Cerebral Palsy

Cerebral palsy (CP) is the most common condition that pediatric physiatrists will manage, although it is not the most common reason for childhood disability, as noted earlier. There are estimates of about 500,000 people in the United States with CP.

The health of adults with CP is generally good. Although cerebral palsy may affect multiple organ systems, in general, the long-term health problems are related to pain, fatigue, and the musculoskeletal sys­tem (see Table 15.2).

Mortality

Mortality for people with CP appears to be related to severity of impairments. This is very clear in the pediatric population, but less so for adults who have survived into their late twenties and thirties. There is also an obvious cohort bias when comparing mortal­ity data from those born prior to the 1980s to mortality data of a younger adult population, and it is not clear that the information about the adults of today may be used specifically for predicting life expectancies.

431

Continued

433

Continued

434

15.2

Aging Health and Performance Changes (Continued)

DEXA, dual energy X-ray absorptiometry; BTX, botulinum toxin; ITB, intrathecal baclofen; UTI, urinary tract infection; CIC, clean intermittent catheterization; GERD, gastroesophageal reflux disease; AD, autonomic dysreflexia; CVD/AVD, cardiovascular disease/atherosclerotic vascular disease; TSH, thyroid-stimulating hormone.

435

Through a large database in California defined by financial and service support needed and especially representative of the more severely impaired individu­als with CP, survival of higher-functioning adults was close to that of the general population (26).

Reports from abroad also identify life expectan­cies for adults with CP to be close to the general pop­ulation for those with mild to moderate impairments. The Western Australia Cerebral Palsy Registry noted the strongest single predictor of mortality was intel­lectual disability, with survival exceeding 92% for IQ/ DQ scores >34 (30). This study noted motor impair­ment severity increased the risk of early mortal­ity, with mortality declining after age 5 to 15 years, and remaining steady at 0.35% for the next 20 years. Providing insights on era of disability onset, Hemming et al reported on adults with CP in the 1940-1950 birth cohort in the UK. Assuming survival to age 20 years, almost 85% survived to age 50 years compared to 96% of the general population (31). Again comparing to the general population, many of the deaths noted in ages 20s-30s were respiratory, and deaths in ages 40s-50s were circulatory conditions and neoplasms. Few deaths in adulthood were attributed to CP, although the ner­vous system was implicated more than in the general population.

Health and Functional Status

The general health of adults with CP is self-reported as good or satisfactory to excellent (32,33), and this can be comparable to that of the community at large (16). In a population-based study of adults with cere­bral palsy in a mid-sized metropolitan area, persons with cerebral palsy were generally healthy (based on clinical information and self-report), but noted wor­ries and concerns about their health status and futures (34). Self-perceived health ratings and life satisfac­tion may be related to the presence of pain or func­tional changes over time, but not to the severity of impairment (35-37). Despite reports of good health, a Canadian publication notes adults with CP attended outpatient physician visits 1.9 times higher than age- matched peers (38).

The functional status of adults with CP is not static over time, and with aging there can be modest decreasing function, as there is for the general popu­lation. A number of studies, both in the United States and abroad, with small to large convenient samples, have noted that about a third of subjects report modest to significant decreases in walking or self-care tasks (16,27,39-41). Changes in dressing and walking with relative sparing of other self-care or social activities were reported in two of these studies (16,27). Day et al used the large California database to determine the probabilities of loss or gain of walking skills into adult­hood for those with CP (42). They noted that by age 25 years, there would unlikely be any improvement in walking skill and most would not change over the next 15 years, although there could be some decline.

Decreased independence (increased need for assis­tance) in mobility and self-care is a common complaint of adults with mobility impairments. The reasons for change are varied, and may include those related to age changes (eg, decreased endurance, flexibility, strength, or balance), progressive pathology or second­ary conditions (eg, pain, contractures, spasticity, oste­oporosis and fractures, stenosis), or personal choices (eg, use of powered mobility to conserve energy). The change in mobility is often a response to a second­ary condition or age-related change. Falls may also be such a response. Significant change in mobility or falls should not automatically be accepted as a part of a congenital or childhood-onset disabling condition in adult years; treatable etiologies should be sought.

