AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Background

Definition of cerebral palsy

The term cerebral palsy (CP) was originally coined more than a century ago and loosely translates as "brain paralysis." However, a precise definition has remained elusive because CP is not a single diagnosis but an "umbrella" term describing nonprogressive brain lesions involving motor or postural abnormalities that are noted during early development (Mutch, 1992). CP has been described as follows (Bax, 2005):

A group of disorders of the development of movement and posture causing activity limitations that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, cognition, communication, perception, and/or behavior and/or a seizure disorder.

Age of onset

The brain lesions of CP occur from the fetal or neonatal period to up to age 3 years. Insults to the brain after age 3 years through adulthood may manifest clinically as similar or identical to CP, but, by definition, these lesions are not CP. Although the lesion to the developing brain occurs prior to age 3 years, the diagnosis of CP may not be made until after that time. Some authorities advocate not making a definitive diagnosis in some cases until age 5 years or later. This approach allows the clinical picture to be clear and potentially allows exclusion of progressive diseases (Shevell, 2004; Stanley, 2000). In addition, some children have been diagnosed with CP at an early age, only to have the symptoms resolve later (Jacobsson, 2004).

Lesion location

CP is restricted to lesions of the brain only; diseases specific to the peripheral nerves of the spinal cord (eg, spinal muscular atrophy, myelomeningocele) or to the muscles (eg, muscular dystrophies), although causing early motor abnormalities, are not considered CP.

Associated findings

Approximately 30-50% of patients with CP have mental retardation, depending on the type of CP (Odding, 2006; Russman, 2004). However, because of oromotor, fine motor, and gross motor difficulties, communication in CP patients may be impaired and expression of intellectual capacity may be limited. However, if CP is approached in a multidisciplinary manner, with physical, occupational, and nutritional therapy to maximize rehabilitative efforts, patients can be more fully integrated academically and socially.

Approximately 15-60% of children with CP have epilepsy, and epilepsy is more frequent in patients with spastic quadriplegia or mental retardation.

Etiology and risk factors

The etiology of CP is not well understood, and brain lesions are thought to be associated with prenatal, perinatal, or postnatal events of varying causes. Risk factors for CP are multifactorial and can include preterm birth, multiple gestation, intrauterine growth restriction, male sex, low Apgar scores, intrauterine infections, maternal thyroid abnormalities, prenatal strokes, birth asphyxia, maternal methyl mercury exposure, and maternal iodine deficiency (Stanley, 2000; Jacobsson, 2004; Nelson, 2003).

Classification and types

CP is classified according to resting tone and what limbs are involved (called topographic predominance). Spastic CP, due to cortex/pyramidal tract lesions, is the most common type and accounts for approximately 80% of cases (Stanley, 2000). Spastic CP is characterized by spasticity (velocity-dependent increase in tone), hyperreflexia, clonus, and an upgoing Babinski reflex. Extrapyramidal or dyskinetic CP is characterized more by abnormal involuntary movements.

Typical types of CP

• Spastic hemiplegia - CP predominantly affecting one side of the body, with upper extremity spasticity more than lower extremity spasticity (eg, right side involved with right arm more so than right leg)
• Spastic diplegia - CP affecting bilateral lower extremities more than upper extremities
• Spastic quadriplegia - CP affecting all 4 extremities (full body)
• Dyskinetic CP (athetoid CP, choreoathetoid CP, and dystonic CP) - CP with extrapyramidal signs characterized by abnormal movements; hypertonicity often is associated
• Mixed CP - CP with no single specific tonal quality predominating; typically characterized by a mixture of spastic and dyskinetic components
• Hypotonic CP - CP with truncal and extremity hypotonia with hyperreflexia and persistent primitive reflexes; thought to be rare

Functional classification systems generally divide patients into mild, moderate, and severe types (depending on functional limitations). Alternatively, patients may be categorized more comprehensively by their abilities and limitations, as was proposed by the World Health Organization in 2001. See International Classification of Functioning, Disability and Health.

CP is generally considered a static encephalopathy (ie, nonprogressive in nature). However, the clinical presentation of CP changes as children and their developing nervous systems mature.

Pathophysiology

The clinical presentation of CP may result from an underlying structural abnormality of the brain; early prenatal, perinatal, or postnatal injury due to vascular insufficiency; toxins or infections; or the pathophysiologic risks of prematurity. Evidence suggests that prenatal factors result in 70-80% of cases of CP. In most cases, the exact cause is unknown but is most likely multifactorial (Jacobsson, 2004).

