Cerebral Palsy

CHAPTER 61

Cerebral Palsy

Introduction/Etiology/Epidemiology

• Cerebral palsy is a term to describe a group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to nonprogressive disturbances in the developing fetal or infant brain.

• The motor disorders of cerebral palsy are often accompanied by changes in sensation, perception, cognition, communication, and behavior.

• Affecting 1 in 500 neonates, cerebral palsy is the most common childhood-onset neuromuscular disorder, with an estimated prevalence of 17 million people worldwide.

• The brain pathology in cerebral palsy involves the white matter, with periventricular leukomalacia being the most common finding in magnetic resonance imaging (MRI) studies.

• Cerebral palsy may result from events occurring any time from the prenatal period to 2 years of age.

• Prenatal risk factors include infection, substance use, an incompetent cervix, and third-trimester bleeding.

• Perinatal risk factors include preterm birth, low birth weight, multiple births, placental abruption, premature rupture of membranes, infection, birth trauma, perinatal stroke, and hypoxia.

— Less than 10% of cerebral palsy cases are believed to result from perinatal events during delivery.

• Postnatal risk factors include central nervous system infection and any event that results in hypoxia to the immature brain.

Signs and Symptoms

• Developmental delay and failure to meet milestones (eg, head up, pushes up on forearms at 3 months) combined with persistence of primitive reflexes and/or muscle tone abnormalities

• Gait patterns associated with cerebral palsy include equinus gait (toe walking), scissoring gait, crouch gait, and stiff knee gait.

• Most patients have neurologic manifestations such as impaired sensation, cognition, communication, perception, and behavior.

• Seizure disorders are common.

• Gastrointestinal problems are also common, including impaired swallowing, gastroesophageal reflux, and disordered motility, which may result in malnutrition.

Differential Diagnosis

• Metabolic, genetic, or progressive neurodegenerative disorders must be excluded. Unlike in these disorders, in patients with cerebral palsy the brain lesion is static.

Diagnostic Considerations

• The diagnosis is established based on history and physical examination findings.

• MRI of the brain should be performed to rule out other neurologic disorders and confirm the clinical diagnosis of cerebral palsy as appropriate.

— Brain MRI findings are abnormal in 85% of patients with cerebral palsy, with periventricular leukomalacia the most common abnormality.

• Classification can help guide treatment and improve prognostic counseling.

• Topographic classification (Figure 61-1) reflects the location of insult to the motor cortex and pattern of limb involvement.

— Hemiplegia: involvement of one side of the body, with the upper limb more affected than the lower limb. Often associated with perinatal ischemic stroke. Common manifestations are equinus and stiff knee gait. Most patients ambulate independently.

— Diplegia: Both lower limbs are much more affected than upper limbs. Often accompanied by periventricular white matter loss. Most patients ambulate, but many require assistive devices.

— Quadriplegia: All 4 limbs and the trunk are involved. Associated with severe birth asphyxia. Patients are rarely able to functionally ambulate.

• Physiologic classification describes nature of movement disorder

— Spasticity: velocity-dependent increase in muscle tone in response to stretch and hyperexcitable stretch reflexes because of upper motor neuron dysfunction

■Most common form of cerebral palsy

■Spasticity often results in poor motor control, decreased balance, and weakness.

■Joint contractures frequently develop over time.

Figure 61-1. Topographical classification of cerebral palsy depicts the pattern of limb involvement arising from location of insult to the motor cortex.

Reprinted by permission from Springer Nature: Nature Reviews Disease Primers. Graham HK, Rosenbaum P, Paneth N, et al: Cerebral Palsy. Nat Rev Dis Primers. 2016;2:15082. © Jan 7, 2016

— Dystonia: sustained or intermittent muscle contractions causing abnormal, repetitive movements and/or postures

— Chorea: jerky, dance-like movements

— Athetosis: slow, writhing movements

— Ataxia: difficulty with coordinated movements and balance

— Hypotonia: low muscle tone with abnormal reflexes

— Mixed: a combination of these types in varying degrees

• The Gross Motor Function Classification System (GMFCS) (Table 61-1) is the most clinically relevant classification, used to describe motor function and predict outcome.

— Five-level grading system based on self-initiated movement, emphasizing function with regard to sitting and walking

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