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  Vol. 9 No. 5, May 2000 TABLE OF CONTENTS
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The Adult With Cerebral Palsy

Clyde E. Rapp, Jr, MD; Margarita M. Torres, MD

Arch Fam Med. 2000;9:466-472.

ABSTRACT

Important aspects of eliciting a proper history, obtaining a review of systems, and performing a physical examination in adults with cerebral palsy are presented. Information regarding diagnosis, etiology, and epidemiology of cerebral palsy as well as suggestions for performing examinations and procedures on uncooperative and extremely dysmorphic patients are included. A MEDLINE search of all English-language publications related to cerebral palsy from 1985 to 1999 was conducted. Other older references also were obtained from articles published during this period. Our personal experiences in caring for a group of approximately 300 adults with cerebral palsy and other developmental disabilities in specialized centers for nearly a decade are used frequently throughout this review. Emphasis is given to studies of adults. Studies of children are included because there is a lack of data on adults. These studies are identified as such in the text, with extrapolation to adults only where there is a sound clinical or scientific basis. The number of adults with cerebral palsy is increasing. This growth is due to increased survival of low-birth-weight infants and increased longevity of the adult population. Depending on clinical status and the age at which survival is calculated, 65% to 90% of children survive until adulthood. Despite these observations, there is a lack of information in the literature and a lack of relevant postgraduate training programs for physicians in the adult health care system.



INTRODUCTION
 Jump to Section
 •Top
 •Introduction
 •The history
 •Review of systems
 •The physical examination
 •Conclusions
 •Author information
 •References

This article provides information about performing a physical examination, eliciting a history, and a review of systems in adult patients with cerebral palsy, which should equip the primary physician with a basic approach to this group of patients.

Cerebral palsy is usually defined as a nonprogressive disorder of motor function occurring in young children in the prenatal or perinatal period.1 Cerebral palsy frequently includes paresis and incoordination and can result in involuntary movements.1-2

The prevalence of cerebral palsy among children is 1.5 to 2.5 per 1000.3-5 There has also been a gradual increase in the prevalence of cerebral palsy from the mid-1960s to the mid-1980s, which parallels the increasing survival of low-birth-weight infants.6-9 Two recent studies have shown a decrease of approximately 0.5 per 1000 live births from the mid-1980s until 19925 and 1989.10 This does not negate the earlier significant increase in prevalence.

The precise number of adults with cerebral palsy is not known, but the number has been estimated at 400,000.11 Ninety-five percent of children with diplegia and 75% of children with quadriplegia survive until the age of 30 years.12 Ninety percent of children with mild mental retardation and 65% of children with severe mental retardation survive until the age of 38 years.12 Overall survival of all children with cerebral palsy until the age of 20 years is 90%.13

Therefore, cerebral palsy, which is second to mental retardation as the most frequent developmental disability in children,14 is becoming more prevalent in the adult population. Concurrently, there is an increasing trend to transfer adults with cerebral palsy from long-term care facilities to the community.15 Primary care physicians and specialists in the adult health care system, however, have not had special training dealing with adults with developmental disabilities.16-20

A recent study documented excess mortality in adults with cerebral palsy from cancer, stroke, and ischemic heart disease (including a ratio of observed to expected cases of breast cancer of 3.021). The authors postulate that their findings may result from poor surveillance and a lack of early detection. A second study documented a lack of periodic health care, including breast examinations and routine gynecologic care.11


THE HISTORY
 Jump to Section
 •Top
 •Introduction
 •The history
 •Review of systems
 •The physical examination
 •Conclusions
 •Author information
 •References

Eliciting a history may be difficult in view of speech and language deficits or varying levels of cognitive impairment. A number of patients may lack the ability to give a history orally but can achieve some level of expression, such as accurate yes or no responses. It is important to remember that many patients who do not speak can achieve some level of comprehension. Patients may use augmentative communication devices (such as letter and picture boards or talking computers), and caregivers may assist in interpretation of gestures and unintelligible speech patterns. It is important to identify the patient's level of comprehension and ability to actively participate in health care decision making. If the patient has moderate to severe cognitive defects, informed consent should be obtained from a health care proxy, such as a parent, eldest sibling, or court-appointed guardian, but it is important to inform the patient about procedures to the extent that his or her cognitive state allows.

