Cerebral Palsy

Question 1

Define cerebral palsy (CP)?

Answer 1

Neither a disease nor etiology, but rather a descriptive term covering a group of motor impairments involving poor posture, balance, muscle control, and movement

Question 2

What causes CP?

Answer 2

Anomalies or injury occurring early in brain development - in utero, delivery, infancy, or early childhood (often considered within 2-3 years)

Question 3

What are most cases of CP attributed to?

Answer 3

Prematurity and low birth weight, particularly less than 1.5kg. Periventricular leukomalacia is the predominant pathology

Question 4

Aside from prematurity, what are risk factors for CP?

Answer 4

hypoxia before/during labor, prolonged bradycardia, in utero intracerebral hemorrhage, perinatal ischemia, postpartum multisystem organ failure, genetic or congenital malformations such as microgyria/pachygyria

Question 5

Incidence of CP, and what trends are identified in prevalence rates?

Answer 5

Improved prenatal obstetric and postpartum care has reduced incidence of CP to about 2% of births. However, CP remains the most common explanation of pediatric motor impairment, in part due to high survival rates of very low birth weight

Question 6

When is CP typically diagnosed?

Answer 6

Never in utero because it’s based on clinical motor manifestations of brain pathology. Usually not diagnosed until infants are at least 4 months, and in some cases up to age 4. Once a child has an established motor deficit attributable to perinatal cerebral injury, it must not progress as the child grows

Question 7

How does CP progress over time?

Answer 7

CP is nonprogressive, however its motor signs and associated functional impairments can change in response to multiple influences, i.e., brain maturation, intervention, compensatory strategies, or worsening due to aging.

Question 8

How common is intellectual disability in CP? What is ID associated with in cases of CP?

Answer 8

Approximately 50% of individuals with CP have normal intelligence despite major motor deficits.
Proportion of ID (& other complications such as epilepsy) increases with more extensive cerebral disease. Therefore, it’s associated with few extrapyramidal or choreoathetosis CP cases (10%), diplegic CP (25%), half of hemiplegic CP (50%), and most quadriplegic CP (75%) and mixed CP (95%)

Question 9

What brain abnormalities are seen in children with CP and how common are they?

Answer 9

Neuroimaging shows brain abnormalities in 80-90%.
White matter damage is most frequent, followed by cortical or subcortical lesions (basal ganglia), brain malformations, postnatal injuries such as focal infarction

Question 10

What semi-independent motor systems are involved in CP and their frequency?

Answer 10

Pyramidal -precentral motor cortex, spinal motor neurons, corticospinal tract- accounts for 70-85% of CP
Extrapyramidal - cerebellum, basal ganglia, brain stem- accounts for 15-30% of cases

Question 11

In general, what patterns result from damage to the pyramidal system?

Answer 11

Pyramidal damage causes spastic CP, characterized by abnormally high muscle tone. Spasticity usually impairs mobility more than paresis. Upper motor neuron injury signs are present (hyperactive DTR, clonus, Babinski sign).

These children are slow, clumsy with stiff movements, walk with extended unbending legs. Cerebral injury occurring prior to physical maturation can cause limb growth arrest, arms or legs, already weak and stiff, fail to grow to their proper length and muscle structure. A short Achilles tendon forces children to walk on the toes of the affected foot.

Question 12

In general, what patterns result from damage to the extrapyramidal system?

Answer 12

Extrapyramidal damage causes abnormal involuntary movements that typically involve all extremities, upper more than lower. Many individuals with this form have normal intellectual functioning.

Question 13

What are the characteristics of spastic diplegic CP?

Answer 13

Bilateral symmetric paresis involving the legs more than the arms though hands may be clumsy. Forces children to hold their legs adducted and scissored, with feet and toes pointed downward.
Most common form of CP in children born preterm.
Usually associated with parenchymal or intraventricular hemorrhage, PVL, or both

Question 14

What are the characteristics of spastic hemiplegic CP?

Answer 14

Involves spastic hemiparesis of one side of the body, typically with greater involvement of face and arm than the leg. Resembles adults with MCA strokes, however hemiplegic CP children show premature handedness and lack of aphasia even if they have right hemiparesis

Question 15

What are the characteristics of spastic quadriplegic CP (bilateral CP)?

Answer 15

Paresis of all four limbs plus trunk and neck, usually accompanied by pseudobulbar palsy and associated with extensive cerebral damage often from anoxia (50% prenatally, 30% perinatally, 20% postnatally).
Higher prevalence of ID and epilepsy that resists treatment

Question 16

What are two subtypes of extrapyramidal CP?

Answer 16

Dyskinetic CP

Ataxic CP

Question 17

What are the characteristics of dyskinetic CP?

Answer 17

Athetoid or dystonic movements that often are caused by an underlying hypoxic-ischemic injury; abnormal movements make it difficult to sit comfortably and coordinate the muscles necessary for walking and speaking

Question 18

What are the characteristics of ataxic CP?

Answer 18

Lack of coordination during voluntary gross and fine motor movements. Typical manifestations are poor balance, unsteadiness, a wide-based gait, and shakiness or tremors during activities involving manual dexterity

Question 19

What is mixed CP?

Answer 19

motor abnormalities that reflect involvement of both pyramidal and extrapyramidal motor systems

Question 20

What are the common prenatal risk factors for CP?

Answer 20

Maternal intellectual disabilities; maternal infection or toxic exposure during pregnancy; maternal diseases during pregnancy (epilepsy, thyroid disorder, high blood pressure); multiple birth gestation; male sex of the fetus; intrauterine growth restriction; brain malformations

Question 21

What are the common perinatal risk factors for CP?

