Guideline appendices A-D, F, H-I, K

347 Pages·2016·4.76 MB·English

by  Taryn Krause

National Guideline Alliance

Version 1.0
Cerebral palsy: diagnosis
and management in
children and young people
Appendices A-D, F, H-I, K
NICE Guideline
Methods, evidence and recommendations
30 June 2016
Draft for pre consultation checks

Commissioned by the National Institute for
Health and Clinical Excellence

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Contents

Disclaimer
Healthcare professionals are expected to take NICE clinical guidelines fully into account
when exercising their clinical judgement. However, the guidance does not override the
responsibility of healthcare professionals to make decisions appropriate to the circumstances
of each patient, in consultation with the patient and/or their guardian or carer.
Copyright
© 2016 National Guideline Alliance
Funding
Registered charity no. 213280

© 2016 National Guideline Alliance
Cerebral Palsy: diagnosis and management in children and young people
Contents

Contents
Appendices .......................................................................................................................... 8
Appendix A: Scope ...................................................................................................... 8
Appendix B: Stakeholders .......................................................................................... 21
Appendix C: Declarations of Interest .......................................................................... 27
Appendix D: Review Protocols ................................................................................... 40
D.1 Risk factors ................................................................................................... 40
D.2 Causes of cerebral palsy .............................................................................. 41
D.3 Clinical and developmental manifestations of cerebral palsy ........................ 43
D.4 Red flags for other neurological disorders..................................................... 46
D.5 MRI and identification of causes of cerebral palsy ........................................ 47
D.6 MRI and prognosis of cerebral palsy............................................................. 49
D.7 Prognosis for walking, talking and life expectancy ........................................ 51
D.8 Information and support ................................................................................ 53
D.9 Assessment of eating, drinking and swallowing difficulties ............................ 55
D.10 Management of eating, drinking and swallowing difficulties ......................... 56
D.11 Optimising nutritional status ........................................................................ 59
D.12 Improving speech, language and communication: speech intelligibility ....... 61
D.13 Improving speech, language and communication: Communication
Systems ....................................................................................................... 63
D.14 Managing saliva control .............................................................................. 65
D.15 Risk factors for low bone mineral density .................................................... 67
D.16 Prevention of reduced bone mineral density ............................................... 69
D.17 Causes of pain, discomfort, distress, and sleep disturbance ....................... 71
D.18 Assessment of pain, distress, discomfort, and sleep disturbances .............. 73
D.19 Management of pain, distress and discomfort ............................................. 74
D.20 Management of sleep disturbance .............................................................. 77
D.21 Assessment of mental health problems ....................................................... 78
D.22 Management of mental health problems ..................................................... 80
D.23 Management of sensory and perceptual difficulties ..................................... 82
D.24 Other comorbidities in cerebral palsy .......................................................... 85
D.25 Social care needs ....................................................................................... 86
D.26 Transition to adult services ......................................................................... 88
Appendix E: Search Strategies .................................................................................. 90
Appendix F: Summary of Identified Studies ............................................................... 90
F.1 Risk factors ................................................................................................... 90
F.2 Causes of cerebral palsy ............................................................................... 92
F.3 Clinical and developmental manifestations of cerebral palsy ......................... 93
F.4 Red flags for other neurological disorders ..................................................... 94
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Cerebral Palsy: diagnosis and management in children and young people
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F.5 MRI and identification of causes of cerebral palsy ........................................ 94
F.6 MRI and prognosis of cerebral palsy............................................................. 96
F.7 Prognosis for walking, talking and life expectancy ........................................ 97
F.8 Information and support ................................................................................ 98
F.9 Assessment of eating, drinking and swallowing difficulties ............................ 99
F.10 Management of eating, drinking and swallowing difficulties ........................ 100
F.11 Optimising nutritional status ....................................................................... 101
F.12 Improving speech, language and communication: Speech intelligibility ..... 102
F.13 Improving speech, language and communication: Communication
systems ...................................................................................................... 103
F.14 Managing saliva control ............................................................................ 104
F.15 Risk factors for low bone mineral density .................................................. 105
F.16 Prevention of reduced bone mineral density .............................................. 106
F.17 Assessment of pain, distress and discomfort and sleep disturbance ......... 107
F.18 Causes of pain, discomfort, disress, and sleep disturbance ...................... 108
F.19 Management of pain, distress and discomfort ........................................... 109
F.20 Management of sleep disturbances ........................................................... 110
F.21 Assessment of mental health problems ..................................................... 111
F.22 Management of mental health problems ................................................... 112
F.23 Management of sensory and perceptual difficulties ................................... 113
F.24 Other comorbidities in cerebral palsy ........................................................ 114
F.25 Social care needs...................................................................................... 115
F.26 Transition to adult services ........................................................................ 116
F.27 Health economics...................................................................................... 117
Appendix G: Health Economics ................................................................................ 118
Appendix H: GRADE Tables .................................................................................... 119
H.1 Risk factors ................................................................................................. 119
H.2 Causes of cerebral palsy ............................................................................ 119
H.3 Clinical and developmental manifestations of cerebral palsy ...................... 120
H.4 Red flags for other neurological disorders................................................... 133