Pregnancy and Early Medical History

Cerebral palsy is a clinical syndrome with a variety of etiologies including vascular, genetic, metabolic, traumatic, and primary neurologic causes.22 Detailed description of the etiology is beyond the scope of this article, and often the cause is not known.

A series of studies and reviews in the last 20 years has demonstrated the poor correlation of Apgar scores and the occurrence of cerebral palsy.23-28 Low birth weight and prematurity, however, have been shown to be correlated with cerebral palsy.29-31 There is no conclusive evidence that cerebral palsy is related to injury sustained at birth or perinatal asphyxia related to delivery.23-28,32

Children with cerebral palsy have delayed developmental milestones, and usually develop spasticity before the age of 15 months.1 In addition, athetoid or ataxic movements may develop during the first several years of life. After this period, both the neurologic examination and neurologic symptoms remain static during childhood in cerebral palsy. Seizures usually occur before the end of the second year (Table 1).33


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Table 1. Cerebral Palsy Typical Scenario


It is important to stress that many infants who develop cerebral palsy are not premature and many premature infants do not develop cerebral palsy. When inquiring about developmental milestones, the physician should stress the eventual achievement of these milestones (Table 2). It may be of interest to review brain imaging studies obtained previously or to obtain a new magnetic resonance image if there has not been a recent one. Any doubts regarding the accuracy of the diagnosis will confirm the need for an evaluation by a neurologist or a physiatrist.


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Table 2. Abbreviated History of Age of Attainment of Developmental Milestones*


The Functional and Social History

The functional and social history should be elicited with the focus on social and daily living skills. Social skills include school, leisure and recreational activities, involvement with peers, and other interests.

An inquiry should be made about the patient's ambulatory status. Twenty percent of adults with cerebral palsy can walk, 40% are only able to walk with assistance, and 40% are nonambulatory.34 A large number of patients do not have an appropriate wheelchair or device to assist with ambulation, so an inquiry about such mobility devices is appropriate.

One should inquire about the patient's domicile, which should be as nonrestrictive as possible35-36 so that it facilitates the skills of independent living. Instrumental activities of daily living include household chores, use of a calculator and checkbook, and community level mobility. These skills should be developed to the level that the patient's physical and cognitive abilities allow, increasing personal and societal independence.

Approximately one third of adults with cerebral palsy live at home.34 Many of the parents of adults with cerebral palsy are elderly and do not have the physical capability to care for an adult with cerebral palsy.37 Thus, one should inquire about the physical capacities of the family and contingency plans for future care.

Patients who are able to use computers and family members of other patients may benefit from a Web site from United Cerebral Palsy (http://www.ucpa.org), which includes information about recent research, computer-assisted communication devices, and employment opportunities.


REVIEW OF SYSTEMS
 Jump to Section
 •Top
 •Introduction
 •The history
 •Review of systems
 •The physical examination
 •Conclusions
 •Author information
 •References

Cerebral palsy involves the neuromuscular system primarily. However, the standard review of systems should be augmented by specific inquiries concerning other commonly involved bodily systems. Areas that should be stressed are discussed in the following section.

Hearing and Vision

Visual defects exist in 25% to 39% of adult patients.34, 38 The etiology among adults has not been studied, but in children causes include cataracts, optic atrophy, and retinitis.1 Inquiries relating to feeding and handling of small objects may provide clues that point to visual deficits, and these patients must be further examined by eye care specialists.

Eight percent to 18% of adult patients with cerebral palsy have hearing problems.34, 38-39 Gross testing may include reaction or response to noise, although specific audiologic testing will be necessary to diagnose and treat hearing loss.

There is evidence that both hearing and vision become worse with age39 so that periodic reexamination becomes necessary.

Cardiovascular System

Detailed studies are not available, but 9% to 10.5% of adult patients with cerebral palsy have cardiovascular problems, including arterial hypertension and coronary artery disease. However, a recent study documents excess mortality among adults with cerebral palsy from ischemic heart disease,21 which may reflect a failure to detect subtle clues to ischemic heart disease (usually offered by a family member or caregiver), such as agitation or change in skin color.

