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Pediatric Patients With Cerebral

Palsy or Other Developmental DisabilitiesAnn Tilton, MD, FAAN,* and Mauricio R. Delgado, MD, FRCPC, FAAN

Cerebral palsy (CP) is the most common chronic motor dis-order in childhood. Reports of the incidence of CP vary by anorder of magnitude, with the most recent report from theCenters for Disease Control as 3.6 per 1,000 live births. Theincidence reported has remained surprisingly stable; figuresin this range have been reported for over 40 years. Thus, thechild neurologist will be frequently called on to evaluate and

treat these children.Etiologies of CP have been better identified with theadvent

of magnetic resonance imaging. Furthermore, the identifica-tion of intrauterine infection/inflammation and the neuro-logic implications of such have provided further insight intothe cause of CP. It is now understood that well over half of thechildren with CP were born prematurely. This observationdoes not necessarily identify the etiology of the CP (eg, theproblem could be intrauterine poor nutrition to the fetus,developmental brain abnormalities, and so on).

The responsibility the child neurologist is to confirm theCP diagnosis and exclude other neurologic disorders that

may resemble CP (eg, hereditary spastic paraparesis, dopa-responsive dystonia, and so on). In addition, the child neu-rologist, in coordination with other health professionals, canassess and treat the different types of hypertonia that mayinterfere with function, care, and hygiene and improve painwhenever is needed. These teams often include orthopedicsurgery, physical medicine and rehabilitation, neurosurgery,allied health professionals, and orthotics.

It is recognized that in addition to the motor deficits, morethan half the children with CP will also have associated prob-lems, such as cognitive impairment, seizure disorder, behav-ioral problems, and sensory impairments including hearingand vision deficits. The child neurologist can play a majorrole in the identification and management of such comor-bidities, and he/she should have knowledge of the appropri-ate referrals that would be necessary to provide the child withdisabilities full care.

The approach to the child with chronic motor disordershas also changed dramatically over time. The child neurolo-gist now has multiple interventions at his/her disposal, withthe potential to substantially improve long-term outcomeswhen chosen and applied appropriately. It is imperative thatthe child neurologist becomes familiar with the InternationalClassification of Functioning, Disability and Health proposed

in 2001 by the World Health Organization. This interna-tional rehabilitation framework allows health professionals totreat patients in the context of 4 domains: structure/function,activity, participation, and personal and environmental fac-tors. The effectiveness and ability to communicate with pa-tients, families, and other health care professionals and toprovide meaningful treatments to the child with CP and de-velopmental disabilities will depend on how much the childneurologist understands and follows this process.

Essential skills for the assessment and management of thechild with CP and developmental disabilities include the fol-lowing:

1. Skills best developed from exposure to a wide range ofchildren with and without CP will result in a detailedunderstanding of the normal range of motor and cog-nitive developmental milestones. It will also give an Indepth appreciation of the full range of potential motorand cognitive aspects of CP and other developmentaldisabilities in the context of the Gross Motor FunctionClassification System (GMFCS), the Manual AbilitiesClassification System (MACS) and newly developedCommunication Function Classification System(CFCS). The child neurologist should also be able to

recognize the full range of competencies that remainintact in the child.2. Skills best developed through study, observation, and

practice with a highly experienced clinician includingskill in taking a careful and thorough history. A chrono-logic approach is helpful, particularly in children withdevelopmental disabilities. As with any medical prob-lem, a thorough history and physical examination isnecessary and specifically in CP because it is the bestway to identify the etiology and, ultimately, in conjunc-tion with the examination and other modalities, the

From the *Louisiana State University Health Sciences Center, New Orleans,

Louisiana.

University of Texas Southwestern Medical Center, Dallas.