H.5 MRI and identification of causes of cerebral palsy ...................................... 133
H.6 MRI and prognosis of cerebral pasly........................................................... 133
H.7 Prognosis for walking, talking and life expectancy ...................................... 133
H.8 Information and support .............................................................................. 133
H.9 Assessment of eating, drinking and swallowing difficulties .......................... 134
H.10 Management of eating, drinking and swallowing difficulties ....................... 136
H.11Optimising nutritional status ....................................................................... 152
H.12 Improving speech, language and communication: Speech intelligibility ..... 155
H.13 Improving speech, language and communication: Communication
systems ...................................................................................................... 156
H.14 Managing saliva control ............................................................................ 160
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H.15 Risk factors for low bone mineral density .................................................. 171
H.16 Prevention of reduced bone mineral density ............................................. 172
H.17 Causes of pain, distress, discomfort and sleep disturbance ...................... 180
H.18 Assessment of pain, distress, discomfort and sleep disturbances ............. 180
H.19 Management of pain, distress and discomfort ........................................... 180
H.20 Management of sleep disturbances........................................................... 181
H.21 Assessment of mental health problems ..................................................... 185
H.22 Management of mental health problems ................................................... 186
H.23 Management of sensory and perceptual difficulties ................................... 195
H.24 Other comorbidities in cerebral palsy ........................................................ 209
H.25 Social care needs ..................................................................................... 209
H.26 Transition to adult services ....................................................................... 209
Appendix I: Forest Plots ......................................................................................... 210
I.1 Risk factors................................................................................................... 210
I.2 Causes of cerebral palsy .............................................................................. 211
I.3 Clinical and developmental manifestations of cerebral palsy ........................ 211
I.4 Red flags for other neurological disorders .................................................... 211
I.5 MRI and identification of causes of cerebral palsy ........................................ 211
I.6 MRI and prognosis of cerebral palsy ............................................................ 211
I.7 Prognosis for walking, talking and life expectancy ........................................ 211
I.8 Information and support ................................................................................ 211
I.9 Assessment of eating, drinking and swallowing difficulties ............................ 211
I.10 Management of eating, drinking and swallowing difficulties ........................ 212
I.11 Optimising nutritional status ........................................................................ 215
I.12 Improving speech language and communication: Speech intelligibility ....... 216
I.13 Improving speech, language and communication: Communciation
systems ...................................................................................................... 216
I.14 Managing saliva control .............................................................................. 216
I.15 Risk factors for low bone mineral density .................................................... 216
I.16 Prevention of reduced bone mineral density ............................................... 216
I.17 Causes of pain, distress, discomfort and sleep disturbances ...................... 217
I.18 Assessment of pain, distress, discomfort and sleep disturbances ............... 217
I.19 Management of pain, distress and discomfort ............................................. 217
I.20 Management of sleep disturbances ............................................................ 217
I.21 Assessment of mental health problems ...................................................... 218
I.22 Management of mental health problems ..................................................... 218
I.23 Management of sensory and perceptual difficulties .................................... 218
I.24 Other comorbidities in cerebral palsy .......................................................... 218
I.25 Social care needs ....................................................................................... 218
I.26 Transition to adult services ......................................................................... 218
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Appendix J: Evidence Tables .................................................................................. 219
Appendix K: Excluded Studies ................................................................................. 220
K.1 Risk factors ................................................................................................. 220
K.2 Causes of cerebral palsy ............................................................................ 236
K.3 Clinical and developmental manifestations of cerebral palsy ...................... 238
K.4 Red flags for other neurological disorders................................................... 243
K.5 MRI and identification of causes of cerebral palsy ...................................... 250
K.6 MRI and prognosis of cerebral palsy........................................................... 259
K.7 Prognosis for walking, talking and life expectancy ...................................... 266
K.8 Information and support .............................................................................. 273
K.9 Assessment of eating, drinking and swallowing difficulties .......................... 274
K.10 Management of eating, drinking and swallowing difficulties ....................... 276
K.11 Optimising nutritional status ...................................................................... 280
K.12 Improving speech and communication: Speech intelligibility ..................... 286
K.13 Improving speech, language and communication: Communication
systems ...................................................................................................... 289
K.14 Managing saliva control ............................................................................. 297
K.15 Risk factors for low bone mineral density .................................................. 300
K.16 Prevention of reduced bone mineral density.............................................. 301
K.17 Causes of pain, distress and discomfort and sleep disturbance ................ 305
K.18 Assessment of pain, distress and discomfort and sleep disturbances ....... 311
K.19 Management of pain, distress and discomfort ........................................... 313
K.20 Management of sleep disturbances ........................................................... 313
K.21 Assessment of mental health problems ..................................................... 315
K.22 Management of mental health problems ................................................... 317
K.23 Management of sensory and perceptual difficulties ................................... 319
K.24 Other comorbidities in cerebral palsy ........................................................ 322
K.25 Social care needs...................................................................................... 337
K.26 Transition to adult services ........................................................................ 342
K.27 Health Economic global search ................................................................. 347