It has been suggested through cross-sectional and convenience samples that adults with congenital or childhood-onset disabilities may show musculo­skeletal or performance changes typical of advanced aging earlier than their nondisabled peers (32,26,43). These observations require confirmation through longitudinal controlled studies. While risk factors may predispose a person to these changes, they are, as yet, unproven. If these earlier-than-expected aging changes are confirmed, they should be considered sec­ondary conditions.

Pain and Fatigue

Pain is the most consistent health condition reported by adults with cerebral palsy (17,32,44,45). It has been reported in a number of samples of adults with CP at a variety of ages to be 30% to 80%, with activity limitation from this at >50%. For this reason, it will be covered as a separate topic. Pain may be present for a variety of reasons; it may be acute, recurrent, or chronic. Increased spasticity, weakness, falls, or pro­gression of contractures or deformities can result from pain, particularly when pain is not reported because of communication difficulties or severe intellectual dis­ability. Because of the high prevalence, the health care provider should try to elicit complaints or indications of pain, and evaluation, diagnosis, and intervention should ensue. Pain is often the reason for a change in function, living arrangement, or social interaction.

Pain is usually identified by proximity to a joint, and less often a limb. Most people report “arthritis” as the etiology of these pain complaints; however, these pains may originate from either joints or muscles. A good history and clinical exam will help sort out the issues and direct appropriate treatment. Back, leg, and hip pain complaints are common in persons with cerebral palsy (46,47). There are usually more pain complaints in those with spasticity (46). It has been reported that fatigue often incites pain, and exercise most commonly relieves pain (46,48).

Fatigue is a common complaint of adults with CP, and is associated with pain (49). It is also associated with deterioration of skills and low life satisfaction, with no association with any specific type or severity of CP. As noted, it may incite pain. The fatigue may also be associ­ated with the reported coping strategies sometimes used for chronic pain by adults with CP (50). Sleep disruption should also be questioned since it is commonly seen with pain and fatigue. Anecdotally, the pain/fatigue complex appears to respond positively to directed pain manage­ment, good sleep hygiene, medications, and exercise.

Appropriate management includes early identifi­cation of the problem and its source. Common mus­culoskeletal etiologies include poor ergonomics and biomechanics in tasks (secondary to deformity or limited motor control (41)), underlying weakness and therefore overuse (51), hypertonia (52), and degenera­tive joint disease (53). Typical management strategies should be offered, and referral for additional inter­ventional, orthopedic, or neurosurgical consultation should be considered. However, adults with CP tend to self-manage their pain complaints (54), and for those who seek medical care, report is minimal improve­ment and few options offered (55).

Musculoskeletal and Neurologic Conditions

Contractures. Contractures are a common secondary condition, and reported in multiple case series. Their impact on functional status or general health care needs is variable. Increasing contractures, particularly when associated with pain or increased spasticity, may be an indication of progressing pathology. Aging changes include decreased flexibility, and the clini­cian must distinguish pathological causes of increas­ing contracture through appropriate diagnosis.

Osteoarthritis. Because of the significant pain com­plaints that adults with CP offer, it is often stated that there is an early onset of osteoarthritis. Conceptually, this has been explained by unusual and possibly increased forces on joints that may have malalign­ment and/or deformity, and associated with under­lying weakness and poor motor control (32). In fact, health care providers often will make a presumed diagnosis of “arthritis” for pain complaints in adults with disabilities. Clinically, it is not surprising to find significant arthritic changes with radiographs of painful joints, and sometimes at young adult ages. However, the presence of early-onset arthritic changes has been documented by case reports, and studies that report arthritis among subjects base this information on self-report of arthritis or presence of pain. Often, the pain complaint is not evaluated fully, and may have an etiology in soft tissue injuries or problems and not degenerative changes within the joint. There may, in fact, be premature osteoarthri­tis, but it has not been documented definitively. Of importance is the recognition of pain, appropriate evaluation, and treatment.

Hip Pathology. Degenerative changes have been noted radiographically in dislocated and subluxed hips, not always related to weight bearing activities, in persons with cerebral palsy (44,56). Use of tone reduction strat­egies may be helpful. Femoral head resection as a treat­ment strategy for control of pain in hip disease for persons with cerebral palsy has been suggested; however, pain often persists or recurs postoperatively (57-60). Total hip and knee replacements as a treatment option for pain from severe arthritis in adults with cerebral palsy are becoming more common; however, as their lifelong efficacy remains unknown (61-64), revision may be anticipated with placement at younger ages.

