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TAS Development ✅ > Cerebral Palsy ✅ > Flashcards

Cerebral Palsy ✅ Flashcards

1
Q

How do upper motor neuron lesions usually present?

A

Hypertonia

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2
Q

What is the most common disabling condition in children with hypertonia?

A

Cerebral palsy

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3
Q

What are the most disabling types of CP?

A

Spastic and dystonic types

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4
Q

What is lost in spastic and dystonic types of CP?

A

There is a loss of the usual balance of excitatory and inhibitory muscle control

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5
Q

What is CP?

A

A descriptive term which has been defined as ‘a group of permanent disorders of movement and posture causing activity limitation that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain’

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6
Q

What are the motor disorders of CP often accompanied by?

A
  • Disturbances of sensation, perception, cognition, communication and behaviour
  • Epilepsy
  • Secondary musculoskeletal disorders
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7
Q

Are children with CP always hypertonic?

A

No

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8
Q

What are the four main clinical types of CP?

A
  • Spastic
  • Dystonic
  • Ataxic
  • Choreo-athetoid
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9
Q

What characterises spastic CP?

A

A velocity dependent increase in tonic stretch receptors

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10
Q

How must spasticity be assessed in spastic CP?

A

Must be dynamic

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11
Q

What is the spasticity described as in spastic CP?

A

‘Clasp knife’

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12
Q

What characterises dystonic CP?

A

Spasm and sustained contraction of muscles, leading to abnormal posturing

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13
Q

What can dystonic CP lead to over time?

A

Contractures

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14
Q

What is dystonic CP often described as feeling like?

A

Lead pipe rigidity

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15
Q

What characterises ataxic CP?

A

Uncoordinated movements linked to a disturbed sense of balance and depth perception

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16
Q

What characterises choreo-athetoid CP?

A

Hyperkinesia (excess of involuntary movements), which are often writhing in nature

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17
Q

What % of cases of CP are unilateral spastic?

A

25%

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18
Q

What areas of the brain are most commonly affected in unilateral spastic CP?

A

Infarction within the distribution of middle cerebral artery (left MCA distribution more commonly affected than right)

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19
Q

What are the potential causes of unilateral spastic CP?

A
  • Intrapartum asphyxia
  • Ischaemia (stroke) in late third trimester
  • Intraventricular haemorrhage related to prematurity
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20
Q

What % of cases of CP are bilateral spastic?

A

55%

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21
Q

What is the more common type of bilateral spastic CP?

A

Roughly equal proportions diplegia to quadriplegia

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22
Q

What area of the brain is most commonly affected in bilateral spastic CP?

A
  • Bilateral cerebral hemisphere infarction

- Cerebral dysgenesis

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23
Q

What are the most common causes of bilateral spastic CP?

A
  • Genetic
  • Infections in early pregnancy, e.g. CMV, toxoplasmosis
  • Vascular accidents/malformations
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24
Q

What are the less common causes of bilateral spastic CP?