Patients (or their caregivers) may mention swelling, discoloration, or change in temperature of the lower limbs. Decreased temperature of the limbs has also been noted in the literature.15 A precise explanation for these presumably vasomotor findings of the limbs is lacking. Although not adequately described in the literature, a low incidence of deep vein thrombosis despite the presence of swelling and immobility in those with spastic quadriplegia has been noted in our patient population. Proper shoes and compression stockings and careful skin and toenail care should prevent complications such as hypertrophic and mycotic toenails, pressure ulceration, cellulitis, and phlebitis.

Pulmonary System

Patients with quadriparesis34 and those with gastrostomy tubes are at increased risk of aspiration.40 Nasal regurgitation, elevated temperature, lethargy, and slowly progressive dyspnea are indicators of aspiration of oral contents or gastric contents.41 Aspiration may result from swallowing dysfunction or impaired gastric emptying, causing reflux into the esophagus.

An inquiry about the presence of coughing during or immediately after a meal should be made. Clinical swallowing evaluation and a video fluoroscopic examination allow one to determine if a swallowing dysfunction exists. A change in solid and liquid diet consistency, teaching techniques to protect the airway,41-42 and proper positioning during and after meals decrease the occurrence of pulmonary involvement.

Gastrointestinal System

The major gastrointestinal complaints are vomiting and constipation. Vomiting is frequently the result of delayed gastric emptying (which predisposes the patient to gastroesophageal reflux and esophagitis). We have documented delayed emptying in a number of our patients, and it has been noted to be delayed in neurologically impaired children.42-43 Gastroesophageal reflux also becomes more severe as the patient progresses through adolescence.44

Constipation is frequent. Abnormal autonomic control of gastrointestinal motility,45 immobilization, inadequate oral intake, and prolonged colonic transit46 are probably important concurrent factors. Therefore, an inquiry should be made about bowel habits, and increased fluid and fiber with or without laxatives should be prescribed when appropriate.

Speech and Swallowing Problems

Approximately 60% of young adults with cerebral palsy have a speech or communication problem.47-48 This disability may stem from cognitive impairments or oral motor apraxia. Functionally, the person presents with mild to severe dysarthria, difficulty with management of oral secretions, and dysphagia.49-50

Feeding adults with cerebral palsy may take 45 minutes to 11/2 hours, and this may result in poor energy (caloric) intake and weight loss if the caregiver or relative does not allow adequate time to feed the patient. If there is evidence of weight loss, then a food intake and weight diary should be kept to monitor nutritional status.

Musculoskeletal System

Degenerative hip disease and acetabular dislocation are common complications during the adolescent growth spurt, particularly in children with athetoid cerebral palsy.51-52 Increased pain when sitting and standing is usually a manifestation of this condition. Pain is often experienced in the groin and anterior thigh. These patients should be referred to an orthopedist.

Patients with cerebral palsy frequently have various and often asymmetric abnormalities of the ankle-foot system, such as flat feet, varus, valgus, or equinus of the foot. Because the foot bears weight during standing and sitting, it is important to examine the foot, prescribe adequate footwear, and promote proper nail and skin care, with referral to an orthopedist when appropriate.

Scoliosis is more common in patients with cerebral palsy, occurring in 25% to 64% of institutionalized adults.53 Orthopedic referral is important for all patients with cerebral palsy who have scoliosis, since uncorrected scoliosis may result in decreased ambulation and decubiti.53

Osteopenia54-55 and low bone density56 have been found in children with cerebral palsy, particularly in the nonambulatory patient.57 We have found a disproportionate number of adults with osteopenia as well. Adults with cerebral palsy have a lower dietary intake of calcium.58 Factors such as decreased exposure to sunlight, immobility, spasticity, and the metabolic conversion of the precursors of vitamin D to inactive metabolites56 by anticonvulsant medications predispose the patients to fractures. In our experience, osteoporosis becomes worse as the patient ages. Patients with risk factors (such as family history, postmenopausal state, and fracture) should undergo x-ray absorptiometry. If osteoporosis is documented, then appropriate exercises should be prescribed. Endocrinologic referral should also be considered, since the patient may benefit from calcium supplements or a specific medication (such as one of the biphosphonates) for osteoporosis.

Neurologic System

Any neurologic change, such as a change in balance or the frequency of seizures, should be taken seriously, since it may reflect a progressive condition. When neurologic change is reported, the patient should be referred to a neurologist.

