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Cerebral Palsy: A Multisystem Review: Page 2 of 3

Cerebral Palsy: A Multisystem Review: Page 2 of 3

MULTISYSTEMIC MANIFESTATIONS

Neurologic. CP is associated with motor and mental disabilities of variable severity in a growing child. The motor abnormalities include spasticity (spastic quadriplegia, hemiplegia, and diplegia) and movement disorders that may coexist in the same patient. Because bone growth exceeds muscle growth in the presence of spasticity in a growing child, contractures and bone deformities develop.11

Dystonia, athetosis, or both are present in 10% to 20% of patients, and children with dystonia may have eye movement and oromotor abnormalities.12 The ataxic form of CP is uncommon and presents with gait abnormalities and fine motor dysfunction.

Although not a defining feature of CP, cognitive impairment occurs in many patients and ranges from learning disabilities to attention deficit disorders to severe mental retardation. Motor development is not a reliable predictor of cognitive development.13 For example, children with athetotic CP may have normal intelligence.14

Hearing loss of the sensorineural or conductive type and visual impairment (refractory errors, visual field defects, cortical visual impairment, faulty accommodation, strabismus, nystagmus, and optic atrophy) are common.14 Other sensory defects may include impairment of touch and pain perception, proprioception, and ste- reoagnosis in spastic forms of CP.15

Seizures--typically grand mal, which often are difficult to treat14--occur in many children with CP. They usually manifest in the first year of life, more so in children with spastic hemiplegia or quadriplegia.

Musculoskeletal. Dislocations and bony deformities are not uncommon in the CP population. Spinal deformities such as kyphoscoliosis and lordosis develop in children with CP, particularly those who cannot ambulate. Incidence is as high as 60%.16 The spinal deformities progress despite skeletal maturity and may interfere with good seating posture and cause pain, pelvic misalignment, and pressure sores. Cervical and lumbar spondylosis and myelopathy may occur in athetoid CP secondary to stresses incurred by repetitive complex trunk movements.17

Nonambulators also are at high risk for osteopenia and fractures. Reduced bone mineral density is attributed mainly to immobility, poor nutrition, and use of anticonvulsant medication.18 Fractures usually occur at the metaphysis of the distal femur and proximal tibia and may be caused by contractures, falls, seizures, and overzealous physical therapy.19

Contractures at the hip and knee in particular are a source of pain and disruption to daily activities such as sitting, standing, and perineal hygiene.19 Equinus deformity occurs in almost all patients with spastic CP secondary to gastronemius and soleus muscle spasticity, which results in toe walking.

Upper limb deformities are seen in patients with spastic hemiplegia and quadriplegia. The shoulders are internally rotated and flexion contractures occur at the elbow and wrist. Forearm pronations, fisted hand with thumb in palm, and swan finger deformities also are common.

Oromotor. Oromotor muscle incoordination and spasticity may interfere with speech, suck, and swallow mechanisms and may cause temporomandibular joint contractures.15 Speech delay and dysarthria are potential sequelae. Also, dental hygiene can be difficult to maintain. Malocclusion, enamel dysplasia, and dental caries occur frequently.14,20 Gastroesophageal reflux disease (GERD) has been associated with dental erosion in CP, and degree of GERD severity has been correlated with degree of tooth erosion.21

Drooling is one of the most troublesome oromotor problems for patients with CP. Poor head control and posture, impaired lip closure, abnormal tongue movements, and oral sensory impairment may contribute.

Swallowing difficulties, GERD, and constipation also contribute to feeding problems, which may occur very early in life. In a population survey of children with CP aged 12 to 72 months, more than 90% had clinically significant oromotor dysfunction. Severe feeding problems preceded the CP diagnosis in 60% of these children.22 The undernourishment that results from feeding problems leads to growth failure.23 In those children who do not have major feeding disturbances, however, excessive weight gain can occur because of the child's inability to participate in physical activities.

Cardiopulmonary. Congential heart disease may be a component of genetically mediated CP. Pneumonia may occur frequently in the severely affected patient secondary to immobility, weak cough, contorted posture (scoliosis or chest wall deformities), GERD, and risk of aspiration.

GI. Over 90% of children with CP have associated GI symptoms, including constipation, swallowing problems, regurgitation and/or vomiting and abdominal pain.24,25 GERD, which is often difficult to control, has been found in up to 75% of patients21,26 and increases the risk of hematemesis, rumination, and dental erosion. Constipation has been associated with transit time delay in at least one segment of the colon and to ambulatory function.27

Genitourinary. Bladder difficulties often occur concurrently with bowel difficulties in children with CP. Dysfunctional voiding symptoms, such as incontinence and urinary urgency, have been noted in more than half of all patients with CP.28

Reproductive issues are often overlooked in the adolescent patient with CP. According to a recent cross-sectional survey, puberty tends to begin earlier in children with CP compared with the general population.29 However, menarche occurs later, with the median age being 14 years, as opposed to 12.8 years in the general population.29

It is important to note that children with developmental disabilities are vulnerable to sexual abuse. Findings based on data from the National Center on Child Abuse and Neglect suggest that children with disabilities are twice as likely to be sexually abused as are children in the general population.30 Furthermore, the US Department of Justice reports that up to 83% of women with developmental disabilities are sexually abused at some point in their lives.31

Behavioral. Behavioral disorders, including attention-deficit/hyperactivity disorder, are more prevalent in children with CP.32 Frustration, aggression, and mood disturbances may be part of the picture. A change in behavior may signal that the child is in pain, especially in a child with communication difficulties. The degree of pain is difficult to assess and may also manifest as sleep disturbance, decreased appetite, or intolerance of movement. Several potential sources of pain include dental caries, GERD and esophagitis, constipation, infections, and musculoskeletal abnormalities.20

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