The GP Curriculum and Cerebral Palsy
The GP Curriculum and Cerebral Palsy
The GP Curriculum and Cerebral Palsy
1177/1755738013479046
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Cerebral palsy
C
erebral palsy (CP) is the most common cause of physical disability in child-
hood. It is a non-progressive disorder of postural and motor dysfunction.
This complex, lifelong condition impacts on both children and their
families. Care of these children is therefore an important part of general practice.
Not only must the GP correctly identify CP and coordinate multidisciplinary care,
he or she is the key to ensuring a family-centred service is delivered and provide
the important continuity in care during transition to adult services. This article aims
to provide an overview of the knowledge base needed by the GP when faced with a
child with CP and to promote a biopsychosocial approach to service delivery.
The relevant clinical examples for this article can be found in 2.01: The GP consultation in practice, 3.04:
Care of children and young people, 3.11: Care of people with intellectual disability and 3.18: Care
of people with neurological problems. These statements list the learning objectives required for a GP dealing
with patients and families with CP. In particular, GPs should be able to demonstrate a sound knowledge base of
aetiology, symptoms, investigations, treatment and prognosis of CP.
. Coordinate care with other primary care professionals, paediatricians and other appropriate specialists, leading to
effective and appropriate care provision, taking an advocacy position for the patient or family when needed.
. Provide longitudinal continuity of care as determined by the needs of the patient and family:
Understanding the problems with transitions from child to adolescent, and from adolescent to adult. This
applies to all children but especially the vulnerable
Post-natal (after Account for about 10% of all cases of CP and include:
28 days of life) . Accidental head injury
. Anoxic insult
. Child abuse
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birth weight. Infants weighing less than 2500 g at birth are ganglia. Detection rates are highest amongst spastic-
20–80 times more likely to have CP than those weighing quadraplegic CP and lowest in ataxic/hypotonic CP.
more than 2500 g regardless of gestational age. Should evidence of cerebral infarction be found on an
MRI scan then full coagulopathy screening should be
undertaken as between 50 and 60% of these children
can have a coagulation abnormality. MRI can also help
Diagnosis
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in determining the timing of the insult; whether it was
pre-, peri- or post-natal and that is why MRI is preferred
The diagnosis of CP remains a clinical diagnosis of exclu- to computed tomography.
sion. It depends on the presence of a combination of
neuro-behavioural signs, motor abnormalities and
inappropriate developmental reflexes. Table 2 summar-
ises these abnormalities. Classification
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Although the lesion in CP is static, as the developing The syndromes of CP are classified by the type, distri-
nervous system matures clinical signs will also evolve. bution and severity of the motor abnormality as demon-
Thus, a definitive diagnosis of CP will require serial strated in Box 1. Spastic CP is an upper motor neurone
assessments, especially in pre-term infants. In most syndrome which includes hypertonia, hyperreflexia,
cases, if abnormalities persist at 9 months of age a diag- extensor plantar responses and clonus. It is subdivided
nosis of CP is highly probable. However, diagnosis of the according to topographical distribution and is associated
specific type of CP is best made after 5 years of age. with slow effortful voluntary movements, impaired fine
motor function, fatigueability and contractures.
These signs are also present in other conditions, e.g.
neuro-degenerative diseases, inborn errors of metabolism, Dyskinetic CP is the syndrome of CP linked to peri-natal
developmental traumatic brain/spinal lesions and neo- asphyxia. It presents as encephalopathy which in the
plasms. Therefore, it is important to accurately differenti- infant is characterised by lethargy, decreased movement,
ate these often progressive or congenital disorders from hypotonia and suppressed primitive reflexes. It subse-
CP. This can prove particularly difficult as no specific test to quently develops into an involuntary movement disorder
confirm the diagnosis of CP exists. However, features that such as athetosis, chorea or dystonia. Finally, ataxic CP
should raise the possibility of an alternative diagnosis
include: a family history of neurological conditions, loss
Box 1. Syndromes of cerebral palsy.