Seizures are present in 30% of individuals with cerebral palsy.33 The type, frequency, and duration of seizures should be noted. Neurologic evaluation, including an electroencephalogram, would be helpful to determine need for the continuation of anticonvulsant medication use into the adult years, particularly when patients have been seizure free for 2 years or more.

A history should include a description of the patient's functional capacity, which is an indicator of the extent to which cerebral palsy has interfered with activities of living. An inquiry should be made concerning the extent of the patient's independence in regard to grooming, toileting, dressing, transferring (from a wheelchair to a bed), and ambulating.

Reproductive System and Sexuality

Our observations and those of others indicate that adolescents with cerebral palsy and other static encephalopathies have delayed and prolonged puberty with a poor nutritional state as the primary reason.59 They may develop precocious puberty as well.60 The necessity of recognizing the timing of sexual maturation and providing age-appropriate sexuality education is important. It is also important to determine if the patient is sexually active. Questions regarding sexuality should be posed privately, using normalizing statements and open-ended questions.

Urinary Tract

Bladder dysfunction, including a small irritable bladder that results in frequent urination and possible ureteral reflux, or a hypotonic enlarged bladder is found in a small percentage of children.61 Both can result in infection and incontinence. Although our experience with adults is similar, lack of learning capacity, resulting in the inability to train the bladder and incontinence, is the most common reason. Structural abnormalities that lead to recurrent infection are rare.62

Dysuria may be difficult to assess. Grimacing or distress as noted by the caregivers when the patient urinates often indicates infection. A change in the frequency of urination also provides a diagnostic clue.

Diet and Nutrition

One should inquire about a typical day's meals and snacks. Studies have documented that many immobilized or partially immobilized children with cerebral palsy consume enough food to meet their energy needs but not enough to meet their nutritional needs.63 Fat-free mass is thus deficient in these patients.64 Feeding problems in adolescents with low caloric intake may result in poor growth and decreased muscle mass at maturity.65 These childhood and adolescent nutritional profiles are relevant to adult patients, since they result in an adult with low fat-free mass. Athetoid patients require special attention, since they may have higher caloric requirements.66

Reductions in appetite and weight can be harmful to the adult who already has a low fat-free mass and resultant malnutrition. A diet with sufficient iron (particularly in female patients) is also important, since iron deficiency anemia is common in women with cerebral palsy.58


THE PHYSICAL EXAMINATION
 Jump to Section
 •Top
 •Introduction
 •The history
 •Review of systems
 •The physical examination
 •Conclusions
 •Author information
 •References

A comprehensive physical examination should be performed on patients with cerebral palsy. In addition, the physician's office should be wheelchair accessible, which includes a doorway and bathroom entrance of adequate width and an examination room where a patient in a wheelchair can turn around.67

The Neuromuscular System

Cerebral palsy is frequently described as hemiplegic, diplegic, quadriplegic, or choreoathetoid. Changes in limb weakness, spasticity, or involuntary movements over time and the extent and location of limb involvement should be noted.

Physiologically, in cerebral palsy there is a loss of inhibitory control of spinal reflexes, which results in spasticity68 and exaggerated reflexes, including deep tendon reflexes accompanied by clonus1 in some cases. In many adults, spasticity evolves into contractures.69 The shortened, contracted muscle has fewer sarcomeres (and, therefore, less weight and power) than the normal muscle.70-71 Shortening of the muscles by contractures in cerebral palsy decreases the range of length over which tension can be actively generated,72 resulting in weakness, decreased range of motion, and poor coordination, which become worse as the patient ages.18 Therefore, the effect of the patient's contractures and spasticity on his or her mobility and coordination during activities, such as reaching or walking, should be noted. Spasticity and contractures vary with position and patient stress; the patient should be examined in active and passive movement and voluntary functional activities.

Range-of-motion charts are frequently available. Figure 1 shows charts for the large joints of the arms and legs. The most common contractures should be noted. In the upper limbs, these are flexion and pronation of the forearm, internal rotation and adduction of the shoulder, flexion of the wrist, flexion of the fingers at the interphalangeal joints, and adduction of the thumb or "thumb in palm deformity." In the lower limb, the most common contractures are flexion of the hip and knee, adduction of the hip, flexion of the toes, and plantar flexion of the foot. The Ashworth scale is a nonquantitative scale that has been widely used as a clinical measure of spasticity and seems to have good face validity (Table 3).73 Release of joint contractures has been successfully performed, and orthopedic referral should be considered when they are present.



