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Paediatrics > Neurological disability > Flashcards

Neurological disability Flashcards

1
Q

What is the definition of cerebral palsy?

A

= a permanent disorder of movement and of motor function due to a non-progressive abnormality of the developing brain

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2
Q

What is the most common motor impairment of children?

A

Cerebral palsy

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3
Q

How common is cerebral palsy?

A

2 per 1000

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4
Q

What proportion of cerebral palsy cases are antenatal in origin?

A

80%

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5
Q

What proportion of cerebral palsy cases are perinatal in origin?

A

10%

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6
Q

What proportion of cerebral palsy cases are postnatal in origin?

A

10%

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7
Q

What are the antenatal causes of CP?

A

1) Cerebrovascular haemorrhage or ischaemia
2) Cortical migration disorders
3) Structural maldevelopment of the brain
4) Congenital infection (e.g. CMV)

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8
Q

What are the perinatal causes of CP?

A

HIE before/during delivery

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9
Q

What are the postnatal causes of CP?

A

1) Meningitis/encephalitis/encephalopathy
2) Head injury
3) Symptomatic hypoglycaemia
4) Hydrocephalus
5) Hyperbilirubinaemia

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10
Q

What are the antenatal RISK FACTORS for CP?

A

1) Pre-term birth
2) Chorioamnionitis
3) Maternal respiratory tract/GU infection requiring hospital treatment

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11
Q

What are the perinatal RISK FACTORS for CP?

A

1) LBW
2) Chorioamnionitis
3) Neonatal encephalopathy
4) Neonatal sepsis
5) Maternal respiratory tract/GU infection requiring hospital treatment

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12
Q

How might CP appear on MRI?

A
  1. White matter damage
  2. Basal ganglia or deep grey matter damage
  3. Congenital malformation
  4. Focal infarcts
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13
Q

What are the most commonly delated motor milestones seen in CP?

A
  1. Not sitting by 8 months
  2. Not walking by 18 months
  3. Early asymmetry of hand function (hand preference) before 1 year
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14
Q

How may CP be categorised?

A
  1. Spastic (90%)
  2. Dyskinetic
  3. Ataxic
  4. Other
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15
Q

What is the pathophysiology behind spastic CP?

A

Damage to the UMN pathway - pyramidal or corticospinal tract

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16
Q

What are the different types of spastic CP?

A
  1. Unilateral (hemiplegia)
  2. Bilateral (quadriplegia)
  3. Bilateral (diplegia)
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17
Q

How do children with hemiplegic spastic CP tend to present?

A

Between 4-12 months

Fisting of the affected hands, flexed arm and pronated forearm

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18
Q

Describe typical features of a child with quadriplegic spastic CP:

A

All limbs involved
Poor head control
Trunk involved, tendency for extensor posturing
Associated with seizures, microcephaly, intellectual, speech, vision, hearing and feeding impairments

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19
Q

Describe the typical features of a child with diplegic spastic CP:

A

All limbs affected, but legs to a much greater extent
Young children will walk on their toes with scissoring of their legs
Older children may adopt a crouching gait pattern as they gain weight and are no longer able to walk sustained periods on tip-toes

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20
Q

What type of CP is most commonly associated with preterm birth and PVL?

A

Diplegic spastic CP

21
Q

What defines dyskinetic CP?

A

Involuntary uncontrolled movements - may be 1) chorea (irregular, brief, repetitive); 2) athetosis (slow writhing movements occurring more distally, e.g. finger fanning) or 3) dystonia (twisting appearance of movement)

22
Q

What is the pathophysiology of dyskinetic CP?

A

Damage to the basal ganglia and their associated (extra-pyramidal) pathways

23
Q

How will children with dyskinetic CP typically look?

A

Open mouth posture with internal rotation and extension of the arms

24
Q

How is CP managed?

A

By the MDT

25
Q

What are some novel approaches to treating hypertonia?

