InnovAiT, 7(8), 484–492 DOI: 10.

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 GP curriculum and cerebral palsy

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.

Contextual statement 2.01: The GP consultation in practice

. Recognise the roles of health and social care colleagues and draw on this expertise appropriately
. Share information with patients in an honest and unbiased manner, in order to educate them about their health
(doctor as teacher)
. Negotiate a shared understanding of the problem and its management with patients, so that they are empowered
to look after their own health
. Understand the importance of continuity of care and long-term relationships with your patient and their family in
identifying and understanding the values that influence a patient’s approach to healthcare
. Understand that consultations have a clinical, a psychological and a social component, with the relevance of each
component varying from consultation to consultation (the ‘triaxial’ model)
. Recognise that episodes of illness usually affect more than merely the patient
. Understand the relationship between the interests of patients and the interests of their carers
. Negotiate whether and how relatives, friends and carers might become involved, while balancing your patient’s
right to confidentiality

Clinical example 3.04: Care of children and young people

. Manage conditions and problems which may present early and in an undifferentiated way by:
 Being aware of normal growth and development in children and young people
 Having a thorough understanding of normal development, and being able to recognise delayed development
through childhood and adolescence
 Recognising normal growth, and dealing with faltering growth and failure to thrive
. Manage primary contact with children and their families – and, with older children, on their own
. Adopt a family-centred approach in dealing with patients, their families and their problems. This requires:
 Effective communication and engagement (listening to and involving children and young people, and working
with parents, carers and families)
. Assess children and young people’s developmental needs in the context of their family and environmental factors
including school and community, and parenting capacity
. Demonstrate an understanding of the importance of multiagency working (working across professional and
agency boundaries) and the principles of information-sharing

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. 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

Clinical example 3.11: Care of people with intellectual disability

. Be aware of likely associated conditions, the high mortality, the high morbidity and the difference in morbidity
compared to the rest of the population
. Understand the need to support adolescents with intellectual disability as they become adults and no longer have
the multidisciplinary support of community paediatricians

Clinical example 3.18: Care of people with neurological problems

. Knowing the indications for referral to a neurologist for chronic conditions requiring ongoing specialist manage-
ment and conditions that are irreversible without early treatment
. Communicate prognosis, including any uncertainties, truthfully and sensitively to patients with disabling neuro-
logical conditions
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The incidence of CP is around 2–2.5 per 1000 live

Aetiology and births and has remained fairly static over the past 40
epidemiology
...........................................................
years. The aetiology of CP is often divided into three
broad categories: pre-natal, peri-natal and post-natal.
Table 1 summarises the causes of CP based on these
Cerebral palsy (CP) was first described over 150 years categories.
ago. Definitions of the disorder have changed over time
reflecting an increasing understanding of the condition. While it was commonly believed that peri-natal hypoxic
In 2005 the American Academy for Cerebral Palsy and or ischaemic insults caused CP, it is now believed that CP
Developmental Medicine put forward the following is caused by the release of inflammatory mediators caus-
definition: ing damage to brain development at critical moments.
Cerebral palsy describes a group of disorders of the This is supported by the relatively low contribution of
peri-natal compared with pre-natal causes in the devel-
development of movement and posture, causing
opment of CP.
activity limitations, that are attributed to non-pro-
gressive disturbances that occurred in the develop- An important distinction must also be made between
ing foetal or infant brain. The motor disorders of causes and risk factors. Prematurity is a well-recognised
cerebral palsy are often accompanied by disturb- risk factor but not a cause of CP. This is because co-
ances of sensation, cognition, communication per- existence of other risk factors associated with CP are
ception and/or by a seizure disorder. more common in pre-term infants, most significantly low

Table 1. Causes of cerebral palsy.

Pre-natal Account for 75% of all cases of CP and include:

. Brain malformations
. Maternal infections, e.g. toxoplasmosis, rubella, cytomegalovirus and herpes
simplex virus
. Vascular events, e.g. middle cerebral artery occlusion

Peri-natal Account for 10–15% of cases of CP and include:

. Hypoxic ischaemic encephalopathy, e.g. antepartum haemorrhage, cord prolapse
. Viral encephalitis
. Meningitis

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
...........................................................
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
...........................................................
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

Table 2. Signs of cerebral palsy.

