellar

Question 8

Spastic quadriplegia CP

Answer 8

whole body, global brain

Question 9

Spastic diplegia

Answer 9

• Legs > arms: increased tone
• Periventricular white matter abnormality
• Prematurity

Question 10

Dyskinetic (athetoid), dystonic CP

Answer 10

• Variable
• Different brain parts (BG, thalamus, cerebellum)
• Perinatal asphyxia and kernicterus

Question 11

Spastic hemiplegia CP

Answer 11

• Arm and leg on one side
• Unilateraly upper motor neuron abnormality
• Stroke

Question 12

Ataxic CP

Answer 12

Whole body, cerebellar

Question 13

Developmental monitoring domains

Answer 13

• Gross motor
• Fine motor
• Communication (receptive and expressive)
• Personal-social
• Problem-solving

Question 14

Developmental surveillance

Answer 14

Recommended by AAP at every preventive care visit:

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

Question 15

Developmental screening

Answer 15

Recommended by AAP using validated
screening tool at least at 9, 18, 30 months
-ASQ
-M-CHAT for ASD 16-30 months (rec 18, 24)

Question 16

Corrected age

Answer 16

• When assessing growth and development in former premature infants, use their “corrected age” (chronological age minus the weeks of prematurity) until two years. Based on 40 weeks normal gestation.
• If born at 29 weeks, use age - 11 weeks.