A
  • Neonatal meningitis or metabolic insult

- Postnatal causes

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25
What % of cases of CP are dyskinetic?
15%
26
What area of the brain is most commonly affected in dyskinetic CP?
Discrete gliotic lesions in the putamina and thalami
27
What are the potential causes of dyskinetic CP?
- Acute profound asphyxia in the late third trimester or intrapartum - Bilirubin encephalopathy
28
What are some causes of intrapartum asphyxia?
- Cord prolapse - Antepartum haemorrhage - Uterine rupture
29
What % of cases of CP are ataxic?
5%
30
What are the most common causes of ataxic CP?
- Abnormal development of cerebellum | - Dysmorphic syndromes
31
What are the less common causes of ataxic CP?
- Postnatal infection or trauma
32
Do children with CP always fit into one category?
No, they often have a mixed picture of movements
33
What is the Gross Motor Function Classification System used for?
To describe the functional ability of a child's gross motor skills
34
What other conditions can mimic CP?
- Spinal cord tumours - Channelopathies - Sandifer syndrome - MECP2 duplication - Congenital dopa-responsive disorders - Genetic spastic paraplegia - Some metabolic conditions - Ataxia telangiectasia
35
Give 2 examples of metabolic conditions that can mimic CP?
- GLUT1 deficiency | - Glutaric aciduria type 1
36
When does particular care need to be taken in the diagnosis of CP to check there is no underlying cause?
- When there is a family history, to ensure not an underlying inheritable cause - When child presents with 'ataxic cerebral palsy'
37
What is required to meet a child with CP's complex needs?
MDT approach
38
What are the potential sites of action of drugs that manage spasticity?
- Motor cortex - Spinal cord - Muscle
39
What kind of drug is diazepam?
Gamma-aminobutyric acid (GABA) agonist
40
What is GABA?
The principal inhibitory neurotransmitter regulating neuronal excitability in the nervous system
41
What does GABA have a direct effect on?
Muscle tone
42
What is the result of GABA acting centrally?
It relieves spasticity and muscle spasm, but has additional effects of sedation and acts as an anxiolytic
43
What can long-term use of GABA lead to?
Dependency
44
What is short term use of GABA useful for?
Painful spasms, e.g. after surgery, or to break cycles of distress and increasing spasticity
45
What kind of drug is baclofen?
A GABA agonist
46
At what level does baclofen inhibit neuronal transmission?
At a spinal level
47
What is the main adverse effect of baclofen?
Sedation
48
Is sedation a significant side effect of baclofen?
Yes, it often limits its use
49
Why are high doses of baclofen often needed?
Because its passage into the CSF when given orally is poor
50
How can the dose requirement of baclofen be reduced?
By using intrathecally
51
How is baclofen given intrathecally?
Via a surgically implanted continuous-delivery pump
52
What is the advantage of giving baclofen intrathecally?
Only a tiny fraction of the equivalent oral dose needs to be given, which allows greater efficacy with fewer adverse effects
53
What kind of drug is tizanidine?
An alpha-2 adrenergic receptor agonist
54
Where does tizanidine act?
At the spinal level
55
Who is tizanidine particularly useful in?
Children who are severely disabled by CP, and in those with night-time spasms
56
Why is tizanidine useful in night-time spasms?
As it commonly has a sedative effect
57
How does botulinum toxin A work?
By chemically denervating the muscle, allowing the muscle to relax and thus improving function and cosmesis in some cases
58
How does botulinum toxin A act?
By cleaving synaptosomal-associated protein (SNAP-25)
59
What is SNAP-25?
A cytoplasmic protein which aids in the process of attaching the synaptic vesicle to the pre-synaptic membrane
60
What is the effect of cleaving SNAP-25?
It stops the release of acetylcholine at the synapse, and blocks neurotransmission
61
What happens to the effects of botulinum toxin A over time?
Gradually wear off over 3-6 months
62
Why do the effects of botulinum toxin A gradually wear off?
Because the myelin sheath retracts and new terminals are made, which form contacts with the myocyte to resume neurotransmission
63
How does dantrolene work on spasticity and spasms?
By affecting calcium uptake into skeletal muscles and decreasing free intracellular calcium concentration
64
Is sedation a problem with dantrolene?
Less so than other drugs used in CP
65
Why should dantrolene be caused cautiously?
It can cause hepatic dysfunction and blood dyscrasias
66
What non-drug treatment may be used for spasticity?
Selective dorsal rhizotomy (SDR)
67
When is SDR particularly used in treating spasticity in CP?
In more mobile groups of children with CP (GMFCS levels 2-3)
68
What happens in SDR?
Surgical procedure to divide some of the lumbar sensory nerve roots
69
Why does dividing lumbar sensory nerve roots in SDR improve spasticity?
It interrupt the sensory-motor reflex arc responsible for increased muscle tone
70
What should pre-op assessment for SDR include?
Bladder assessment
71
Why is bladder assessment required prior to SDR?
Due to the risk of affecting continence
72
What is the limitation of SDR?
The child’s general level of gross motor function unlikely to change significantly
73
What is the benefit of SDR?
May be improvements in quality of gait, and improved comfort and ability to tolerate splints and postural management programmes

TAS Development ✅ (21 decks)

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