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Range-of-motion chart for large joints of the upper and lower limbs.



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Table 3. Ashworth Scale


The patient's gait pattern should be observed. Gait analysis studies in patients with spastic hemiplegia show the following: (1) variable involvement of the limb from mild plantar flexion of the foot during the swing phase of gait to persistent equinus, (2) knee extension, and (3) dynamic hip flexion contracture with lumbar lordosis and exaggerated pelvic tilt.74 These abnormalities may occur in variable combinations.

Patients with spastic diplegia most commonly show scissoring or hip adduction due to spasticity of the adductors during the swing phase.75-76 Initial contact of the foot with the floor often does not occur in the normal heel-first manner, causing calluses in the metatarsal area along with pain and joint deformities. An abnormal ankle-foot system (which may take a variety of forms) may be caused by a tight heel cord, spasticity of the anterior or posterior tibialis muscles, or a spastic gastrocnemius soleus muscle. Computer-assisted gait analysis will indicate which of these abnormalities is causing the problem. Computer-assisted gait analysis is also essential before treatment interventions, since a dynamic change may be the result of bone and ligament malalignment, soft tissue laxity, muscle timing (activation and shut-off during the cycles of gait), spasticity, or contractures. Intervention depends on the specific cause, since surgical approaches often follow gait analysis.

Examination of the Genitals

There is a higher incidence of cryptorchidism in adults with cerebral palsy than in the general population, with an incidence of 53% at the age of 21 years.77 The patient must be standing (or supported in a standing position) to determine with accuracy whether the patient has undescended testes or retracted testes. If the testes are not visualized or palpated, then further examination by a urologist is necessary.

The Pelvic and Breast Examination

A pelvic examination may be a particularly difficult experience for an adult with cerebral palsy, leading to poor cooperation. The difficulty is the result of fear and tension and spasticity of the adductors of the thighs. The examination may be performed in the lateral decubitus position, which may improve cooperation and allow easier insertion of the speculum and collection of the specimen for the Papanicolaou smear. Use of a small speculum may also facilitate successful completion of the examination. If the patient is uncooperative, anesthesia may be required. After the initial examination is performed with the patient under anesthesia, a pelvic ultrasound may be substituted for the pelvic examination for the following 2 years.78 In the absence of symptoms suggesting a pelvic pathologic condition, another examination with the patient under anesthesia need not be done for another 2 years (Table 4).


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Table 4. Suggestions for Performing Examinations and Procedures


Mammography is often difficult because of poor cooperation and because the patient's dysmorphologic condition does not allow her to be properly positioned on the x-ray machine. In these cases, a good breast examination (which should be done anyway), with referral to a specialist in breast disease and ultrasound if abnormalities are suspected, is recommended (Table 4).

The Dental Examination

A description of the technical aspects of dental care for the patient with cerebral palsy is beyond the scope of this article. Lack of cooperation in the case of those patients with cerebral palsy who have mental retardation is frequently a problem, however, and the dentist and primary physician should discuss appropriate management.

The 4 methods of managing an agitated patient with poor cognitive skills are (1) oral sedation, (2) restraints and oral sedation, (3) intramuscular or intravenous sedation in the dental office, and (4) general anesthesia.79 The primary care physician should inform the dentist about which type of oral sedation has been successful in other situations where sedation is needed (our experience has been that restraints without other sedation are not effective). If general anesthesia is selected, it is often expeditious to do as much dental work as possible at one time so that frequent general anesthesia is not necessary. The procedure should be scheduled so that the effects of sedation are still present when the patient is examined or undergoes treatment.

Miscellaneous Dysmorphic Findings

Miscellaneous findings that are increased in frequency in cerebral palsy include a variety of dysmorphic features. Prominence of the occiput, hyperextensibility of the digits, multiple nevi, microcephaly, a long thin upper lip, malformed ears, chorioretinitis, syndactyly, and polydactyly have all been described.1 Obviously, some of these findings may reflect the cause of the patient's cerebral palsy, such as microcephaly in cerebral palsy secondary to cytomegalovirus infection, whereas others reflect manifestations of genetic syndromes.22 No doubt many other dysmorphic features occur in cerebral palsy that have not been described in the literature because of the role of genetic factors in the etiology of cerebral palsy,71 since genetic syndromes are frequently accompanied by dysmorphic features.

