Cerebral Palsy Clinical Presentation

The child with cerebral palsy can present after failing to meet expected developmental milestones or failing to suppress obligatory primitive reflexes. The 2003 American Academy of Neurology (AAN) practice parameter suggests screening for the following potential cerebral palsy–associated deficits at the initial assessment^[16] :

• Mental retardation
• Ophthalmologic and hearing impairments
• Speech and language disorders
• Oromotor dysfunction

The diagnosis begins with a history of gross motor developmental delay in the first year of life. Cerebral palsy frequently manifests as early hypotonia for the first 6 months to 1 year of life, followed by spasticity.

Abnormal muscle tone is the most frequently observed symptom. The child may present as either hypotonic or, more commonly, hypertonic with either decreased or increased resistance to passive movements, respectively. Children with cerebral palsy may have an early period of hypotonia followed by hypertonia. The longer the period of hypotonia before hypertonia, the greater the likelihood that the hypertonia will be more severe.

Definite hand preference before age 1 year is a red flag for possible hemiplegia. Asymmetric crawling or failure to crawl may also suggest cerebral palsy. Growth disturbance is often noted in children with cerebral palsy, especially failure to thrive.

The general medical history should include a review of systems to evaluate for the multiple complications that can occur with cerebral palsy (see Complications under Prognosis).

Prenatal history

The prenatal history should include information on the mother's pregnancy, such as prenatal exposure to illicit drugs, toxins, or infections; maternal diabetes; acute maternal illness; trauma; radiation exposure; prenatal care; and fetal movements.

A history of early frequent spontaneous abortions, parental consanguinity, and a family history of neurologic disease (eg, hereditary neurodegenerative disease) is also important.

Perinatal history

The perinatal history should include the child's gestational age (ie, degree of prematurity) at birth, presentation of the child and delivery type, birth weight, Apgar score, and complications in the neonatal period (eg, intubation time, presence of intracranial hemorrhage on neonatal ultrasonogram, feeding difficulties, apnea, bradycardia, infection, and hyperbilirubinemia).

Developmental history

The child's developmental history should review his/her gross motor, fine motor, language, and social milestones from birth until the time of evaluation.

Gross motor milestones of concern with cerebral palsy include head control at age 2 months, rolling at age 4 months, sitting at age 6 months, and walking at age 1 year. Infants with cerebral palsy may have significantly delayed gross motor milestones or show an early hand preference when younger than 1.5 years, suggesting the relative weakness of one side (eg, reaching unilaterally).

The presence of an unexplained regression would be more suggestive of a hereditary neurodegenerative disease than cerebral palsy.

Current social skills, academic performance, and participation in an early intervention program (if < 3 y) or school support (if >3 y) should be reviewed, including resource room assistance; physical, occupational, and speech and language therapy; and adaptive physical education.

Standardized cognitive and educational testing and a current individualized education plan can be used to determine whether speech therapy, occupational therapy, and physical therapy are in place or whether referrals for these are needed.

Review the patient's equipment or need for equipment such as adaptive and communication devices (eg, computer-assisted speech programs), orthotics (eg, ankle-foot orthoses, walkers, wheelchair), and/or seating (may require straps to keep in place). See Rehabilitation and Cerebral Palsy.

Physical indicators of cerebral palsy include joint contractures secondary to spastic muscles, hypotonic to spastic tone, growth delay, and persistent primitive reflexes.

The initial presentation of cerebral palsy includes early hypotonia, followed by spasticity. Generally, spasticity does not manifest until at least 6 months to 1 year of life. The neurologic evaluation includes close observation and a formal neurologic examination.

Before the formal physical examination, observation may reveal abnormal neck or truncal tone (decreased or increased, depending on age and type of cerebral palsy); asymmetric posture, strength, or gait; or abnormal coordination.

Patients with cerebral palsy may show increased reflexes, indicating the presence of an upper motor neuron lesion. This condition may also present as the persistence of primitive reflexes, such as the Moro (startle reflex) and asymmetric tonic neck reflexes (ie, fencing posture with neck turned in same direction when one arm is extended and the other is flexed). Symmetric tonic neck, palmar grasp, tonic labyrinthine, and foot placement reflexes are also noted. The Moro and tonic labyrinthine reflexes should extinguish by the time the infant is aged 4-6 months; the palmar grasp reflex, by 5-6 months; the asymmetric and symmetric tonic neck reflexes, by 6-7 months; and the foot placement reflex, before 12 months. Cerebral palsy may also include the underdevelopment or absence of postural or protective reflexes (extending arm when sitting up). For a good discussion of this topic, see Capute AJ, Accardo PJ, eds. Developmental Disabilities in Infancy andChildhood. 2^nd ed.2001;95-100.^[17]

The overall gait pattern should be observed and each joint in the lower extremity and upper extremity should be assessed, as follows:

• Hip – Excessive flexion, adduction, and femoral anteversion make up the predominant motor pattern. Scissoring of the legs is common in spastic cerebral palsy.
• Knee – Flexion and extension with valgus or varus stress occur.
• Foot – Equinus, or toe walking, and varus or valgus of the hindfoot is common in cerebral palsy.