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Scope

Appendices
1
Appendix A: Scope
2
3
NATIONAL INSTITUTE FOR HEALTH AND
4
CARE EXCELLENCE
5
SCOPE
6
1 Guideline title
7
8 Cerebral palsy: the diagnosis and management of cerebral palsy in children and
9 young people
10 1.1 Short title
11 Cerebral palsy
2 The remit
12
13 The Department of Health has asked NICE: ‘To prepare a clinical guideline on the
14 diagnosis and management of cerebral palsy’. This guideline will take account of the
15 existing NICE guideline on spasticity in children and young people with non-
16 progressive brain disorders.
3 Need for the guideline
17
18 3.1 Epidemiology
19 a) Cerebral palsy describes a group of permanent disorders of the
20 development of movement and posture, causing activity limitation,
21 resulting from non-progressive disturbances (structural abnormalities) that
22 occurred in the developing fetal or infant brain. There is general
23 consensus of an upper age limit of 2 years for onset of the non-
24 progressive brain disturbance and 5 years for clinical or developmental
25 diagnosis. Patterns of motor disorder are generally subdivided into spastic,
26 dyskinetic (including dystonic) and ataxic forms, depending on the area of
27 the brain that is mainly involved.
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Cerebral Palsy: diagnosis and management in children and young people
Scope

1 b) Although defined primarily as a motor disorder, cerebral palsy is often
2 accompanied by disturbances of sensation, perception, cognition,
3 communication and behaviour, and by epilepsy and musculoskeletal
4 problems. Recognising the interrelationship of these associated disorders
5 and managing them is an essential part of the overall management of
6 cerebral palsy.
7 c) Cerebral palsy registers using agreed definitions of the syndrome have
8 shown a prevalence of 2.0–3.5 per 1000 live births in developed countries.
9 Prevalence is inversely associated with gestational age and with birth
10 weight. Prevalence has been reported as 90 cases per 1000 live births in
11 babies with a birth weight of 1000 g, compared with 1.5 cases per 1000
12 live births for babies weighing 2500 g or more.
13 d) Cerebral palsy is attributable mostly to events that occur before birth or in
14 the neonatal period, with about 10–20% of cases resulting from
15 intrapartum asphyxia. Only about 10% of cases arise from later events
16 such as head injury or central nervous system infection (meningitis or
17 encephalitis).
18 e) In addition to prematurity and low birth weight, a wide range of risk factors
19 for cerebral palsy exist, including multiple pregnancy and especially
20 stillbirth or infant death of a co-twin, placental abnormalities, birth defects,
21 meconium aspiration, emergency caesarean section, birth asphyxia,
22 neonatal seizures, respiratory distress syndrome, hypoglycaemia and
23 maternal, fetal or neonatal infection.
24 f) It is important that disorders resulting from a progressive brain injury are
25 distinguished from cerebral palsy. Although in cerebral palsy the causative
26 brain injury is static, the secondary musculoskeletal problems and motor
27 manifestations change over time. Typically, abnormalities of movement
28 and posture are first recognised during infancy or early childhood, and
29 secondary disability can then be progressive. Attention should be paid to
30 the evolution of the condition. If this differs from the pattern expected with
31 cerebral palsy then other disorders should be considered, such as genetic
32 and metabolic disorders and disorders resulting from progressive brain
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Cerebral Palsy: diagnosis and management in children and young people
Scope

1 injury. In children and young people with dystonia the possibility of a
2 dopamine-responsive disorder should be considered.
3 g) Severe cerebral palsy can be associated with a reduced life expectancy.
4 The effect may be minimal, but if gross and fine motor functioning,
5 independent feeding, mental and visual capacities are severely impaired,
6 survival to 40 years of age may be as low as 40%. Causes of early death
7 may include pulmonary aspiration and pneumonia, accidents, associated
8 disorders (for example, congenital heart disease) and delayed recognition
9 of illness. Prognosis is an important issue that should be discussed with
10 people with cerebral palsy and their family members and carers as
11 appropriate. It can also potentially influence the approach to treatment.
12 3.2 Current practice
13 a) Management of cerebral palsy depends on a multidisciplinary team of
14 many specialists, across primary, district and regional services. The
15 multidisciplinary team works with the child or young person with cerebral
16 palsy, and their family members and carers as appropriate, to optimise
17 development and minimise the impact of the brain impairment and
18 comorbidities. The focus of social and clinical care during childhood and
19 into young adulthood, which also involves colleagues from social care and
20 education, is on facilitating function and inclusion, minimising ‘activity
21 limitation’ and enabling individual ‘participation’. These concepts are in line
22 with the World Health Organization (WHO) framework, the International
23 Classification of Functioning, Disability and Health, in which participation
24 refers to involvement in life situations across a number of functional
25 domains, including self-care, relationships, education and, later,
26 employment. This focus on functional ability and quality of life is key to
27 managing cerebral palsy, with the perspective of the child or young person
28 and their family members and carers at the centre of all decisions.
29 b) Many specialists and experts may contribute to the recognition, diagnosis
30 and management of cerebral palsy. The movement disorder itself is
31 generally picked up either because of antenatal or neonatal concern about
32 a potential brain impairment (from causes such as infection, epilepsy,
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