Major events in human brain development and their peak times of occurrence (Volpe, 1995) include the following:

• Primary neurulation - Weeks 3-4 of gestation
• Prosencephalic development - Months 2-3 of gestation
• Neuronal proliferation - Months 3-4 of gestation
• Neuronal migration - Months 3-5 of gestation
• Organization - Month 5 of gestation to years postnatal
• Myelination - Birth to years postnatal

Given the complexity of prenatal and neonatal brain development, injury or abnormal development may occur at any time, resulting in the varied clinical presentations of CP (whether due to a genetic abnormality, toxic or infectious etiology, or vascular insufficiency). For example, cerebral injury before the 20th week of gestation can result in a neuronal migration deficit; injury between the 26th and 34th weeks can result in periventricular leukomalacia; injury between the 34th and 40th weeks can result in focal or multifocal cerebral injury.

Brain injury due to vascular insufficiency depends on various factors at the time of injury, including the vascular distribution to the brain, the efficiency of cerebral blood flow and regulation of blood flow, and the biochemical response of brain tissue to decreased oxygenation.

The physical stress on premature infants and the immaturity of the brain and cerebral vasculature likely explain why prematurity is a significant risk factor for CP. Prior to term, the distribution of fetal circulation to the brain results in the tendency for hypoperfusion to the periventricular white matter. Hypoperfusion can result in germinal matrix hemorrhages or periventricular leukomalacia. Between weeks 26 and 34 of gestation, the periventricular white matter areas near the lateral ventricles are most susceptible to injury. Because these areas carry fibers responsible for the motor control and muscle tone of the legs, injury can result in spastic diplegia (ie, predominant spasticity and weakness of the legs, with or without arm involvement of a lesser degree).

When larger lesions extend past the area of descending fibers from the motor cortex to involve the centrum semiovale and corona radiata, both the lower and upper extremities may be involved. Periventricular leukomalacia is generally symmetric and thought to be due to ischemic white matter injury in premature infants. Asymmetric injury to the periventricular white matter can result in one side of the body being more affected than the other. The result mimics a spastic hemiplegia but is best characterized as an asymmetric spastic diplegia. The germinal matrix capillaries in the periventricular region are particularly vulnerable to hypoxic-ischemic injury because of their location at a vascular border zone between the end zones of the striate and thalamic arteries. In addition, because they are brain capillaries, they have a high requirement for oxidative metabolism.

Many authorities grade the severity of periventricular hemorrhage-intraventricular hemorrhage using a classification system originally described by Papile et al in 1978 (see Periventricular Hemorrhage-Intraventricular Hemorrhage).

• Grade I - This is subependymal and/or germinal matrix hemorrhage.
• Grade II - This is subependymal hemorrhage with extension into the lateral ventricles without ventricular enlargement.
• Grade III - This is subependymal hemorrhage with extension into the lateral ventricles with ventricular enlargement.
• Grade IV - A germinal matrix hemorrhage that dissects and extends into the adjacent brain parenchyma, irrespective of the presence or absence of intraventricular hemorrhage, is also referred to as an intraparenchymal hemorrhage when found elsewhere in the parenchyma. Hemorrhage extending into the periventricular white matter in association with an ipsilateral germinal matrix hemorrhage/intraventricular hemorrhage is termed a periventricular hemorrhagic venous infarction.

At term, when circulation to the brain most resembles adult cerebral circulation, vascular injuries at this time tend to occur most often in the distribution of the middle cerebral artery, resulting in a spastic hemiplegic CP. However, the term brain is also susceptible to hypoperfusion, which mostly targets watershed areas of the cortex (eg, end zones of the major cerebral arteries), resulting in spastic quadriplegic CP. The basal ganglia also can be affected, resulting in extrapyramidal or dyskinetic CP.

Dyskinetic (extrapyramidal) CP is associated with several unique etiologies. Historically, kernicterus, or acute neonatal bilirubin encephalopathy, was a major cause. With improvement in early management of hyperbilirubinemia, the vast majority cases of dyskinetic CP are currently associated with presumed hypoxic ischemic injury rather than with hyperbilirubinemia (Capute, 2001). In the absence of hypoxia, hyperbilirubinemia, or prematurity, the possibility of a metabolic or neurodegenerative disorder as a basis for this presentation must be considered.

