Nervous System

Question 1

Definition of CP:

Answer 1

• Permanent disorder of motor and posture development
• Caused by non-progressive insult to developing brain (occurring in the developing brain)
• Can be associated with other developmental difficulties and epilepsy.

Question 2

When does the damage in CP occur?

Answer 2

For 50-75% of children with cerebral palsy, the causative lesion occurs between 24 weeks of postmenstrual age and term. It can occur during the birth and postnatal period also though.

Question 3

Antenatal risk factors for CP

Answer 3

preterm birth, with risk increasing with decreasing gestational age (cerebrovascular haemorrhages/ ischaemia)
chorioamnionitis
maternal respiratory tract or genito-urinary infection treated in hospital
Intrauterine infections- TORCH
Congenital syndroms/ malformations
Teratogen consumption e.g. smoking, alcohol , drugs
Multiple births
Periventricular leukomalacia

Question 4

Name some perinatal risk factors for CP:

Answer 4

low birth weight
chorioamnionitis
neonatal encephalopathy
neonatal sepsis (particularly with a birth weight below 1.5 kg)
maternal respiratory tract or genito-urinary infection treated in hospital

Question 5

Name a postnatal risk factor for CP:

Answer 5

meningitis 20%
other infections 
head injury
hypoglycaemia
hyperbilirubinaemia

Question 6

Name the four types of CP:

Answer 6

Spastic type - there may be intermittently increased tone and pathological reflexes. 70%

Athetoid - this is characterised by increased activity (hyperkinesia). This has been described as ‘stormy movement’.

Ataxic type - there may be a loss of orderly muscular co-ordination so that movements are performed with abnormal force, rhythm or accuracy.

Mixed - there may be a combination of several forms.

Question 7

Presentations of CP:

Answer 7

• unusual fidgety movements or other abnormalities of movement, including asymmetry or paucity of movement
• abnormalities of tone, including hypotonia (floppiness), spasticity (stiffness) or dyskinesia (fluctuating tone)
• abnormal motor development, including late head control, rolling, and crawling
• feeding difficulties.

Question 8

What are the most common developmental delays in children with CP?

Answer 8

• not sitting by 8 months (corrected for gestational age)
• not walking by 18 months (corrected for gestational age)
• early asymmetry of hand function (hand preference) before 1 year (corrected for gestational age).

Question 9

What is the most common to least common types of CP?

Answer 9

Bilateral spastic cerebral palsy is the most prevalent, followed by unilateral or hemiplegic cerebral palsy. Bilateral dystonic cerebral palsy is the least common.

Question 10

Associated potential problems with CP:

Answer 10

• Learning disability (IQ below 70) occurs in around 1 in 2. Severe learning disability (IQ below 50) occurs in around 1 in 4.
• Communication difficulties occur in around 1 in 2 children and young people with cerebral palsy. At least 1 in 10 need augmentative and alternative communication because of cognitive and sensory impairments and communication difficulties.
• Emotional and behavioural difficulties (eg, low self-esteem) are reported in up to 1 in 4 children and young people with cerebral palsy. Including depression, anxiety and conduct disorders, challenging behaviours, problems with peer relationships, neurodevelopmental disorders, including autism spectrum disorder (ASD) and attention deficit hyperactivity disorder (ADHD).
• Around 1 in 2 children will have some form of visual impairment. Hearing impairment occurs in around 1 in 10.
• Pain is common in people with cerebral palsy, especially those with more severe motor impairment. Common condition-specific causes of pain, discomfort and distress include: MSK problems (eg, scoliosis, hip subluxation and dislocation), Increased muscle tone (including dystonia and spasticity), Muscle fatigue and immobility.
• Vomiting, regurgitation and gastro-oesophageal reflux are common in children and young people with cerebral palsy. Around 3 in 5 children and young people with cerebral palsy have chronic constipation.

Epilepsy occurs in around 1 in 3 children with cerebral palsy. It is reported in around 1 in 2 children with dyskinetic cerebral palsy.

