BNF for Children (BNFC) 2018-2019

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3 Movement disorders


Cerebral palsy and spasticity 27-Feb-2017


Description of condition


Cerebral palsy is a group of permanent, non-progressive
abnormalities of the developing fetal or neonatal brain that
lead to movement and posture disorders, causing activity
limitation and functional impact. There can be
accompanying clinical and developmental comorbidities.
These include disturbances of sensation, perception,
cognition, communication and behaviour; epilepsy; and
secondary musculoskeletal problems (such as muscle
contracture and abnormal torsion). Cerebral palsy is not
curable and the comorbidities can impact on many areas of
participation and quality of life, particularly eating, drinking,
comfort, and sleep.


Spasticity
Spasticity in children is most commonly associated with
cerebral palsy, but can also be associated with other non-
progressive brain disorders.


Aims of treatment


Treatment involves managing spasticity to optimise
movement and posture, while minimising potential
secondary musculoskeletal deformity, as well as managing
developmental and clinical comorbidities.


Drug treatment


Spasticity
gOral diazepam p. 220 or oral baclofen p. 649 can be
used to treat discomfort, pain, muscle spasm and functional
disability. If oral diazepam is initially used because of its
rapid onset of action, consider changing to oral baclofen if a
sustained, longer duration of effect is required or if long-
term treatment is necessary. If the response to diazepam or
baclofen after 4 – 6 weeks is unsatisfactory, a trial of
combined treatment using both drugs can be considered.
Consider reducing the dose if adverse effects such as
drowsiness occur. Treatment cessation should be considered
when management is reviewed and at least every six months.
Ifdystoniais considered to contribute significantly to
problems with posture, function and pain, a trial of
treatment with oral trihexyphenidyl hydrochloride p. 257 ,
levodopa, or baclofen [unlicensed indications] can be
considered.
Treatment with botulinum toxin type A p. 258 [unlicensed
under 2 years] should be considered for those in whom focal
spasticity of the upper or lower limbs is inhibitingfine motor
function, affecting care and hygiene, causing pain or
disturbing sleep, impeding tolerance of other treatments
(such as orthoses), or causing cosmetic concerns. A trial of
botulinum toxin type A treatment can also be considered in
those with spasticity whenfocal dystoniais causing
problems, such as postural or functional difficulties or pain.
Ongoing assessment of muscle tone, range of movement and
motor function is required. Botulinum toxin type A
treatment shouldnotbe offered to children receiving
treatment with Aminoglycosides p. 310.
Treatment with intrathecal baclofen [unlicensed under
4 years] (administered by continuous pump) can be
considered in children with spasticity if (despite the use of
non-invasive treatments) spasticity or dystonia are causing
difficulties with pain, muscle spasm, posture, function or
self-care. Before deciding to implant the intrathecal baclofen
pump, an intrathecal baclofen test should be performed to
assess the therapeutic effect and to check for adverse
effects.h


Developmental and clinical comorbidities
gRegular assessment and appropriate nutritional
support should be provided, especially if there are concerns
about oral intake, growth or nutritional status. Enteral tube
feeding can be provided if oral intake is insufficient to
provide adequate nutrition.h
Children with cerebral palsy are likely to have risk factors
for low bone mineral density and can have an increased risk
of low-impact fractures.gCalcium and vitamin D
supplementation (such as colecalciferol with calcium
carbonate p. 633 ) may be required. Children with reduced
bone density and a history of low-impact fracture can be
considered for bisphosphonate therapy (under specialist
guidance).h
Pain is common in children with cerebral palsy, especially
those with more severe motor impairment. Condition-
specific causes of pain and discomfort include
musculoskeletal problems (for example, scoliosis, hip
subluxation and dislocation), increased muscle tone
(including dystonia and spasticity), muscle fatigue and
immobility, constipation, vomiting, and gastro-oesophageal
reflux disease. Reversible causes of pain should be treated as
appropriate, seeSpasticity(above), Constipation p. 39 ,
Gastro-oesophageal reflux disease p. 59 , Urinary frequency,
enuresis and incontinence p. 488 , Nocturnal enuresis in
children p. 488 and Urinary-tract infections.
Other types of pain include non-specific back pain,
headache, non-specific abdominal pain, dental pain and
dysmenorrhoea.gInitially, a’stepped approach’trial of
simple analgesia (such as paracetamol p. 271 , ibuprofen
p. 655 , or both) for mild-to-moderate pain can be
considered. If such a trial is unsuccessful, the child should be
referred to a specialist pain team.h
In children with cerebral palsy, sleep disturbances are
common.gIf no treatable cause is found, a trial of
melatonin p. 295 [unlicensed] can be considered to manage
sleep disturbances, particularly for problems with falling
asleep. Regular sedative medication shouldnotbe used to
manage primary sleep disorders in children with cerebral
palsy without seeking specialist advice.h
Epilepsy can be associated with cerebral palsy; for its
management, see Epilepsy p. 191.
Children with cerebral palsy are at greater risk of mental
health problems, in comparison with the general age
comparison population. For management of these
conditions, see Depression (Antidepressant drugs p. 235 ),
Anxiety (Hypnotics and anxiolytics p. 293 ), and Attention
deficit hyperactivity disorder p. 225.
Drooling
gDrooling can be managed with an antimuscarinic drug,
such as glycopyrronium bromide p. 811 [unlicensed in under
3 years] (oral or by enteral tube) or hyoscine hydrobromide
p. 266 [unlicensed] (transdermal). For children with
dyskinetic cerebral palsy, trihexyphenidyl hydrochloride
[unlicensed] can be used (on specialist advice). If
antimuscarinic drugs provide insufficient benefit or are not
tolerated, botulinum toxin type A [unlicensed indication]
injections to the salivary glands can be considered. High-
dose botulinum toxin type A [unlicensed indication]
injections to the salivary glands can, rarely, cause breathing
and swallowing difficulties, which requires urgent hospital
admission. Surgery can be an option in cases resistant to
drug treatment.h

Non-drug treatment
gAll children with spasticity should be offered
physiotherapy and, where necessary, occupational therapy.
They can also benefit from orthoses.
Orthopaedic surgery can be used, as an adjunct to other
interventions, to prevent deterioration and improve
function.h

BNFC 2018 – 2019 Movement disorders 255


Nervous system

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