In summary, no set rules exist as to where or when the brain injury can occur, and injury may occur at more than one stage of fetal brain development. Additionally, causes are multiple and potentially multifactorial, including vascular insufficiency, infection, maternal factors, or underlying genetic abnormalities. Regardless of the etiology, however, the underlying brain anomaly in CP is static, although the motor impairment and functional consequences may vary over time. By definition, cases associated with underlying disorders of a progressive or degenerative nature are excluded when diagnosing CP.

Frequency

International

In developed countries, the overall estimated prevalence of CP is 2-2.5 cases per 1000 live births (Majnermer, 2004). The prevalence of CP among preterm and very preterm infants is substantially higher (Vincer, 2006; Ancel, 2006). In the developing world, the prevalence of CP is not well established but estimates are 1.5-5.6 cases per 1000 live births. These figures may represent an underestimation because of a paucity of data, the lack of health care access, an overrepresentation of severe cases, and inconsistent diagnostic criteria (Stanley, 2000).

Mortality/Morbidity

See Complications.

Race

CP affects persons of all races. Lower socioeconomic status may be an increased risk factor for CP (Dolk, 2001).

Sex

Male sex may be a risk factor for CP (Stanley, 2000).

CLINICAL

Section 3 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

History

CP diagnosis begins with a history of gross motor developmental delay in the first year of life (see Developmental history for milestones). CP frequently manifests as early hypotonia for the first 6 months to 1 year of life, followed by spasticity.

• Prenatal history
• • This should include information on 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 neurological disease (eg, hereditary neurodegenerative disease) also is important.
• Perinatal history: This should include gestational age (ie, degree of prematurity), 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 ultrasound, feeding difficulties, apnea, bradycardia, infection, and hyperbilirubinemia).
• Developmental history
• • This should review gross motor, fine motor, language, and social milestones from birth until the time of evaluation.
  • Gross motor milestones of concern with CP include head control at age 2 months, roll at age 4 months, sit at age 6 months, and walk at age 1 year. Infants with CP may have significantly delayed gross motor milestones or show an early hand preference when younger than 1.5 years, suggesting relative weakness of one side (eg, reaching unilaterally).
  • The presence of an unexplained regression would be more suggestive of a hereditary neurodegenerative disease than CP.
  • 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.
• Need for adaptive equipment: 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).
• General medical history: This should include a review of systems to evaluation for the multiple complications that can occur with CP (see Complications).

Physical

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

• Prior to the formal physical examination, observation may reveal abnormal neck or truncal tone (decreased or increased, depending on age and type of CP); asymmetric posture, strength, or gait; or abnormal coordination.
• Patients with CP may show increased reflexes, indicating the presence of an upper motor neuron lesion. Patients with CP also present with 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 extended and other flexed). CP 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 Developmental Disabilities in Infancy and Childhood, pages 95-100, by Capute et al.
• Patients with spastic CP evidence spasticity (ie, a velocity-dependent increase in tone). It may be evident by a tendency to keep the elbow in a flexed position or the hips flexed and adducted with the knees flexed and the valgus and ankles in equinus, resulting in toe walking.
• Patients with dyskinetic or extrapyramidal CP may have decreased head and truncal tone and defects in postural control and motor dysfunction such as the following:
• • 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)
  • Dystonia (ie, slow rhythmic movements with increased muscle tone and abnormal postures, eg, in the jaw and upper extremities)
• Classic physical presentations of the different types of CP include the following:
• • Spastic hemiplegic CP
  • • 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 affected side
    • Specific learning disabilities
    • Oromotor dysfunction
    • Possible unilateral sensory deficits
    • Visual-field deficits (eg, homonymous hemianopsia) and strabismus
    • Seizures
  • Spastic diplegic CP
  • • 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 CP
  • • 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)
    • Seizures
    • Legs generally affected equally or more than arms
    • Categorized as double hemiplegic if arms more involved than legs
  • Dyskinetic (extrapyramidal) CP
  • • 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

Causes

The etiology may be multifactorial; however, in most cases, it is unknown. Interpretation of the literature is limited by the lack of strict definitions in studies attempting to define a pathogenesis of CP and the relatively small size of certain studies. An increasing amount of literature suggests a link between various prenatal, perinatal, and postnatal factors and CP. Epidemiologic studies suggest that prenatal factors play a predominant role in the etiology of CP.