• Eating, drinking and swallowing difficulties and poor nutrition: failure to thrive and malnourishment.
• Bladder problems: incontinence and infections are common.
• Sleep disturbances: for example, fragmented sleep, which can occur in up to 50% of children.
• Drooling associated with poor saliva control.

-Orthopaedic problems: eg, progressive joint contractures, shortened muscles, hip or foot deformities and scoliosis.
Increased risk of low bone mineral density and therefore fractures due to osteomalacia or osteoporosis.

Question 11

Red flag indicators for neurological disorders other than cerebral palsy include:

Answer 11

• absence of known risk factors (see risk factors)
• family history of a progressive neurological disorder
• loss of already attained cognitive or developmental abilities
• development of unexpected focal neurological signs
• MRI findings suggestive of a progressive neurological -disorder
• MRI findings not in keeping with clinical signs of cerebral palsy.

Question 12

How is a diagnosis of CP made?

Answer 12

The diagnosis of cerebral palsy is based on clinical examination and parental observation. However, investigations occasionally required to exclude other diagnoses might include:

• Thyroid studies.
• Chromosomal analysis.
• Pyruvate and lactate levels to exclude mitochondrial cytopathies
• Organic and amino acid levels to exclude inborn errors of metabolism presenting with neurological symptoms
• Cerebrospinal fluid - protein, lactate and pyruvate levels may be helpful in determining whether there has been any asphyxia in the neonatal period.

Question 13

Out line the GrossMotor Function Classification System level I:

Answer 13

GMFCS Level I – walks without limitations.

Question 14

Out line the GrossMotor Function Classification System level II:

Answer 14

GMFCS Level II – walks with limitations. Limitations include walking long distances and balancing, but not as able as Level I to run or jump; may require use of mobility devices when first learning to walk, usually prior to age 4; and may rely on wheeled mobility equipment when outside of home for traveling long distances.

Question 15

Out line the GrossMotor Function Classification System level III:

Answer 15

GMFCS Level III – walks with adaptive equipment assistance. Requires hand-held mobility assistance to walk indoors, while utilizing wheeled mobility outdoors, in the community and at school; can sit on own or with limited external support; and has some independence in standing transfers.

Question 16

Out line the GrossMotor Function Classification System level IV:

Answer 16

GMFCS Level IV – self-mobility with use of powered mobility assistance. Usually supported when sitting; self-mobility is limited; and likely to be transported in manual wheelchair or powered mobility.

Question 17

Out line the GrossMotor Function Classification System level V:

Answer 17

GMFCS Level V – severe head and trunk control limitations. Requires extensive use of assisted technology and physical assistance; and transported in a manual wheelchair, unless self-mobility can be achieved by learning to operate a powered wheelchair.

Question 18

What type of gait would a patient with hemiplegic cerebral palsy have?

Answer 18

Circumduction gait- Due to excessive hip abduction as the leg swings forward creating a semi-circular movement of the leg.

Question 19

What type of gait would a patient with di/ quadriplegic cerebral palsy have?

Answer 19

spastic gait

Stiff walking and the foot is seen to be inverted and dragged along. Often accompanied by flexion of upper limbs.

Question 20

What type of gait would a patient with ataxic cerebral palsy have?

Answer 20

Ataxic gait

Instability and alternating between a narrow to wide base of gait.

Question 21

What type of gait would a patient with diplegic cerebral palsy have?

Answer 21

Toe-walking gait (“equinus”). Can get scissoring of legs

Walking on tip toe with lack of heel contact (also seen in Duchenne’s)

Question 22

Pathology of spastic CP:

Answer 22

In this type, there is damage to the upper motor neurone (pyramidal or corticospinal tract) pathway. Limb tone is persistently increased (spasticity) with associated brisk deep tendon reflexes and extensor plantar responses. The tone in spasticity is velocity dependent.

• Limb involvement is increasingly described as unilateral or bilateral to acknowledge asymmetrical signs.
• Spasticity tends to present early and may even be seen in the neonatal period. Sometimes there is initial hypotonia, particularly of the head and trunk.