Evans et al13 wrote that it was their hope that cerebral palsy will become "a condition with which one lives rather than one from which one dies." It is suggested that this is an appropriate mission statement as we attempt to develop a medical care delivery system for adults with cerebral palsy and provide appropriate training for physicians to meet their needs.


CONCLUSIONS
 Jump to Section
 •Top
 •Introduction
 •The history
 •Review of systems
 •The physical examination
 •Conclusions
 •Author information
 •References

From 65% to 90% of children with cerebral palsy survive into adulthood, creating a large number of adults with a need for medical care. The incidence may continue to increase because of increased survival of very low-birth-weight infants and/or increased longevity of the general population.

Currently, specialized training programs dedicated to the care of adults with cerebral palsy are practically nonexistent. In addition, there is a paucity of contributions to the literature concerning adults with cerebral palsy. This article has reviewed the important aspects of the history, review of systems, and physical examination of adults with cerebral palsy. Suggestions for performing examinations and procedures on patients with cerebral palsy were provided as well.


AUTHOR INFORMATION
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 •Introduction
 •The history
 •Review of systems
 •The physical examination
 •Conclusions
 •Author information
 •References

Accepted for publication January 13, 2000.

We thank Michael Alexander, MD, Steven Bachrach, MD, and Edith Hasbrouck, MD, for reviewing the manuscript. In addition, the assistance of Walter Gerber, MD, for providing guidelines for the evaluation of cryptorchidism is greatly appreciated. We would also like to thank our consultant colleagues whose perseverence has helped us develop an approach to adults with cerebral palsy.

Reprints: Clyde E. Rapp, Jr, MD, Center for Adults with Developmental Disabilities, Albert Einstein Medical Center, 5501 Old York Rd, Philadelphia, PA 19141.

From the Center for Adults with Developmental Disabilities, Department of Pediatrics, Albert Einstein Medical Center (Dr Rapp), and Eastern Regional Center for Adults with Spina Bifida, Moss Rehab (Dr Torres), Albert Einstein Healthcare Network, Philadelphia, Pa.


REFERENCES
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 •Top
 •Introduction
 •The history
 •Review of systems
 •The physical examination
 •Conclusions
 •Author information
 •References

1. Ingram TTS. Pediatric Aspects of Cerebral Palsy. Edinburgh, Scotland: E & S Livingstone Ltd; 1964.
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24. Naeye RL, Peters EC, Bartholomew M, Landis R. Origins of cerebral palsy. AJDC. 1989;143:1154-1161.
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26. Grether JK, Nelson KB, Emery ES, Cummins SK. Prenatal and perinatal factors and cerebral palsy in very low birth weight infants. J Pediatr. 1996 Mar;128:407-414.
27. Nelson KB, Ellenberg J. Apgar scores as predictors of chronic neurologic disability. Pediatrics. 1981;68:36-44. FREE FULL TEXT
28. Nelson KB, Ellenberg J. Antecedents of cerebral palsy: multivariate analysis of risk. N Engl J Med. 1986;315:81-86. ABSTRACT
29. Bhushan V, Paneth N, Kiely JL. Impact of improved survival of very low birthweight infants on recent secular trends in the prevalence of cerebral palsy. Pediatrics. 1993;91:1094-1100. FREE FULL TEXT
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32. Bedrick AD. Perinatal asphyxia and cerebral palsy: fact, fiction or legal production? AJDC. 1989;143:1139-1140.
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34. Brown MC, Bontempo A, Turk MA. Secondary Consequences of Cerebral Palsy: Adults With Cerebral Palsy in New York State. Albany, NY: Developmental Disabilities Planning Council; 1992.
35. Hallum A, Krumboltz JD. Parents caring for young adults with severe physical disabilities: psychological issues. Dev Med Child Neurol. 1993;35:24-32. ISI | PUBMED
36. Kokkonen J, Saukkonen AL, Tinonen E, Sarls W, Kinnunen P. Social outcome of handicapped children as adults. Dev Med Child Neurol. 1991;33:1095-1100. ISI | PUBMED
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