• The following maternal and prenatal risk factors statistically correlate with CP:
  • Long menstrual cycle
  • Previous pregnancy loss
  • Previous loss of newborn
  • Maternal mental retardation
  • Maternal thyroid disorder, especially iodine deficiency
  • Maternal seizure disorder
  • History of delivering a child weighing less than 2000 g
  • History of delivering a child with a motor deficit, mental retardation, or a sensory deficit
• The following factors during pregnancy also correlate statistically with CP:
  • Polyhydramnios
  • Treatment of the mother with thyroid hormone
  • Treatment of the mother with estrogen or progesterone
  • Maternal seizure disorder
  • Maternal severe proteinuria or high blood pressure
  • Maternal methyl mercury exposure
  • Congenital malformations in the fetus
  • Male sex of fetus
  • Bleeding in third trimester
  • Intrauterine growth retardation
  • Multiple gestation: The apparent overrepresentation of CP in multiple gestation pregnancies may relate more to the presence of prematurity or intrauterine growth retardation. Multiple gestations may not be an added risk for CP. The exception is when one twin dies; the surviving twin has a higher chance than a singleton of developing CP.
• The following perinatal factors are associated with an increased risk of CP:
  • Prematurity
  • Chorioamnionitis
  • Nonvertex and face presentation of the fetus
  • Birth asphyxia
    • In 10% or less of cases of CP, birth asphyxia can be determined as the definitive cause.
    • Even when birth asphyxia is thought to be associated clearly with CP, abnormal prenatal factors (eg, intrauterine growth retardation, congenital brain malformations) may have contributed to perinatal fetal distress.
    • Cases of CP attributed to birth asphyxia must document clear evidence of acidosis, moderate-to-severe neonatal encephalopathy, restriction to spastic quadriplegia, dyskinetic or mixed types of CP, and exclusion of other etiologies. Additionally, an intrapartum event must be suggested by a sentinel event, fetal heart rate changes, Apgar score less than 4 at 5 minutes, organ system damage related to tissue hypoxia, and early imaging abnormalities (American College of Obstetricians and Gynecologists, 2003).
    • While Apgar scores provide a method for documenting cardiopulmonary and neuromotor status in the minutes following birth, low scores alone cannot be used as an indicator of birth asphyxia. Such scores may reflect circumstances unrelated to birth asphyxia, such as infections and other preexisting prenatal conditions.
• The following postnatal factors may contribute to CP:
  • Infections (eg, meningitis, encephalitis)
  • Intracranial hemorrhage (eg, due to prematurity, vascular malformations, or trauma)
  • Periventricular leukomalacia (in premature infants)
  • Hypoxia-ischemia (eg, from meconium aspiration)
  • Persistent fetal circulation or persistent pulmonary hypertension of the newborn
  • Kernicterus
• Possible causes of CP by type include the following:
  • Spastic hemiplegic
    • Of all cases, 70-90% are congenital and 10-30% are acquired (eg, vascular, inflammatory, traumatic).
    • In unilateral lesions of the brain, the vascular territory most commonly affected is the middle cerebral artery; the left side is involved twice as commonly as the right.
    • Other structural brain abnormalities include hemibrain atrophy and posthemorrhagic porencephaly.
    • In premature infants, this may result from asymmetric periventricular leukomalacia.
  • Spastic diplegic
    • In premature infants, spastic diplegia may result from parenchymal-intraventricular hemorrhage or periventricular leukomalacia.
    • In term infants, no risk factors may be identifiable or the etiology might be multifactorial.
  • Spastic quadriplegic
    • Approximately 50% of cases are prenatal, 30% are perinatal, and 20% are postnatal in origin.
    • This type is associated with cavities that communicate with the lateral ventricles, multiple cystic lesions in the white matter, diffuse cortical atrophy, and hydrocephalus.
    • The patient often has a history of a difficult delivery with evidence of perinatal asphyxia.
    • Preterm infants may have periventricular leukomalacia.
    • Full-term infants may have structural brain abnormalities or cerebral hypoperfusion in a watershed (ie, major cerebral artery end zone) distribution.
  • Dyskinetic (extrapyramidal)
    • This type may be associated with hyperbilirubinemia in term infants or with prematurity without prominent hyperbilirubinemia.
    • Hypoxia affecting the basal ganglia and thalamus may affect term infants more than preterm infants.

