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Child Health > Paediatric Neurology > Flashcards

Paediatric Neurology Flashcards

1
Q

What is the underlying cause of cerebral palsy?

A

An insult to the developing brain before 3 years of age

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2
Q

Most insults leading to cerebral palsy are thought to occur at what time period?

A

Antenatally

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3
Q

What is the most common presentation of cerebral palsy, generally?

A

Delayed motor development

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4
Q

What is the most common subtype of cerebral palsy, making up 75-80% of cases? What characterises this type?

A

Spastic- a persistent increase in muscle tone

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5
Q

Which subtype of cerebral palsy affects the basal ganglia, and is characterised by involuntary movements and variable tone?

A

Dyskinetic

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6
Q

Which subtype of cerebral palsy affects the cerebellum, and is characterised by unsteadiness and poor spatial awareness?

A

Ataxic

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7
Q

What term is used to describe spastic cerebral palsy affecting a) both legs, b) a unilateral arm and leg, and c) all four limbs?

A

a) diplegic, b) hemiplegic, and c) quadriplegic

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8
Q

How should a child with cerebral palsy be fed if they are deemed to be at high risk of aspiration?

A

NG tube or gastrostomy

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9
Q

What medications can be used to help reduce spasticity associated with cerebral palsy?

A

Botox injections or oral baclofen

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10
Q

What are some musculoskeletal complications that may occur as a result of cerebral palsy, especially in those bound to a wheelchair?

A

Scoliosis and hip dislocation

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11
Q

What is the most likely cause of death in a child with cerebral palsy?

A

Aspiration pneumonia

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12
Q

Which anatomical structures are affected in muscular dystrophy?

A

Lower motor neurones and muscles

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13
Q

How do children with neuromuscular diseases usually present?

A

Floppy weakness and delayed motor milestones

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14
Q

How are muscular dystrophies inherited?

A

X linked recessive

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15
Q

The defect that occurs in muscular dystrophy impairs the formation of what protein?

A

Dystrophin

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16
Q

Gower’s sign is seen in what neurological condition affecting children?

A

Duchenne’s muscular dystrophy

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17
Q

What is the typical age of onset of a) Duchenne’s and b) Becker’s muscular dystrophy?

A

a) 2-3 years, b) teenage years

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18
Q

What is the most important blood test to perform on children with suspected muscular dystrophy?

A

Creatinine kinase

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19
Q

What investigation is used to make a definitive diagnosis of muscular dystrophy?

A

Genetic testing

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20
Q

Most patients with Duchenne’s muscular dystrophy die in their mid twenties from what complication?

A

Respiratory failure

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21
Q

Most patients with Becker’s muscular dystrophy die in their forties from what complication?

A

Cardiac failure (often cardiomyopathy)

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22
Q

What orthopaedic complication is likely to be seen in children with muscular dystrophy, particularly Duchenne’s?

A

Scoliosis

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23
Q

Congenital anomalies of the CNS are the result of insults to the developing foetus at any time up to when in development?

A

20 weeks

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24
Q

What are the two most common congenital anomalies of the CNS?

A

Neural tube defects and hydrocephalus

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25
Q

How are congenital anomalies of the CNS usually detected?

A

Antenatal ultrasound

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26
Q

The major cause of neural tube defects is maternal deficiency of what in the first month of pregnancy?

A

Folic acid

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27
Q

What is the term for the most common neural tube defect, involving a herniation of both the meninges and spinal cord tissue through a defect?

A

Myelomeningocele

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28
Q

What is the term for a neural tube defect involving a herniation of the meninges only through a defect?

A

Meningocele

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29
Q

What is the term for a neural tube defect involving herniation of brain tissue through a skull defect?

A

Encephalocele

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30
Q

What is the term for a neural tube defect where there is failure to develop part of the skull and brain, which is incompatible with life?

A

Anencephaly

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31
Q

What is the most common clinical sign of spina bifida occulta?