A

1) Botox
2) Selective dorsal rhizotomy - proportion of nerve roots in the spinal cord = selectively cut to reduce spasticity
3) Intrathecal baclofen
4) Deep brain stimulation of the basal ganglia

26
Q

What are the types of disorder that can result in a floppy (hypotonic) infant?

A
  1. Disorders of the anterior horn cell
  2. Disorders of the peripheral nerve
  3. Disorders of the neuromuscular transmission
  4. Muscle disorders
27
Q

What are the disorders of the anterior horn cell?

A
  1. SMA

2. Poliomyelitis

28
Q

What are the disorders of the peripheral nerve?

A
  1. Bells palsy
  2. Guillan-Barre
  3. Hereditary motor sensory neuropathies
29
Q

What are the disorders of the neuromuscular transmission?

A
  1. Myasthenia gravis
30
Q

What are the muscle disorders?

A
  1. DMD
  2. Becker muscular dystrophy
  3. Congenital muscular dystrophy
  4. Metabolic myopathies
  5. Congenital myopathies
  6. Polymyositis/dermatomyositis
  7. Benign acute myositis
31
Q

What is the most common neuromuscular disorder in children?

A

DMD

32
Q

What is the second most common neuromuscular disorder in children?

A

SMA

33
Q

How is SMA inherited?

A

Autosomal recessive

34
Q

How is DMD inherited?

A

X-linked recessive, but approx. 1/3rd = de novo mutations

35
Q

How can DMD be Dx?

A

Clinically + markedly elevated plasma CK

36
Q

What is the pathophysiology of DMD?

A

Mutation in DMD gene results in absence of dystrophin
Absence = influx of Ca2+, breakdown of Ca2+ calmodulin complex + excess of free radicals
Results in myofibre necrosis

37
Q

What are the 3 classical features of DMD?

A
  1. Waddling gait
  2. Gower’s sign
  3. Pseudo-hypertrophy
38
Q

What causes calf pseudo-hypertrophy?

A

Muscle fibres are replaced by fat and fibrous tissue

39
Q

Becker muscular dystrophy is caused by a mutation in which gene?

A

DMD also - it is allelic to Duchenne’s = different mutation, same gene

40
Q

Why is Becker’s clinically milder + progresses more slowly than Duchenne’s?

A

Some functional dystrophin is produced

41
Q

What is the location of 60% of childhood brain tumours?

A

Infratentoril (i.e. below the tentori cerebelli)

42
Q

What are the 2 types of hydrocephalus?

A
  1. Non-communicating - obstruction in the ventricular system

2. Communicating - failure to resorb CSF

43
Q

What can cause non-communicating hydrocephalus?

A
  1. Congenital malformation - e.g. aqueduct stenosis, chiari malformation, atresia of outflow foramina of the 4th ventricle
  2. Posterior fossa neoplasm or vascular malformation
  3. IVH in pre-term infant
44
Q

What can cause communicating hydrocephalus?

A
  1. Subarachnoid haemorrhage

2. Meningitis

45
Q

Is shunt infection were to occur, what is the most common causative organism?

A

Coagulase -ve staphylococci

46
Q

What is the definition of microcephaly?

A

Head circumference >2 SDs BELOW the mean for age and sex

47
Q

What are the congenital causes of microcephaly?

A
  1. Familial/inherited
  2. Syndromes - e.g. Down’s, William’s, DiGeorge
  3. Congenital infection - e.g. CMV, zika, rubella, toxoplasmosis
  4. Drugs - e.g. alcohol
  5. Other - e.g. maternal radiation, PKU, hypothyroidism
48
Q

What are the post-natal onset causes of microcephaly?

A
  1. Inborn errors of metabolism
  2. Syndromes - e.g. Rett syndrome
  3. TBI/HIE/stroke
  4. Infection - e.g. meningitis
  5. Toxins - e.g. chronic renal failure, lead poisoning
  6. Deprivation (of nutrient/hormone etc.) - e.g. anaemia, hypothyroidism, CHD

Paediatrics (27 decks)

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