DIFFERENTIALS

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• Workup
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Other Problems to be Considered

Hereditary spastic paraplegias
Rett syndrome
Tethered spinal cord

WORKUP

Section 5 of 11  

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• Follow-up
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• Multimedia
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Lab Studies

• The diagnosis of CP is generally made based on the clinical picture. The 2004 American Academy of Neurology (AAN) practice parameter on CP suggests laboratory studies if (1) the clinical history or findings from neuroimaging do not indicate a specific structural abnormality, (2) additional and atypical features are present in the history or clinical examination, or (3) a brain malformation is detected in a child with CP.
• • The 2004 AAN practice parameter suggests considering diagnostic testing for coagulation disorders if a cerebral infarction is seen. However, available data were insufficient for guiding what precise studies should be ordered.
  • The 2004 AAN practice parameter did not recommend an EEG unless suspicion for epilepsy or an epileptic syndrome is present.
  • If a diagnosis of a hereditary or neurodegenerative disorder is suspected, screening for an underlying metabolic or genetic disorder should be performed. However, specific studies were not recommended by the 2004 AAN practice parameters. Specific studies should be guided by the clinical picture. The following blood and urine studies may be considered:
    • Lactate and pyruvate values: Abnormalities may indicate an abnormality of energy metabolism (ie, mitochondrial cytopathy).
    • Thyroid function studies: Abnormal thyroid function may be related to abnormalities in muscle tone or deep tendon reflexes or to movement disorders.
    • Ammonia level: Elevated ammonia levels may indicate liver dysfunction or urea cycle defect.
    • Serum quantitative amino acid and urine quantitative organic acid values: These studies may reveal inherited metabolic disorders.
    • Chromosomal analysis, including karyotype analysis and specific DNA testing: These may be indicated to rule out a genetic syndrome, if dysmorphic features or abnormalities of various organ systems are present.

Imaging Studies

• Cranial ultrasonography performed in the early neonatal period can be helpful in medically unstable infants until they are able to tolerate transport for more detailed neuroimaging. Ultrasonography can delineate clear-cut structural abnormalities and show evidence of hemorrhage or hypoxic-ischemic injury.
• CT scanning of the brain helps identify congenital malformations, intracranial hemorrhage, and periventricular leukomalacia in infants more clearly than ultrasonography.
• MRI of the brain is the diagnostic neuroimaging study of choice for older children because it defines cortical and white matter structures and abnormalities more clearly than any other method. It also allows for the determination of appropriate myelination for a given age.
• At this time, the AAN 2004 practice parameter recommends neuroimaging "to establish that a brain abnormality exists in children with CP, that may, in turn, suggest an etiology and prognosis."
  • MRI is preferred over CT scanning.
  • Although the precise role for MRI in the diagnosis and workup of children with CP or suspected CP has not been fully elucidated, recent literature suggests that MRI should be strongly considered in all cases; in one study, 89% children with CP were found to have abnormal MRIs (Bax, 2006).
  • Additionally, MRI may have a role in predicting neurodevelopmental outcomes in preterm infants (Woodward, 2006).
• Head ultrasonography, CT scanning, and MRI may be helpful for diagnosing and monitoring findings of hydrocephalus.
• In children with spasticity of the legs and worsening of bowel and bladder function, a spine MRI may help identify a tethered spinal cord.
• Patients who present clinically with CP may have normal results from brain imaging studies. Normal results from a neuroimaging studies do not exclude a clinical diagnosis of CP. However, in these cases, other underlying metabolic and genetic etiologies should be considered and excluded.

Other Tests

• EEG is important in the diagnosis of seizure disorders but is not indicated if seizures are not suspected along with CP.
• Electromyography and nerve conduction studies are helpful when a muscle or nerve disorder is suspected (eg, a hereditary motor or sensory neuropathy as a basis for equinus foot deformities and toe walking).

Histologic Findings

Periventricular leukomalacia refers to foci of coagulative necrosis in the white matter adjacent to the lateral ventricles.

TREATMENT

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Medical Care

The physician's responsibility is to closely supervise and manage the multiple medical complications associated with CP (see Complications).

• The 2004 AAN practice parameter suggests screening for the following potential CP-associated deficits at the initial assessment:
  • Mental retardation
  • Ophthalmologic and hearing impairments
  • Speech and language disorders
  • Oromotor dysfunction
• Various medications may improve spasticity.
• Numerous medications, although often used off label for age and indication, may relieve the movement difficulties associated with CP. These drugs target dystonia, myoclonus, chorea, athetosis, and spasticity.
• While antiparkinsonian drugs (eg, anticholinergic and dopaminergic drugs) and antispasticity agents (eg, baclofen) have primarily been used in the management of dystonia, anticonvulsants, antidopaminergic drugs, and antidepressants have also been tried.
• Anticonvulsants (including benzodiazepines such as diazepam, valproic acid, and barbiturates) have been useful in the management of myoclonus. Chorea and athetosis are often difficult to manage, although benzodiazepines, neuroleptics, and antiparkinsonian drugs (eg, levodopa) have been tried. Benzodiazepines and baclofen are commonly used to manage spasticity.
• Neurologists and rehabilitation medicine specialists (physiatrists) play significant roles in the management of antispasticity medications.
• Seizure disorders are common in persons with CP, and the clinician should be comfortable with the management of anticonvulsant medications (see Antiepileptic Drugs: An Overview).