A

A tuft of hair over the affected vertebra

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32
Q

Most children with a neural tube defect have difficulty with what two things?

A

Bowel function and bladder control

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33
Q

Neural tube defects most commonly arise in what area of the spine?

A

Lumbo-sacral

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34
Q

Which type of neural tube defect will typically cause weakness or paralysis of the lower limbs and loss of sensation of the lower limbs and perineum?

A

Myelomeningocele

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35
Q

In children with a neural tube defect, what investigation is used to assess for any associated hydrocephalus?

A

Cranial ultrasound

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36
Q

How are neural tube defects treated in the first instance?

A

Surgical correction

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37
Q

What investigation is required for monitoring in children with a neural tube defect?

A

Ultrasound scan of the renal tract

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38
Q

Which anatomical structures are affected by polio?

A

Anterior horn (motor) cells of the spinal cord

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39
Q

A flu-like illness followed by variable degrees of paralysis is suggestive of what diagnosis?

A

Polio

40
Q

Is congenital hydrocephalus usually communicating or non-communicating?

A

Non-communicating

41
Q

What are some potential causes of congenital hydrocephalus?

A

Aqueductal stenosis or Chiari malformations

42
Q

What is often the first sign of hydrocephalus in a baby?

A

Increased head circumference

43
Q

What is a typical late presenting sign of hydrocephalus, caused by raised ICP?

A

Sunsetting gaze

44
Q

What investigation is used to detect hydrocephalus in a) babies, b) older children?

A

a) Cranial US, b) CT/MRI

45
Q

How is hydrocephalus managed?

A

VP shunting

46
Q

What is a Chiari malformation?

A

A downward displacement of the cerebellar tonsils through the foramen magnum

47
Q

An EEG showing a 3Hz/s spike and wave pattern is suggestive of what diagnosis?

A

Absence seizures

48
Q

What 3 types of seizures typically occur in juvenile myoclonic epilepsy?

A

Myoclonic, tonic-clonic and absence

49
Q

When do the myoclonic jerks associated with juvenile myoclonic epilepsy typically occur?

A

In the morning upon wakening

50
Q

What is the best treatment option for juvenile myoclonic epilepsy?

A

Sodium valproate

51
Q

What investigation should be performed in all children with a seizure?

A

ECG

52
Q

If a child presents with a seizure which is ongoing, what blood tests should be taken to exclude other causes?

A

Glucose and U&Es

53
Q

What imaging is necessary for a child with a seizure associated with a head injury?

A

CT head

54
Q

What imaging is necessary for a child aged < 2 where epilepsy has developed, or whose seizures have a focal onset?

A

MRI head

55
Q

What is the first line medication for generalised seizures?

A

Sodium valproate

56
Q

What is the first line medication for generalised seizures in sexually active girls?

A

Lamotrigine

57
Q

What is the first line medication for focal seizures?

A

Carbamazepine

58
Q

What medication should be given after a child has been seizing for 5 minutes? In what ways can this medication be given?

A

Benzodiazepines, can be given as IV lorazepam, rectal diazepam or buccal midazolam

59
Q

What medication should be given after a child has been seizing for 10 minutes?

A

A second dose of benzodiazepines

60
Q

What medication should be given after a child has been seizing for more than 10 minutes and has not responded to two doses of benzodiazepine?

A

Phenytoin infusion

61
Q

What age range of children can be affected by febrile seizures?

A

6 months to 7 years

62
Q

Siblings of a child who has a febrile seizure are how likely to develop one themselves?

A

25%

63
Q

What type of seizures usually are febrile seizures?

A

Generalised tonic clonic seizures

64
Q

What type of seizure may occur in young children following a precipitating unpleasant event, usually triggered by breath holding?

A

Reflex anoxic seizures

65
Q

What is the most common organism causing meningitis in neonates and infants aged 0-12 months?