Neurobehavioural signs Motor abnormalities Developmental reflexes

. Excessive docility/irritability . Abnormal tone, hyperreflexia, . Delay in disappearance of

. Poor feeding with or without persistent or asymmetric fisting developmental reflexes
frequent vomiting . Abnormal oromotor patterns – . Exaggeration of develop-
. Poor visual attention tongue retraction and thrust, tonic mental reflexes, e.g. tonic
. Difficult to handle or cuddle, bite, oral hypersensitivity and labyrinthine response
sleeps poorly grimacing . Abnormal postural reactions
. Poor head control . Presence of obligatory
. Delayed/absent motor milestones – developmental reflex (at any
this requires serial examination over age)
a period of months

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includes sensory and language dysfunction as well as the
characteristic uncoordinated movement and posture
exhibited by the other types of CP.
The severity of the motor disorder has been categorised
based on functional ability. The Gross Motor Functional
Classification System (GMFCS) is outlined in Fig. 1 and is
particularly useful in clarifying prognosis and predicting
need for mobility aids.
While CP is primarily a motor disorder it is often accom-
panied by other abnormalities in cerebral function. The
most common being epilepsy, followed by cognitive and
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
...........................................................
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.

Spasticity Musculoskeletal disorders

Initial management of spasticity involves physiotherapy Abnormalities such as contractures, muscle imbalance
to provide passive stretching of the affected area. For and bony deformity are common secondary complica-
localised spasticity likely to interfere with function or tions of the motor dysfunction of CP. Non-ambulatory
lead to joint contracture with growth, the next step in and partially ambulant children are at significant risk of
treatment is botulinum toxin A (BTX A). It should not be hip subluxation which further reduces mobility, causes
used in children under 18 months due to lack of data in pain and difficulty with perineal hygiene. Early detection
this age group. For more generalised spasticity there are allows full hip reconstruction as degenerative changes of
a variety of oral anti-spastic drugs, e.g. tizanidine, baclo- the femoral head have not yet occurred. Timely referral
fen, dantrolene and benzodiazepines, (see Table 3) sur- to the orthopaedic team is therefore associated with
gical interventions such as selective dorsal rhizotomy better outcomes in these children. Thus, yearly radio-
(sectioning of specific posterior spinal roots to reduce graphic monitoring to screen for hip subluxation and dis-
spasticity) and intrathecal baclofen. location (Fig. 3) is advised in all children from 18 months
to 7 years of age who walk fewer than 10 steps.
Intrathecal baclofen is administered via a pump and
achieves greater cerebrospinal fluid (CSF) drug concen- There is also emerging evidence that due to gastrointes-
trations than oral baclofen (see Fig. 2). Its use, however, tinal complications of CP, bone density can be adversely

Table 3. Common oral anti-spasticity agents used in cerebral palsy.

Drug Mode of action Advantages Disadvantages

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

Baclofen Inhibits transmission at Depresses the central nervous

spinal level system causing sedation and
muscle hypotonia

Dantrolene Reduces muscle contrac-
tion by inhibiting release
of calcium ions from the
sarcoplasmic reticulum
Decreases tone, tendon Serious risk of hepatotoxicity
reflexes and scissoring needing liver function monitor-
and may improve range ing. More common side effects
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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Figure 2. Intrathecal baclofen pump.

ß 2012 Australian Family Physician. Reproduced with permission from the publisher, The Royal Australian College of
General Practitioners

ability to stand for transfers. Scoliosis is a rare complica-

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Table 4. Complications of intrathecal

tion in the most severely affected, non-ambulatory child.
baclofen.
Spinal radiographs at 5 years and 10 years of age are
recommended to monitor these children.
Baclofen withdrawal Baclofen overdose
. Fever . Lethargy
. Itching . Seizures Sialorrhoea
. Increased stiffness Oromotor dysfunction causes incomplete mouth closure
and abnormal swallowing resulting in severe persistent
drooling beyond a socially acceptable age. Complications
include odour, chapping, and, rarely, pulmonary com-
promise. In most children, drooling will improve with
age. Medical management should be attempted before
considering surgical interventions. Pharmacological ther-
apy with anticholinergics such as benzhexol, scopolamine
or glycoproate can help reduce the flow of saliva. BTX A
injected into the salivary glands is an alternative to anti-
cholinergics and studies have shown that it has both
greater efficacy and fewer side effects than scopolamine.
However, the positive effects of BTX A reduce overtime,
lasting a median of 22 weeks. Therefore, repeated injec-
tions will often be necessary.