Gait abnormalities may include the crouch position with tight hip flexors and hamstrings, weak quadriceps, and/or excessive dorsiflexion.

Spastic (pyramidal) cerebral palsy

Patients with spastic (pyramidal) cerebral palsy evidence spasticity (ie, a velocity-dependent increase in tone) and constitute 75% of patients with cerebral palsy. Patients have signs of upper motor neuron involvement, including hyperreflexia, clonus, extensor Babinski response, persistent primitive reflexes, and overflow reflexes (crossed adductor). This may be observed by the child's tendency to keep the elbow in a flexed position or the hips flexed and adducted with the knees flexed and in valgus, and the ankles in equinus, resulting in toe walking.

Dyskinetic (extrapyramidal) cerebral palsy

Dyskinetic (extrapyramidal) cerebral palsy is characterized by extrapyramidal movement patterns, abnormal regulation of tone, abnormal postural control, and coordination deficits. Abnormal movement patterns may increase with stress or purposeful activity. Muscle tone is usually normal during sleep. Intelligence is normal in 78% of patients with athetoid cerebral palsy. A high incidence of sensorineural hearing loss is reported. Patients often have pseudobulbar involvement, with dysarthria, swallowing difficulties, drooling, oromotor difficulties, and abnormal speech patterns. Thus, the classic physical presentations of dyskinetic cerebral palsy include the following:

• Early hypotonia with movement disorder emerging at age 1-3 years
• Arms more affected than legs
• Deep tendon reflexes usually normal to slightly increased
• Some spasticity
• Oromotor dysfunction
• Gait difficulties
• Truncal instability
• Risk of deafness in those affected by kernicterus

These patients with dyskinetic cerebral palsy may have decreased head and truncal tone and defects in postural control and motor dysfunction such as athetosis (ie, slow, writhing, involuntary movements, particularly in the distal extremities), chorea (ie, abrupt, irregular, jerky movements) or choreoathetosis (ie, combination of athetosis and choreiform movements), and dystonia (ie, slow, sometimes rhythmic movements with increased muscle tone and abnormal postures, eg, in the jaw and upper extremities)

Spastic hemiplegic cerebral palsy

Hemiplegia is characterized by weak hip flexion and ankle dorsiflexion, an overactive posterior tibialis muscle, hip hiking/circumduction, supinated foot in stance, upper extremity posturing (that is, often held with the shoulder adducted, elbow flexed, forearm pronated, wrist flexed, hand clenched in a fist with the thumb in the palm), impaired sensation, impaired 2-point discrimination, and/or impaired position sense. Some cognitive impairment is found in about 28% of these patients. Thus, spastic hemiplegic cerebral palsy includes the following classic physical presentations:

• One-sided upper motor neuron deficit
• Arm generally affected more than leg; possible early hand preference or relative weakness on one side; gait possibly characterized by circumduction of lower extremity on the affected side
• Specific learning disabilities
• Oromotor dysfunction
• Possible unilateral sensory deficits
• Visual-field deficits (eg, homonymous hemianopsia) and strabismus
• Seizures

Spastic diplegic cerebral palsy

Patients with spastic diplegia often have a period of hypotonia followed by extensor spasticity in the lower extremities, with little or no functional limitation of the upper extremities. Patients have a delay in developing gross motor skills. Spastic muscle imbalance often causes persistence of infantile coxa valga and femoral anteversion. Cognitive impairment is present in approximately 30% of spastic diplegic patients. Spastic diplegic cerebral palsy includes the following classic physical presentations:

• Upper motor neuron findings in the legs more than the arms
• Scissoring gait pattern with hips flexed and adducted, knees flexed with valgus, and ankles in equinus, resulting in toe walking
• Learning disabilities and seizures less commonly than in spastic hemiplegia

Spastic quadriplegic cerebral palsy

Most patients with spastic quadriplegic cerebral palsy have some cognitive impairment and demonstrate the following classic physical presentations:

• All limbs affected, either full-body hypertonia or truncal hypotonia with extremity hypertonia
• Oromotor dysfunction
• Increased risk of cognitive difficulties
• Multiple medical complications (see Complications under Prognosis)
• Seizures
• Legs generally affected equally or more than arms
• Categorized as double hemiplegic if arms more involved than legs