Consultations

• A rehabilitation medicine specialist (physiatrist) should be consulted for the evaluation and management of the rehabilitation program (eg, equipment, coordination of therapy, spasticity management). Phenol intramuscular neurolysis and botulinum A toxin (BOTOX®) intramuscular blocks may reduce spasticity for 3-6 months. This can allow for improved range of motion, reduced deformity, improved response to occupational and physical therapy, and delay in the need for surgical management of spasticity.
• An orthopedist should be consulted for the surgical management of hip dislocation, scoliosis, and spasticity (eg, tenotomy, a tendon-lengthening procedure).
• A geneticist should be consulted to evaluate for an underlying genetic syndrome, particularly in the setting of dysmorphic features, multiple organ abnormalities, or a family history of a similar neurological syndrome.
• A neurosurgeon should be consulted for identifying and treating hydrocephalus, a tethered spinal cord, or spasticity.
  • By cutting I-a sensory fibers, selective dorsal rhizotomy decreases spasticity by decreasing reflexive motoneuron activation, which is thought to result from the lack of descending fiber input.
  • Intrathecal baclofen can be administered via a pump implanted by a neurosurgeon. The pump is placed in the anterior abdominal wall and connects to a catheter inserted in the subarachnoid space overlying the conus of the spinal cord. Intrathecal baclofen can allow more local presynaptic inhibition of I-a sensory afferents and has fewer adverse effects than oral baclofen.
  • Although data are limited in this population, stereotactic basal ganglia surgery may improve rigidity, choreoathetosis, and tremor.
  • Additionally, reconstructive surgery to the upper extremities can restore muscle balance, release contractures, and stabilize joints to improve placement of the hand in space, as well as voluntary grasp, release, and pinch functions.
• A gastroenterologist, nutritionist, and a feeding and swallowing team should be consulted for management of feeding and swallowing difficulties and gastroesophageal reflux and for assessment of nutritional status.
• A pulmonologist should be consulted for the management of chronic pulmonary disease due to bronchopulmonary dysplasia and frequent or recurrent aspiration.
• A multidisciplinary learning disability team specializing in children with special needs should be consulted to identify specific learning disabilities, monitor cognitive progression, and guide services through early intervention and school. The child should be evaluated by a communication enhancement center to guide speech and language treatment and the use of communicative devices.

Diet

Oromotor dysfunction may require limitations in the texture of food and liquid, feeding only by gastrostomy or jejunostomy tube, supplemental feedings via gastrostomy or jejunostomy tube to increase energy intake, and aspiration precautions.

Activity

Regular physical therapy and occupational therapy are crucial. The goal should be to maximize the functional use of limbs and ambulation and to reduce the risk of contractures.

MEDICATION

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The goal of pharmacotherapy is to reduce symptoms (eg, spasticity) and prevent complications (eg, contractures). Most of the medications used for CP in children are off label for age and indication and should be used only by physicians experienced in their use and familiar with their adverse effects. The indications and doses listed are from a general formulary. A wide range of dosing can be encountered in clinical practice because information in the literature regarding medication for CP in children is scant.

Drug Category: Benzodiazepines

Used in the acute management of seizures that may accompany CP. By binding to specific receptor sites, these agents appear to potentiate the effects of GABA and facilitate neurotransmission of GABA and other inhibitory transmitters.

Drug Category: Anticonvulsants

Used to terminate clinical and electrical seizure activity as rapidly as possible and to prevent seizure recurrence.

Drug Category: Dopamine prodrugs

Dopamine does not cross blood-brain barrier, but levodopa (L-dopa, the metabolic precursor of dopamine) does. L-dopa is decarboxylated to dopamine in the brain and in the periphery. The formation of dopamine in the blood causes many of the adverse effects associated with L-dopa. When administered alone, levodopa induces a high incidence of nausea and vomiting.