A

Group B strep

66
Q

What is the most common organism causing meningitis in older children and adolescents aged > 12 months?

A

Neisseria meningitidis (type B)

67
Q

What is the most common group of organisms causing viral meningitis in all ages?

A

Enteroviruses

68
Q

What is the most common organism causing encephalitis in all ages?

A

Herpes simplex type 1

69
Q

What are the 3 features of meningism?

A

Headache, photophobia and neck stiffness

70
Q

What are the 3 main contraindications to lumbar puncture?

A

Raised ICP, septic shock, coagulopathy

71
Q

In encephalitis, CT may show signs of oedema and inflammation, especially in which lobes?

A

Temporal lobes

72
Q

What antibiotic is started as soon as possible in children with suspected bacterial meningitis?

A

3rd generation cephalosporin i.e. ceftriaxone IV

73
Q

A course of what medication has been shown to decrease the rate of sensorineural hearing loss in children with meningitis?

A

Dexamethasone

74
Q

What is the most common long-term complication as a result of bacterial meningitis?

A

Sensorineural hearing loss

75
Q

What is the most common cause of ataxia in children? When does this usually occur?

A

Acute cerebellar ataxia, usually occurs following a viral infection (often chickenpox)

76
Q

Ataxia which is associated with otalgia, or symptoms of otitis media/URTI, with normal tone, power and reflexes is most likely to be caused by what?

A

Labyrinthitis

77
Q

Friedreich’s ataxia is an autosomal recessive condition occurring in children of what age group? Aside from ataxia, what is a common sign that may be seen in these children?

A

8-15 years, pes cavus

78
Q

Full recovery from ataxia caused by acute cerebellar ataxia or labyrinthitis is expected within how long?

A

Days-weeks

79
Q

How is spinal muscular atrophy most commonly inherited?

A

Autosomal recessive

80
Q

What diagnosis should be suspected in a baby who has marked hypotonia and muscle weakness from birth, or presenting up to 6 months, and a poor suck and swallow reflex?

A

Spinal muscular atrophy

81
Q

Tongue fasciculations, giving the tongue a ‘bag of worms’ appearance, can be seen in which condition?

A

Spinal muscular atrophy

82
Q

What happens to CK in spinal muscular atrophy?

A

It is normal, or mildly raised

83
Q

What is usually the cause of death in babies with spinal muscular atrophy?

A

Respiratory failure

84
Q

How are both neurofibromatosis and tuberous sclerosis most commonly inherited?

A

Autosomal dominant

85
Q

Cafe au lait spots are seen in which neurocutaneous syndrome?

A

Neurofibromatosis type 1

86
Q

Axillary or inguinal freckling is seen in which neurocutaneous syndrome?

A

Neurofibromatosis type 1

87
Q

Iris hamartomas (Lisch nodules) are seen in which neurocutaneous syndrome?

A

Neurofibromatosis type 1

88
Q

Neurocutaneous syndromes, particularly tuberous sclerosis, come with an increased risk of developing what associated conditions?

A

Learning difficulties and epilepsy

89
Q

Ash leaf macules are seen in which neurocutaneous syndrome?

A

Tuberous sclerosis

90
Q

Adenoma sebaceum (acne in a prepubertal child) is seen in which neurocutaneous syndrome?

A

Tuberous sclerosis

91
Q

60% of childhood CNS tumours arise where in the brain?

A

Cerebellum

92
Q

What is the most common glioma to be seen in children?

A

Astrocytomas

93
Q

Most embryonal tumours of the CNS seen in children are what? Where are these located?

A

Medulloblastomas, located in the cerebellum

94
Q

What CNS tumour may cause children to present with visual and endocrine problems?

A

Craniopharyngiomas

95
Q

How are brain tumours in children usually treated?

A

Surgical removal

96
Q

What treatment should be avoided in children aged under 3 years with brain tumours?

A

Radiotherapy (this can damage the developing brain)

Child Health (10 decks)

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