Knee Pathology. Knee contractures are common in those who do not walk and in those who walk with obvious knee flexion and crouch. Not all knee contractures are painful. Tone management may improve range, func­tion, and pain. Patella alta may develop over time, and pain or chondromalacia may result. Joint laxity may also be present. Modalities, exercise, kinesiotap- ing, and other interventions may be helpful. There are advocates for patellar tendon advancement surger­ies, with or without distal femoral extension osteoto­mies, in adolescents and young adults to improve pain and restore knee function in gait, confirmed on gait analysis (65).

Foot or Ankle Pain. Again from biomechanical factors, contractures and pain may develop. Typical interven­tions may assist including orthoses, but not all bracing or shoe inserts are helpful, and biomechanics must be taken into account. Plantar fasciitis with appropriate treatment should be considered.

Spine Pathology. In people with cerebral palsy, severe motor impairment is associated with scoliosis and other deformities (66). Scoliosis may progress dur­ing adulthood, and those at 50 degrees or greater at skeletal maturity may deteriorate more rapidly (67). Scoliosis can cause seating and pressure problems, impaired respiratory function, and pain (52,67,68), and may be associated with windswept hips and pres­sure sores (52). It has been reported that spinal fusion improves the quality of life for those with CP (69).

Spinal stenosis must be ruled out whenever sig­nificant functional change is noted, particularly for change in or loss of walking skills, increased leg spasticity, change in bladder habits, neck pain, vague sensory changes, and (late) change in arm and hand function (70-72). A tethering effect on the spinal cord also may occur, resulting in cranial nerve changes. Some early reports noted a higher risk in those with an athetoid or dyskinetic component (73,74); however, more recent reports show these problems are present in spastic forms of cerebral palsy as well. While it is generally held that stenosis is due to early spondylosis and compression, there may also be a predisposition to it in those with a congenitally narrow canal, espe­cially at C4-C5 (70,73). Diagnosis is made through imaging studies, while comparative evoked poten­tials may also be helpful in determining neurologic function. Surgical decompression may prevent fur­ther, often catastrophic, loss of function, but does not assure return of lost function, particularly in cases of longstanding compression with spinal cord atrophy. Recurrence at levels above or below surgical correc­tion may be noted (75,76). Postoperative management planning should accommodate changes in functional capabilities and care needs. The presence of an athe- toid movement component will affect postoperative spine stabilization and possibly head positioning and neck mobility. When no surgical intervention is under­taken, a frank discussion of possible respiratory com­promise and the future need for ventilator assistance should be provided.

Peripheral Neurologic Compression. Radiculopathies may be a cause for painful complaints, and appropriate eval­uation and treatment should ensue. It is most impor­tant that treatment strategies are based on the person's history of function, that there is effective input from that person or their care provider, and that practical outcome goals are identified. Although not as common as a musculoskeletal etiology, nerve entrapment is also a cause of pain. The most common nerves and areas of entrapment as reported by adults with CP are the same as those susceptible to compression in the nondisabled population: the median nerve at the carpal tunnel and the ulnar nerve in the hand distally and at the elbow. Compression points are often related to use of crutches, transfer techniques, propelling wheelchairs, or exist­ing deformity. Work-related or positional activities may also cause entrapments, just as in the nondisabled pop­ulation. There is no reported increased incidence in CP. All hand pain or sensation change does not repre­sent nerve entrapment. Often, these complaints are actually problems of repetitive motion or are position- related. While they may be ascribed to carpal tunnel syndrome, they often respond poorly to surgery (77). Appropriate testing (including electrodiagnostic test­ing) is necessary to determine their etiology. Where treatment options are similar for disabled and nondis­abled adults, some modification of management will be required if functional independence is changed by or during treatment.

Osteoporosis. Osteoporosis has been documented in at least 50% of children and adults with cerebral palsy (78,79). The aging process may exacerbate this issue, as does anticonvulsant use and mobility impairment. Pathologic fractures occur typically in the long bones, but frequency data vary and no large studies of peo­ple with cerebral palsy have been reported. Low serum 25-OH vitamin D concentrations are not identified as a cause in most cases described in the literature (79). Typical screening devices, such as the Simple Calculated Osteoporosis Risk Estimation (SCORE), do not accurately identify osteoporosis risk in women with disabilities (80); therefore, bone mineral density testing and counseling on fall risk is important for both women and men with disabilities. Dual energy x-ray absorption (DEXA) scans must be read with caution, since contrac­tures often skew results. Recommendation is to use the scan results of the distal femur, as is used in children with CP and contractures (81). Use of bisphosphonates is described, but the functional improvement derived from these drugs over the long term is unknown.