A peripheral decarboxylase inhibitor such as carbidopa is combined with levodopa to reduce the incidence of nausea and vomiting by inhibiting the peripheral conversion of levodopa to dopamine. Levodopa/peripheral decarboxylase inhibitor is the criterion standard of symptomatic treatment for Parkinson disease; it provides the greatest antiparkinsonian efficacy in moderate-to-advanced disease with the fewest acute adverse effects.

Drug Category: Anticholinergics

Provide benefit for tremor in approximately 50% of patients but do not improve bradykinesia or rigidity. If 1 anticholinergic does not work, try another. Adverse effects include dry mouth and dry eyes, memory difficulty, confusion, and rare urinary retention.

Drug Category: Toxins

Botulinum toxin type A is DOC. It causes presynaptic paralysis of myoneural junctions and reduces abnormal contractions. Therapeutic effects may last 3-6 months.

Drug Category: Muscle relaxants

The muscle-relaxing effects may come from inhibition of the transmission of monosynaptic and polysynaptic reflexes at the spinal cord level.

FOLLOW-UP

Section 8 of 11  

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• Medication
• Follow-up
• Miscellaneous
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Further Outpatient Care

• Habilitation is a "comprehensive intervention strategy designed to facilitate adaptation to and participation in an increasing number and variety of settings in a particular society and culture."
• Management often requires the involvement of multiple specialists (see Consultations) in addition to close neurological follow-up.
• Multidisciplinary CP clinics can allow for the frequent, comprehensive follow-up of children with CP while decreasing the need for patient travel.

Complications

• Gastrointestinal and nutritional
  • Failure to thrive due to feeding and swallowing difficulties secondary to poor oromotor control
  • Obesity, less frequently than failure to thrive: Maintaining weight close to idea body weight is important for wheelchair-bound patients or those with ambulatory dysfunction.
  • Gastroesophageal reflux and associated aspiration pneumonia
  • Constipation
  • Dental caries
• Respiratory
  • Increased risk of aspiration pneumonia because of oromotor dysfunction
  • Chronic lung disease/bronchopulmonary dysplasia
  • Bronchiolitis/asthma
• Skin - Decubitus ulcers and sores
• Orthopedic
  • Contractures
  • Hip dislocation
  • Scoliosis
• Neurologic
  • Epilepsy: Epilepsy occurs in 15-60% of children with CP and is more common in patients with spastic quadriplegia or mental retardation. When compared with controls, children with CP have a higher incidence of epilepsy with onset within the first year of life and are more likely to have a history of neonatal seizures, status epilepticus, polytherapy, and treatment with second-line anticonvulsants. Factors associated with a seizure-free period of at least 1 year include normal intelligence, single seizure type, monotherapy, and spastic diplegia.
  • Hearing loss: This occurs particularly in patients who had acute bilirubin encephalopathy (kernicterus).
  • Vision: Visual acuity decreases in premature infants because of retinopathy of prematurity with hypervascularization and possible retinal detachment.
  • Visual-field abnormalities due to cortical injury
  • Strabismus
• Cognitive/psychological/behavioral
  • Mental retardation (30-50%)
  • Attention-deficit/hyperactivity disorder
  • Learning disabilities
  • Impact on academic performance and self-esteem
  • Increased prevalence of depression
  • Sensory integration difficulties
  • Increased prevalence of progressive development disorder or autism associated with concurrent diagnosis of CP