Surgical treatment involves repositioning of the subman-

Figure 3. Child with cerebral palsy with lateral dibular ducts and unilateral ligation of a parotid duct.
dislocation of the left hip. While surgery reduces the frequency and severity of
Image source: www.medcyclo.com by GE Healthcare drooling, there is an increased risk of cavity formation
requiring regular dental follow up.
affected. The GP should ensure optimal intake of cal-
cium, phosphorus and vitamin D to reduce the risk of Feeding problems – swallowing
osteoporosis giving supplements where appropriate. CP carries an increased risk of dysphagia. This can result
in prolonged feeding times and limitation of food choices
Other orthopaedic areas of concern include flexion con- which may compromise nutritional intake. In severe cases
tractures at the knee. These may require hamstring it can lead to recurrent aspiration and pneumonia. These
muscle-tendon surgery to improve gait pattern, or the children need speech and language therapy assessment

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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
...........................................................
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
...........................................................
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.

Encouraging family participation, e.g. in identifying and

Key points
setting goals for therapy, can help the child achieve . CP encompasses a range of non-progressive dis-
greater functional gains with less effort than traditional orders characterised by postural and motor dys-
methods. Moreover, the provision of FCC as promoted function caused by insults to the developing brain
by the ICF model, ensures that the impact on the parents and is the most common cause of disability in children
of caring for a disabled child is not overlooked. While . There are four main types of CP: spastic, dyski-
care-giving is a normal part of being the parent of a netic, ataxic and mixed; each syndrome is classi-
young child, this role takes on an entirely different sig- fied according to type, distribution and severity of
nificance when providing ongoing care to a child and motor dysfunction
young adult with functional limitations. Providing FCC . The syndromes of CP have specific secondary
can go some way to improving parental satisfaction and musculoskeletal, GI and respiratory complications
mental health and ensures any duty of care to parents as and an association with epilepsy, cognitive, sen-
patients is not neglected. sory and communication impairments
. Goals for treatment are to improve quality of life
Finally, the GP is able to offer continuity of care and by improving function and reducing disability; the
longitudinal support. Acting as a point of access through- GP plays a pivotal role in coordinating a multidis-
out the patient’s life the GP can aid in the transition of the ciplinary team, facilitating FCC and ensuring con-
child to adult services and help address the needs of tinuity of care
adults ageing with a disability. . Prognosis of children with CP can usually be pre-
dicted by 2 years of age; survival to adulthood is
common among all but the most severely affected
children but the level of mobility that can be
Prognosis
...........................................................
achieved varies with type of CP and the GMFCS
should be used for accurate predictions
The two most important issues for parents of children . When dealing with those affected by CP an
with CP are whether their child will be able to walk honest, open and empathetic attitude, lifelong
and, more importantly, whether their child will lead a monitoring and referral to appropriate services is
long life. The extent of motor impairment is difficult to key to managing the situation
predict in young children. Prognosis of motor function
and the ability to walk will depend on type of CP, rate of
motor development, presence of developmental reflexes, References and further information
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Dr Zahra Neshat
FY2, Wycombe General Hospital Emergency Medical Centre
Email: zneshat@googlemail.com

DOI: 10.1177/1755738014540778
............................................................................................................................................................

AKT question relating to childhood

asthma
..............................

Single Best Answer Question A. Failure to respond to 10 puffs of salbutamol

B. Heart rate 120 beats/minute
A 4-year-old boy attends with an exacerbation of asthma.
C. Peak expiratory flow rate 66% of predicted
D. Respiratory rate 30 breaths/minute
According to current British Thoracic Society
E. Widespread bilateral expiratory wheeze
guidance, which ONE of the following criteria
warrants referral to hospital? Select ONE
Answer DOI: 10.1177/1755738014540779
option only.

Dr Sean McDermott
Locum GP, Northern Ireland

492
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