Additional Health Conditions

There are no comorbidities known to be associated with CP. As noted, general health is good. A recent study of adults living in group homes from upstate New York notes increasing health conditions with age for adults with CP as would be expected: cardiovascu­lar, respiratory, and hearing/vision (82); this has been replicated in Taiwan and Israel (83,84). Of interest is that in comparison to U.S. national norms, there are fewer cardiovascular risk factors than seen in the gen­eral population; either this is a healthier population or there has not been effective screening and monitoring. In looking more critically at this population, the sever­ity of the CP was related to increasing health problems with aging more than the diagnosis of CP alone (85). Vision and hearing problems may have been present early, and as anticipated, there is an increase in vision and hearing problems with age (82).

Dental issues are reported for adults with CP (46). Medications, nutrition problems, poor dental hygiene, and difficulty with access to dental care all contribute to the ongoing problems into adulthood.

Previously known associated conditions will per­sist into adulthood. Dysphagia will continue, and monitoring is required. Constipation also persists, and adjustments to bowel programs may be needed. Gastroesophageal reflux is often reported, but has not been present at increased rates. Intestinal obstruction is reportedly common in CP, and in an upstate New York cohort living in group homes, adults with CP had an increased rate compared to other adults with devel­opmental disabilities (85).

Urinary incontinence may also continue, and assurance must be made that there is no dyssyner­gia or overflow with retention. Rosasco et al reported adults with CP had a higher incidence of urinary tract infections (UTIs) that was related more to severity than the presence of CP (85), compared to other adults with developmental disabilities living in group homes in upstate New York. Neurogenic bladders in adults with cerebral palsy are only infrequently associated with upper tract pathology (86). Some women report that incontinence consistently occurs at a particular point of their menstrual cycle, often associated with increased spasticity (87). Urinary incontinence can be effectively addressed through well-established diag­nostic and intervention approaches. There are no avail­able data that assess the adverse impact of urinary incontinence on social integration in cerebral palsy, but anecdotal support for this association is abundant. In both men and women, urinary incontinence should be identified and addressed, regardless of age or other conditions.

Respiratory problems have been implicated as cause of death early in life and in early adulthood, as was noted earlier. Use of vaccinations may be helpful, along with vigilance and monitoring. Respiratory prob­lems may increase with progressive scoliosis, and aspi­ration from gastroesophageal reflux disease (GERD) or dysphagia must be recognized. Sleep disorders related to pulmonary problems should be considered with pro­gressive scoliosis, especially with complaints of poor sleep, morning headache, or daytime sleepiness.

There has been suggestion that obesity is a prob­lem in CP, and yet there are no studies to support this. In fact, a small study of adults with CP identified mean body fat percentages and body mass indexes were within normal range, although 40% had heights below the fifth percentile for age and gender. Fifty-five per­cent reported dysphagia (88).

Sexual Functioning

Women's sexual health and functioning is better described than men’s. Women with CP typically have limited participation in health maintenance activities such as routine pelvic examinations, Pap smears, and breast examinations (33,89). Office visit planning is required for those with significant motor impairments to assure a complete examination. Attitudinal barriers of health care providers often limit services and edu­cation. However, women with CP are typically able to conceive and carry pregnancies to term without the expectation of major complications related to their CP. Use of contraceptives has not been well studied, and consideration of thrombotic effects must be considered in choice of options. A commonly offered contraception is nonestrogenic formulations such as Depo-Provera, although long-term effects are not well defined (87,90). Women with CP report fewer sexual encounters as compared to other women with disabilities (17,91). Women with early-onset disabilities also experi­ence high levels of sexual desire compared to other women with disabilities, postulated as being related to reduced social opportunities, frustrated satisfaction of sexual urges, discouragement of childhood sexual expression, or perceived social stereotypes (91).

Men with CP also should receive information on sexual functioning, including information on contra­ception and protection. There have been no reported problems with sexual functioning or fertility.