of developmental milestones, rapid deterioration of neuro-
logical signs and a marked worsening during periods of 1. Spastic: 70%
catabolism. Important blood tests to aid in differentiating . Hemiplegia (unilateral involvement)
metabolic disorders from CP include glucose, ammonia, . Diplegia (disporprotionate lower extremity
lactate and pyruvate, plasma amino-acid analysis, urine involvement)
organic acid analysis and arterial acid-base status. . Quadriplegia (total body involvement)
2. Dyskinetic: 10–15%
The gold standard in diagnosing CP is magnetic reson-
. Choreoathetoid
ance imaging (MRI) of the brain. It can detect an abnor-
. Dystonic
mality in up to 90% of cases but varies according to type
of CP. Examples of cerebral abnormalities include 3. Ataxic/hypotonic: 5%
hypoxic-ischaemic lesions, such as periventricular leuko- 4. Mixed: 10–15%
malacia, cortical malformations and lesions of the basal
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includes sensory and language dysfunction as well as the particularly useful in clarifying prognosis and predicting
characteristic uncoordinated movement and posture need for mobility aids.
exhibited by the other types of CP.
While CP is primarily a motor disorder it is often accom-
The severity of the motor disorder has been categorised panied by other abnormalities in cerebral function. The
based on functional ability. The Gross Motor Functional most common being epilepsy, followed by cognitive and
Classification System (GMFCS) is outlined in Fig. 1 and is sensory impairments.
Figure 1. Diagrammatic representation of the GMFCS for children aged 6–12 years. GMFCS descriptors.
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is limited to non-ambulatory children with severe spasti-
Management
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city. It is important for the GP to ensure parents under-
stand that these treatments only reduce spasticity; they
It is estimated that up to 75% of children with CP will have do not improve muscle weakness or uncoordinated
at least one other associated disorder. Therefore, man- movement.
agement of CP must address the primary motor dysfunc-
tion as well as monitoring and treating any associated Knowledge of the specific medical or surgical interven-
abnormalities. Thus, a multidisciplinary team approach tion to use is unnecessary for the GP as the decision
with ongoing input from various specialists is required. should be made by a specialist paediatrician. Instead,
awareness of the signs and symptoms of baclofen with-
This section summarises the treatment options available drawal and overdose (see Table 4) is important for the GP
for the motor dysfunction of CP and its associated dis- as it can be life-threatening and will often present in the
orders. It also provides guidance on when and where a primary setting. Patients must therefore carry some form
GP should refer children with CP for specialist input. of ‘medic alert’ information with them at all times.
Tizanidine Central alpha 2 adreno- Causes less weakness Risk of hepatotoxicity needing
ceptor agonist than baclofen and can be liver function monitoring.
used alongside it Withdrawal/rebound hyperten-
sion and tachycardia
Dantrolene Reduces muscle contrac- Decreases tone, tendon Serious risk of hepatotoxicity
tion by inhibiting release reflexes and scissoring needing liver function monitor-
of calcium ions from the and may improve range ing. More common side effects
sarcoplasmic reticulum of motion include weakness, drowsiness,
lethargy, paraesthesias, nausea
and diarrhoea
Diazepam Increase gamma-amino Effective at reducing spas- Side effects include sedation,
butyric acid (GABA) ticity but only short term weakness, ataxia, memory dis-
receptor activity use advised turbances and dependence after
prolonged use
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with consideration of formal videofluoroscopic studies, function of oropharyngeal muscles contribute a mechan-
ultrasound assessment of swallowing and alternative ical block to normal speech. In addition, hearing impair-
modes of nutrition and hydration. ment and learning disabilities may also contribute.
Involvement of speech and language therapists and opti-
Pulmonary disease misation of hearing are key to managing the problem.
Pulmonary disease from recurrent aspiration caused by
oromotor dysfunction, dysphagia and gastro-oesopha- Incontinence
geal reflux is a leading cause of death in CP. Therefore, Bladder control may be delayed or remain abnormal in
addressing these issues is crucial in reducing mortality one-third of children with CP. This in part is due to cog-
and morbidity in children with CP. If pulmonary disease nitive deficits, difficulty in accessing the toilet because of
is present then yearly influenza vaccination is advised. physical disability and the inability to communicate.
