Pediatric Patients With Cerebral

Palsy or Other Developmental Disabilities

Ann 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 an
order of magnitude, with the most recent report from the
Centers for Disease Control as 3.6 per 1,000 live births. The
incidence reported has remained surprisingly stable; gures
in this range have been reported for over 40 years. Thus, the
child neurologist will be frequently called on to evaluate and
treat these children.
Etiologies of CP have been better identied with the advent
of magnetic resonance imaging. Furthermore, the identica-
tion of intrauterine infection/inammation and the neuro-
logic implications of such have provided further insight into
the cause of CP. It is nowunderstood that well over half of the
children with CP were born prematurely. This observation
does not necessarily identify the etiology of the CP (eg, the
problem could be intrauterine poor nutrition to the fetus,
developmental brain abnormalities, and so on).
The responsibility the child neurologist is to conrm the
CP 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, can
assess and treat the different types of hypertonia that may
interfere with function, care, and hygiene and improve pain
whenever is needed. These teams often include orthopedic
surgery, physical medicine and rehabilitation, neurosurgery,
allied health professionals, and orthotics.
It is recognized that in addition to the motor decits, more
than half the children with CP will also have associated prob-
lems, such as cognitive impairment, seizure disorder, behav-
ioral problems, and sensory impairments including hearing
and vision decits. The child neurologist can play a major
role in the identication 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 with
disabilities full care.
The approach to the child with chronic motor disorders
has also changed dramatically over time. The child neurolo-
gist now has multiple interventions at his/her disposal, with
the potential to substantially improve long-term outcomes
when chosen and applied appropriately. It is imperative that
the child neurologist becomes familiar with the International
Classication of Functioning, Disability and Health proposed
in 2001 by the World Health Organization. This interna-
tional rehabilitation framework allows health professionals to
treat 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 to
provide meaningful treatments to the child with CP and de-
velopmental disabilities will depend on how much the child
neurologist understands and follows this process.
Essential skills for the assessment and management of the
child with CP and developmental disabilities include the fol-
lowing:
1. Skills best developed from exposure to a wide range of
children with and without CP will result in a detailed
understanding of the normal range of motor and cog-
nitive developmental milestones. It will also give an In
depth appreciation of the full range of potential motor
and cognitive aspects of CP and other developmental
disabilities in the context of the Gross Motor Function
Classication System (GMFCS), the Manual Abilities
Classication System (MACS) and newly developed
Communication Function Classication System
(CFCS). The child neurologist should also be able to
recognize the full range of competencies that remain
intact in the child.
2. Skills best developed through study, observation, and
practice with a highly experienced clinician including
skill in taking a careful and thorough history. Achrono-
logic approach is helpful, particularly in children with
developmental disabilities. As with any medical prob-
lem, a thorough history and physical examination is
necessary and specically in CP because it is the best
way 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
diagnosis. Substantial insights may be gained from the
prenatal and perinatal history. Aspects of prenatal care
and intercurrent infection may offer important clues to
the etiology, especially when combined with imaging.
Many children with CP are born prematurely, but by
contrast a premature birth does not necessarily mean
that there will be disability. Furthermore, problems in
the neonatal period occur not uncommonly and yet do
not necessarily lead to CP. Aspects of the family history
may offer insight into whether the disorder is likely to
be sporadic or familial. The child neurologist must have
ability in the assessment of range of motion, strength,
muscle tone, motor control, gait patterns, and recogni-
tion of the special challenges of these assessments in
children in whom communication skills are impaired
or have not yet developed. The child neurologist
should be knowledgeable about range of motion eval-
uation and contracture development but should also
knowthe important roles of the therapists as well as the
orthopedic surgeon and have skill in administering
the common functional assessment scales, such as the
Gross 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 be
adept at administrating these scales but rather familiar
with these tools and how they can be used to assess
child development over time. He/she also must have
familiarity with the full range of pharmacologic and
surgical treatment options, including timing, potential
benets, adverse effects, contraindications, potential
for synergy with other treatments, and cost impacts of
each.
3. Skills that draw on personal characteristics not specic
to medical training including (1) effective and sympa-
thetic communication with caregivers to assess the
childs and familys needs and to develop a treatment
plan according to their gross motor function level that
will 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 work
professionals to develop, implement, and monitor the
treatment plan. The neurologist is likely to be the co-
ordinator of the treatment plan but must be willing to
take and be grateful to receive advice from other pro-
fessionals. It is most important for the child neurologist
to understand the roles of each member of the care-
giving team. No one person is capable to provide the
total care to the child with signicant disabilities;
rather, the child neurologist must be knowledgeable
about what each member of the team can offer.
As noted previously, extensive observation of both affected
and nonaffected children and close work with an experi-
enced clinician and multidisciplinary team are central to the
training of the child neurologist. Observation offers the
trainee the opportunity to develop a fuller appreciation of
both the range of disability and the range of retained ability in
children with CP. Close work with guidance allows trainees
to hone their skills, ask questions, and test their growing
understanding of assessment and treatment planning. Expe-
rience gained in this type of setting will also be useful in
dealing with children with acquired injury. Children with
traumatic 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 disabilities
provides a template against which to observe and understand
recovery or continued deterioration.
Both outpatient and inpatient settings provide important
opportunities to learn about children with disabilities. Most
patients who a child neurologist sees are coming from their
home setting, and often the referrals are for global delay or for
some formof motor delay. The best setting to learn about the
evaluation and care of the child with developmental disabil-
ity and CP is in an outpatient setting working closely with
other health care professionals who have knowledge about
the care of the child with motor impairment and other asso-
ciated disabilities.
The outpatient clinic may provide a less threatening setting
in which to approach the patient than in the hospital. On the
one hand, the needs and priorities of such children may be
easier for the trainee to assess and comprehend. By contrast,
the ability to effectively handle multiply involved children is
absolutely essential for the child neurologist, and prolonged
experience with such children is the only way to develop this
essential skill. The opportunity to coordinate care among
other subspecialties is far more common in the hospital than
in the outpatient clinic.
Exposure to children with developmental disabilities ide-
ally occurs at all levels of training. Rehabilitation training
should begin early, with training in techniques, such as bot-
ulinum toxin injections or intrathecal baclofen for spasticity
following, after the trainee has gained some working knowl-
edge of electrophysiology, electromyography or electrical
simulation techniques. As noted earlier, close work with a
mentor is ideal because it offers the trainee in-depth exposure
to one persons approach and the opportunity to develop
his/her own approach to care in response.
Pediatric patients with CP 73