Address reprint requests to Ann Tilton, MD, FAAN, Childrens Hospital, 200

Henry Clay Avenue, New Orleans, LA 70118. E-mail:atilto@lsuhsc.edu

72 1071-9091/11/$-see front matter 2011 Elsevier Inc. All rights reserved.doi:10.1016/j.spen.2011.05.016

mailto:atilto@lsuhsc.edu

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diagnosis. Substantial insights may be gained from theprenatal and perinatal history. Aspects of prenatal careand intercurrent infection may offer important clues tothe etiology, especially when combined with imaging.Many children with CP are born prematurely, but bycontrast a premature birth does not necessarily meanthat there will be disability. Furthermore, problems inthe neonatal period occur not uncommonly and yet donot necessarily lead to CP. Aspects of the family historymay offer insight into whether the disorder is likely tobe sporadic or familial. The child neurologist must haveability in the assessment of range of motion, strength,muscle tone, motor control, gait patterns, and recogni-tion of the special challenges of these assessments inchildren in whom communication skills are impairedor have not yet developed. The child neurologistshould be knowledgeable about range of motion eval-uation and contracture development but should alsoknow the important roles of the therapists as well as the

orthopedic surgeon and have skill in administeringthe common functional assessment scales, such as theGross Motor Function Measure and Pediatric Evalua-tion of Disability Index, with an emphasis on consis-tency of evaluation over time and among different pa-tients. It is not necessary for the child neurologist to beadept at administrating these scales but rather familiarwith these tools and how they can be used to assesschild development over time. He/she also must havefamiliarity with the full range of pharmacologic andsurgical treatment options, including timing, potentialbenefits, adverse effects, contraindications, potential

for synergy with other treatments, and cost impacts ofeach.

3. Skills that draw on personal characteristics not specificto medical training including (1) effective and sympa-thetic communication with caregivers to assess thechilds and familys needs and to develop a treatmentplan according to their gross motor function level thatwill be largely carried out at home by caregivers and (2)the ability to work as part of a multi- and interdisciplin-ary team with medical, educational, and social workprofessionals to develop, implement, and monitor thetreatment plan. The neurologist is likely to be the co-ordinator of the treatment plan but must be willing totake and be grateful to receive advice from other pro-fessionals. It is most important for the child neurologistto understand the roles of each member of the care-giving team. No one person is capable to provide thetotal care to the child with significant disabilities;

rather, the child neurologist must be knowledgeableabout what each member of the team can offer.

As noted previously, extensive observation of both affectedand nonaffected children and close work with an experi-enced clinician and multidisciplinary team are central to thetraining of the child neurologist. Observation offers the

trainee the opportunity to develop a fuller appreciation ofboth the range of disability and the range of retained ability inchildren with CP. Close work with guidance allows traineesto hone their skills, ask questions, and test their growingunderstanding of assessment and treatment planning. Expe-rience gained in this type of setting will also be useful indealing with children with acquired injury. Children withtraumatic brain injury, spinal cord injury, or neurodegenera-tive disorders have multiple disabilities in common with chil-dren with CP. Understanding the general pattern of develop-ment and care needs in children with static disabilitiesprovides a template against which to observe and understandrecovery or continued deterioration.

Both outpatient and inpatient settings provide importantopportunities to learn about children with disabilities. Mostpatients who a child neurologist sees are coming from theirhome setting, and often the referrals arefor global delay or forsome form of motor delay. The best setting to learn about theevaluation and care of the child with developmental disabil-ity and CP is in an outpatient setting working closely withother health care professionals who have knowledge aboutthe care of the child with motor impairment and other asso-ciated disabilities.

Theoutpatient clinic may provide a less threatening settingin which to approach the patient than in the hospital. On the

one hand, the needs and priorities of such children may beeasier for the trainee to assess and comprehend. By contrast,the ability to effectively handle multiply involved children isabsolutely essential for the child neurologist, and prolongedexperience with such children is the only way to develop thisessential skill. The opportunity to coordinate care amongother subspecialties is far more common in the hospital thanin the outpatient clinic.

Exposure to children with developmental disabilities ide-ally occurs at all levels of training. Rehabilitation trainingshould begin early, with training in techniques, such as bot-ulinum toxin injections or intrathecal baclofen for spasticityfollowing, after the trainee has gained some working knowl-edge of electrophysiology, electromyography or electricalsimulation techniques. As noted earlier, close work with amentor is ideal because it offers the trainee in-depth exposureto one persons approach and the opportunity to develophis/her own approach to care in response.

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