However, urodynamic abnormalities such as bladder
Seizures hyperreflexia and sphinctor dyssynergia also contribute
Between 30 and 50% of children with CP have an asso- and therefore referral to a urologist is important. So too is
ciated seizure disorder. The management of these chil- advice to parents about continence aids.
dren is the same as any other child with epilepsy with
choice of anticonvulsant determined by the type of seiz-
ure and response to treatment. The main difference is
Growth
Failure to thrive is a very common problem for children
that children with CP are at increased risk of side effects
with CP (up to 25%). It is caused by a combination of
from treatment and therefore require closer monitoring.
inadequate nutritional intake, due to poor swallowing,
Furthermore, intellectual disability is more common in CP
and gastrointestinal (GI) abnormalities. GI abnormalities
patients with seizures. It is therefore recommended that
include gastro-oesophageal reflux, vomiting, constipation
newer generation anticonvulsants be used wherever pos-
and abdominal pain. It is vital that growth is monitored
sible as they have less impact on cognition.
and dietary advice to increase calorific intake is provided
when necessary. Involving occupational therapists is also
Cognitive impairments useful to help provide adaptations to seating, cups, etc.
Nearly one-half of all children with CP have a learning to aid in efficient eating and drinking. If failure to achieve
disability. Severity is directly correlated to the extent of weight gain despite these interventions persists, naso-
the motor impairment. The more severe the motor gastric or gastrostomy feeds need to be considered.
impairment, the more global the brain insult causing it The use of specific growth charts developed for children
and the greater the risk of additional damage to other with CP that define a weight threshold below which mor-
areas. The role of the GP in managing learning disabilities tality and morbidity is increased should be used.
is limited to organising cognitive assessments and acting
as advocate for patients with CP to receive the support
needed, e.g. speech and language therapy, assistance at
school, and access to other services that will help the
child fulfil his or her potential.
Cerebral palsy and
the GP
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Visual impairments
Ophthalmological abnormalities in CP are numerous, GPs have an important contribution to the care of chil-
common and often treatable. Thus, if concerns over dren with CP. They are often a family’s first contact when
vision are expressed the GP should always refer the concerns over their child’s development arise. The GP
child to a paediatric ophthalmologist for a formal evalu- has the responsibility of recognising parental concerns,
ation as soon as possible. If the abnormality is not treat- monitoring children, and having identified the possibility
able then the GP should consider educational and of CP, referring to paediatric neurologists for further
therapy interventions. investigation and diagnosis. The diagnosis of a lifelong
neurodisability can be a time of crisis for the family and
the GP should support the family through this difficult
Hearing impairment stage. This task becomes even more difficult if the family
Hearing impairment will impede learning and language is unaware of the developmental delay.
development. It is particularly common in pre-term
infants and those who suffered a hypoxic or infectious In these crucial early stages the family’s greatest need is
insult in utero. The GP should organise formal hearing for information. The GP should reinforce and supplement
assessments in all children with CP. the detailed and precise information from the neurologist
regarding CP and its impact on their child’s development
Speech disorders and later life. This must be done in an open, honest and
Communication disorders, including receptive and empathetic manner. Non-medical information, for exam-
expressive language delays and articulation problems ple, the availability of educational and social services
are frequent. Oromotor dysfunction and abnormal should also be discussed by the GP.
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Once the diagnosis of CP has been confirmed the role of with severe CP will have a reduced life expectancy. This
the GP becomes that of a coordinator of lifelong multi- is because of the increase in associated disabilities and
disciplinary care which often involves liaison with neuro- complications affecting the GI and pulmonary systems.
surgeons, orthopaedics, rehabilitation therapists, For those children with tube feeding and the inability to
educators, nurses, social care providers and school- lift the head in prone, median survival has been shown to
teachers. GPs must also continue to provide direct med- be as low as 17 years. However, the prognosis for sur-
ical management of intercurrent illnesses which often vival and motor function in CP is very complex and vari-
have greater impact in children with CP. able. Uncertainties should be shared with parents and
unrealistic expectations addressed.
Furthermore, having been part of the process from the
beginning the GP holds a unique position to recognise
the impact of CP on the child and family and offer sup- Summary
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port when needed. This biopsychosocial approach to ser-
vice delivery is at the centre of the new World Health When dealing with CP the GP must have an honest,
Organisation’s international classification model of open and empathetic attitude, provide lifelong monitor-
human functioning and disability – the ICF. The ICF ing and refer to appropriate services when necessary. To
model recognises the interactive relationship between do so requires a good knowledge base of the causes,
disease and environment which includes the family. It types, associations and management options available.
therefore promotes family-centred care (FCC) when
dealing with CP.
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Dr Zahra Neshat
FY2, Wycombe General Hospital Emergency Medical Centre
Email: zneshat@googlemail.com
DOI: 10.1177/1755738014540778
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Dr Sean McDermott
Locum GP, Northern Ireland
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