-Developmental problems and the child with special needs
increased tone soon emerges as the predominant sign.

Question 23

What are the three main types of spastic CP?:

Answer 23

• unilateral (hemiplegia) – unilateral involvement of the arm and leg. The arm is usually affected more than the leg, with the face spared. Affected children often present at 4–12 months of age with fisting of the affected hand, a flexed arm, a pronated forearm, asymmetric reaching, hand function or toe pointing when lifting the child. Subsequently, a tiptoe walk (toe–heel gait) on the affected side may become evident. Affected limbs may initially be flaccid and hypotonic, but increased tone soon emerges as the predominant sign. Often no history of ischaemic event and unrecognised antenatal cause may be assumed.
• bilateral(quadriplegia)–all four limbs are affected, often severely. The trunk is involved with a tendency to opisthotonus (extensor posturing), poor head control and low central tone. This more severe form of CP is often associated with seizures, microcephaly and moderate or severe intellectual impairment. There may have been a history of perinatal hypoxic-ischaemic encephalopathy.
• bilateral(diplegia)–all four limbs,but the legs are affected to a much greater degree than the arms, so that hand function may appear to be relatively normal. Motor difficulties in the arms are most apparent with functional use of the hands. Diplegia is one of the patterns associated with preterm birth due to periventricular brain damage. The MRI brain scan may show periventricular leukomalacia.

Question 24

Pathology of dyskinetic CP:

Answer 24

Perinatal asphyxia – particularly affecting the basal ganglia. Also kernicterus, but this is now rare.

Dyskinesia refers to movements that are involuntary, uncontrolled, occasionally stereotyped and often more evident with active movement or stress. Muscle tone is variable and primitive motor reflex patterns predominate. May be described as:
• chorea–irregular, sudden and brief non-repetitive movements
• athetosis–slow writhing movements occurring more distally such as fanning of the fingers
• dystonia–simultaneous contraction of agonist and antagonist muscles of the trunk and proximal muscles often giving a twisting appearance.

Intellect may be relatively unimpaired. Affected children often present with floppiness, poor trunk control and delayed motor development in infancy. Abnormal movements may only appear towards the end of the first year of life.

Question 25

Pathology of ataxic CP:

Answer 25

Most are genetically determined. When due to acquired brain injury (cerebellum or its connections), the signs occur on the same side as the lesion but are usually relatively symmetrical. There is early trunk and limb hypotonia, poor balance and delayed motor develop- ment. Incoordinate movements, intention tremor and an ataxic gait may be evident later.

Question 26

What is the use of IMAGING in CP?

Answer 26

There has been controversy over the use of neuroimaging in the prediction of cerebral palsy in neonates. However, studies have determined that:

-Sequential ultrasound imaging can detect major intracranial lesions in patients who subsequently develop non-ambulatory cerebral palsy.

MRI can be used to investigate the cause of suspected or known cerebral palsy if this is not clear from the history and results of cranial ultrasound examinations. MRI will not accurately establish the timing of a hypoxic-ischaemic brain injury in a child with cerebral palsy.

-CT scanning may provide information about structural congenital malformations and vascular abnormalities and haemorrhages, especially in babies

Question 27

Management of CP?:

Answer 27

Refer all children with suspected cerebral palsy to a child development service for an urgent multidisciplinary assessment, in order to facilitate early diagnosis and intervention:
paediatric or adult medicine
nursing care
physiotherapy
occupational therapy
speech and language therapy
dietetics
psychology
And any care for any specific health needs or comorbidities

Question 28

Potential medical treatment for CP:

Answer 28

• Consider oral diazepam or baclofen in children and young people if spasticity is contributing to discomfort or pain, muscle spasms (for example, night-time muscle spasms) or functional disability. Diazepam is particularly useful if a rapid effect is desirable (eg, in a pain crisis). Baclofen is particularly useful if a sustained long-term effect is desired (eg, to relieve continuous discomfort or to improve motor function). If oral diazepam is initially used because of its rapid onset of action, consider changing to oral baclofen if long-term treatment is indicated.
• In children and young people with spasticity in whom dystonia is considered to contribute significantly to problems with posture, function and pain, consider a trial of oral drug treatment - eg, trihexyphenidyl, levodopa or baclofen.
• Botulinum toxin type A can be considered if focal spasticity is impeding fine motor function, compromising care and hygiene, causing pain, disturbing sleep, impeding tolerance of other treatments (such as orthoses) or causing cosmetic concerns to the child or young person.
• Phenol and ethyl alcohol are used for severe spasticity when botulinum toxin is not readily available. Phenol and ethyl alcohol are non-selective proteolytic agents and can produce selective denervation when injected into motor nerves or muscles.

Question 29

Potential surgery in Cp?

Answer 29

selective dorsal rhizotomy (a proportion of the nerve roots in the spinal cord are selectively cut to reduce spasticity)

Question 30

Physio input In CP?

Answer 30

Splints

Mobility aids

Question 31

What is a seizure?

Answer 31

A transient occurrence of signs and/or symptoms due to an abnormal excessive or synchronous neuronal activity in the brain

Question 32

In what age range do febrile seizures tend to occur?

Answer 32

6 months to 6 years- toddler

Question 33

How many children are affected by febrile seizures?

Answer 33

3%.

Risk is 10% if have 1st degree relative that had them

Question 34

Are antipyretics and tepid sponging/ cold baths useful and why?

Answer 34

No.

Seizure due to CHANGE in temperature rather than being hot.

Question 35

When to suspect febrile seizure:

Answer 35

• Right age range
• Temp over 37.8
• Clinical history of being unwell (usually viral infection)
• Typical seizure pattern

Question 36

Features of a typical febrile seizure:

Answer 36

These are generalised, tonic-clonic seizures lasting less than 15 minutes, which do not recur within 24 hours or within the same febrile illness.

If simple – does not affect intellectual
performance or risk of developing epilepsy.

Question 37

Features of an atypical febrile seizure:

Answer 37

• Focal features at onset or during the seizure.
• Duration of more than 15 minutes.
• Recurrence within the same febrile illness.

Question 38

Factors that increase the chance of developing epilepsy later in life post a febrile seizure:

Answer 38

• Atypical febrile seizure (20%)
• Abnormal neurology/neurodevelopment prior to event
• Family history in 1st degree relative of epilepsy

1% risk with one risk factor, 2% with 2, 10% with 3.

Question 39

What is the chance of having another febrile seizure in a different illness?

Answer 39

30-40%

Question 40

What should you always rule out in febrile seizures?

Answer 40

-Worrying underlying infection e.g. meningitis, UTI, LRTI

Question 41

Questions for febrile seizures:

Answer 41

• Collateral history from parents, before during and after seizure.
• Worrying features to suggest serious illness. Investigate if ANY doubt. Also think about electrolyte imbalance in D and V.
• Family history of seizures

Question 42

Advice for parent:

Answer 42

Lots of reassurance needed!

• What febrile seizures are.
• How to treat fever at home - remove excess clothing, give fluids, give antipyretics if the child is uncomfortable. Tepid sponging or excessive cooling are not recommended.
• Check for a non-blanching rash, check for dehydration and stay with the child at night.
• First aid if the child has a fit - position; do not put anything in their mouth.
• When to call 999/112/911 ambulance - a seizure lasting more than five minutes.
• When and how to seek urgent medical advice - any seizure, serious symptoms such as non-blanching rash, lack of normal alertness, dehydration, the child getting worse, the parent worried and fever for more than five days.
• If seizing keep away from sharp objects, observe in bath.

Question 43

When do you count a febrile seizure as status?

Answer 43

-Lasting longer than 30 mins. Some now see it as 15 mins. Treat as such with Benz etc. See ACC. Advise parent to come in after 5 mins.