Answer 21

Prematurity is a major risk factor for brain insult

Question 22

What are the common postnatal risk factors for CP?

Answer 22

Infections (meningitis, encephalitis); head trauma

Question 23

What are common comorbidities associated with CP?

Answer 23

Epilepsy (28-50%); cognitive impairment (about half); poor growth; gastroesophageal reflux and aspiration pneumonia; oral-motor impairment; hearing loss; ocular-motor and ophthalmologic defects; urinary incontinence; pain (musculoskeletal, joint dislocation), chronic for 25-70% of adults with CP; fatigue; sleep disruption; social difficulties, depression, low self-esteem

Question 24

Mortality in CP

Answer 24

Life expectancy is shorter than general population unless CP is mild;
Respiratory disease or very low weight have higher risk;

Question 25

How is severity of CP typically defined, and what limitations exsist in grading severity?

Answer 25

There’s no definitive system or approach. One method involves categorizing mild, moderate, or severe. Most emphasize functional parameters but they remain subjective and lack specific quantitative rating criteria.

Question 26

What is the GMFCS?

Answer 26

Gross Motor Function Classification System is a widely used measure for CP severity criteria, designed for peds and emphasis on self-initiated movement, it rates degree to which children independently do developmentally appropriate sets of activities.
Ratings include 5-levels, with 1 being able to walk and climb stairs without limitation and 5 indicating severe limitation in voluntary control of movement

Question 27

What signs of CP may be apparent in the first year of life?

Answer 27

Persisting infantile reflex patterns beyond the time they’re normally suppressed; delays in achieving developmental motor milestones. However, earlier than typical acquisition of milestones can also be a sign, such as standing well before age 1 which may suggest hypertonia in legs, or strong hand preference in first 1-1.5 years

Question 28

What is the purpose of serial examinations after a diagnosis of CP in the toddler stage (1-3 years)

Answer 28

They’re useful for guiding intervention since motor signs can change as brain development and physical growth continue, and it provides prognostic information by tracking the age at which pivotal milestones are achieved

Question 29

What is predictive of eventual ambulation?

Answer 29

Sitting independently by age 2

Question 30

What trends can be seen among children (beyond toddler stage) and adolescents with CP?

Answer 30

Approximately 1/2 will outgrow the diagnosis by age 7, particularly those with mild CP that’s either monoparetic, diplegic, or extrapyramidal

Question 31

For those who outgrow motor deficits of CP in childhood, are they comparable to healthy populations in all domains?

Answer 31

No, they maintain a higher than average rate of neurological problems such as seizures and ID

Question 32

What are the major limitations to assessment of neuropsychological functioning in those with CP?

Answer 32

Impairments in speech, eye-hand coordination, manual dexterity make many instruments impossible to administer, while modified administration can compromise standardization and interpretation

Question 33

What are common trends in neuropsychological findings for individuals with CP?

Answer 33

Heterogeneous outcomes due to multiple etiologies and complications.
Always expect motor abnormalities, and most show better language-based functioning compared to visually-based as well as attention, processing speed, and EF problems associated with white matter/subcortical involvement

Question 34

How often is general cognitive functioning affected in the CP population?

Answer 34

About 75% are affected cognitively in some way, with up to 50% having ID, and 25-30% having a specific LD.
Severity of cognitive impairment is positively correlated with degree of motor impairment (but this relationship is not a perfect predictor)

Question 35

How common are hearing impairments in CP?

Answer 35

Hearing impairments are present in about 12% of individuals with CP

Question 36

How common are language impairments in CP and what factors influence language skills?

Answer 36

Oral-motor dyscontrol commonly leads to dysarthria and reduced speech intelligibility, but most people with mild to moderate CP have normal language skills otherwise.
Even in left-hemisphere damage, novel brain organization appears to allow for preserved language at the expense of visually based functions.

Question 37

What school considerations are most often required?

Answer 37

Modifications and accommodations for problems in functional mobility, sitting posture and balance when at a desk, difficulties completing independent activities, fatigue, pain and discomfort

Question 38

What school considerations are important for children with CP cognitive and social issues?

Answer 38

Special education for any cognitive and learning problems, incorporating communication devices and assistive tech, and a mechanism for completing missed work when attendance is interrupted by medical appts.
Prevention and intervention strategies for social inclusion are also important.

Question 39

What’s the focus of pharmacological interventions in CP?

Answer 39

Reduce symptoms such as spasticity, abnormal motor movement, and prevent complications such as contractures.

Question 40

What medications help reduce spasticity in CP?

Answer 40

Botox
Lioresal (baclofen)
Valium (diazepam)

Dopamine agonists may also be used to decrease abnormal movements- Sinemet (levodopa carbidopa) or artane (trihexyphenidyl)

Question 41

What limitations exist in medication treatment for CP?

Answer 41

Muscle relaxants (lioresal) effect CNS function, cause drowsiness; 
Benzos have limiting side effects such as sedation, increased drooling, ataxia, cognitive dullness

Question 42

What can improve response to muscle relaxants?

Answer 42

Intrathecal delivery of baclofen through a surgically implanted pump can allow higher doses to be tolerated

Question 43

What is APGAR?

Answer 43

Exam performed 1-5 minutes after birth to determine condition of the newborn:
Appearance (skin color)
Pulse (heart rate)
Grimace response (reflex irritability to stimulation)
Activity (muscle tone)
Respiration (breathing rate and effort)

Question 44

What APGAR score is associated with increased risk for CP?

Answer 44

Less than 3, though APGAR score alone cannot predict outcome, and many children with CP had a normal APGAR score (7, 8, 9). A perfect score of 10 rarely occurs even in healthy babies.