Prognosis

• Patients with severe forms of CP may have a significantly reduced life span. Patients with milder forms of CP have a life expectancy close to the general population, although still somewhat reduced (Hemming, 2006; Hemming, 2005; Hutton, 2006).
• Morbidity and mortality relate to the severity of CP and concomitant medical complications, such as respiratory and GI difficulties. In patients with quadriplegia, the likelihood of epilepsy, extrapyramidal abnormalities, and severe cognitive impairment is greater than in those with diplegia or hemiplegia.
• Cognitive impairment occurs more frequently in persons with CP than in the general population. The overall rate of mental retardation in persons with CP is thought to be 30-50%. Some form of learning disability (including mental retardation) has been estimated to occur in perhaps 75% of patients. However, standardized cognitive testing primarily evaluates verbal skills and may result in the underestimation of cognitive abilities in some individuals.
• In some studies, 25% of patients with CP are unable to walk. However, many patients with CP (particularly those with spastic diplegia and spastic hemiplegia types) can ambulate independently or with assistive equipment. A prospective study of children has suggested that being able to sit by age 2 years is a good predictive sign of eventual ambulation. Children who did not sit by age 4 years did not ambulate.
• In patients with spastic quadriplegia, a less favorable prognosis correlated with a longer delay in the resolution of extensor tone. At times, hypertonicity and spasticity may improve or resolve over time in patients with CP. Spasticity in patients with spastic quadriplegia can be more resistant even with services and orthopedic and rehabilitative intervention. Approximately 25% of children with CP have mild involvement with minimal or no functional limitation in ambulation, self-care, and other activities. Approximately half are moderately impaired to the extent that complete independence is unlikely but function is satisfactory. Only 25% are so severely disabled that they require extensive care and are nonambulatory. Maintenance of sitting by age 2 years and suppression of obligatory infantile reflexes by age 18 months are thought to be good prognostic indicators of eventual ambulation.
• As the brain continues to develop postnatally, abnormalities of motor tone or movement in the first several weeks or months after birth may gradually improve over the first year of life (or even later). The Collaborative Perinatal Project found that almost 50% of individuals diagnosed with CP and 66% of children diagnosed with spastic diplegia "outgrew" findings that were suggestive of CP by age 7 years. Others do not manifest full motor signs suggestive of CP until aged 1-2 years. Thus, some propose that the diagnosis of CP should be deferred until the child is aged 2 years or older.
• With appropriate therapeutic services, patients may be able to fully integrate academically and socially.

Patient Education

• For excellent patient education resources, visit eMedicine's Brain and Nervous System Center. In addition, see eMedicine's patient education article Cerebral Palsy.

MISCELLANEOUS

Section 9 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical/Legal Pitfalls

• Medicolegal issues are outlined extensively in a 1997 review article by Perlman. Obstetricians are at risk of malpractice claims because of the association of CP with birth asphyxia, even though most CP cases are thought to be caused by prenatal insult.
• To determine the presence of medical negligence related to birth asphyxia, the following must be documented:
• • An adverse outcome occurred (eg, CP as a consequence of intrapartum asphyxia).
  • Standard of care was breached during labor or delivery, directly causing the asphyxia.
  • An alternative medical strategy more likely than not would have altered the outcome in a positive fashion.
• To ascribe the cause of CP to intrapartum asphyxia, the following must not be present: (1) clinical evidence indicating any potential antenatal injury, (2) neuroimaging evidence of antenatal cerebral injury, (3) clinical evidence of severe perinatal asphyxia, and (4) evidence of other causes of neonatal encephalopathy.
• Given that prenatal factors greatly outnumber perinatal and postnatal factors in the origin of CP and that prenatal factors are difficult to isolate from perinatal and postnatal factors as a cause for CP, determining causality due to intrapartum asphyxia or medical neglect is difficult.

Special Concerns

• Advances in neonatal neurology continue to focus on potentially modifiable factors during the neonatal period that contribute to the development of CP. Many of these studies focus on the role of excitable amino acids and their role in neurological injury. The hope is that more can be done in the neonatal period to prevent the permanent neurologic deficit resulting in CP.

MULTIMEDIA

Section 10 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Media file 1:  MRI of a 1-year-old boy who was born at gestational week 27. Clinical examination is consistent with spastic diplegic cerebral palsy. Pseudocolpocephaly and decreased volume of the white matter posteriorly are consistent with periventricular leukomalacia. Evidence of diffuse polymicrogyria and thinning of the corpus callosum is noted.
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Media type:  MRI

Media file 2:  MRI of a 16-month-old boy who was born at term but had an anoxic event at delivery. Examination findings are consistent with a spastic quadriplegic cerebral palsy with asymmetry (more prominent right-sided deficits). Cystic encephalomalacia in the left temporal and parietal regions, delayed myelination, decreased white matter volume, and enlarged ventricles can be seen. These findings are most likely the sequelae of a neonatal insult (eg, periventricular leukomalacia with a superimposed left-sided cerebral infarct).
	View Full Size Image
Media type:  MRI

Media file 3:  MRI of a 9-day-old girl who was born full-term and had a perinatal hypoxic-ischemic event. Examination of the patient at 1 year revealed findings consistent with a mixed quadriparetic cerebral palsy notable for dystonia and spasticity. Severe hypoxic-ischemic injury to the medial aspect of the cerebellar hemispheres, medial temporal lobes, bilateral thalami, and bilateral corona radiata is observed.
	View Full Size Image
Media type:  MRI

REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

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