Nervous system 2 Flashcards

1
Q

List causes of fits, faints and funny turns:

1) In infancy
2) Beyond infancy
3) School-age

A

1) In infancy:
- Apnoea and acute life-threatening episodes
- Febrile convulsions
- Breath-holding
- Infantile spasms
- Epilepsy
- Hypoglycaemia and metabolic conditions

2) Beyond infancy
- Febrile convulsions
- Breath-holding
- Night terrors
- Epilepsy
- Benign paroxsymal vertigo

3) School-age
- Epilepsy
- Syncope
- Hyperventilation
- Hysteria
- Tics

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2
Q

What is important to include in a hx of a child presenting with a fit, faint or funny turn?

A

Episode:

  • What child was doing at onset / any precipitating factors
  • Length of episode
  • Loss / alteration of consciousness
  • Any involuntary movements
  • Change in colour: pallor / cyanosis
  • Reaction of the child to the event
  • Postictal phase?

BIND
FH

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3
Q

What physical examinations should be performed of a child presenting with a fit, faint or funny turn?

A

Cardiac and neurological examination

Check for dysmorphic features, micro- or macrocephaly and hepatosplenomegaly = suggest metabolic disorders

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4
Q

What investigations should be done child presenting with a fit, faint or funny turn?

A

EEC
ECG
Blood glucose

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5
Q

What is BIND?

A

Birth
Immunisations
Neonatal
Development

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6
Q

What are characteristic features of apnoea and acute life threatening events (ALTE)?

A

Usually found limp or twitching
Age <6 months
Usually no precipitating event

Concern over whether a cardiac arrhythmia, convulsion or choking episode has precipitated the event

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7
Q

What is the usual course of management of apnoea and acute life threatening events (ALTE)?

A

Usually admitted for observation and discharged after an uneventful night

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8
Q

What can precipitate syncope?

A

= Vasovagal

Pain
Emotion
Prolonged standing

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9
Q

How does syncope present?

A

Blurred vision
Light-headedness
Sweating
Nausea

Resolved on lying down

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10
Q

List some causes of headaches (11)

A

1) Tension headache
2) Migraine
3) Raised ICP
4) HTN
5) Dental caries
6) Infection
7) Meningitis (acute)
8) Haemorrhage (rare)
9) Tumour (rare)
10) Eye strain
11) Sinusitis

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11
Q

List some red flags of a child presenting with a headache (10)

A

1) Acute onset of severe pain
2) Fever
3) Headache intensified by lying down
4) Associated vomiting
5) Fall-off in school performance or regression of developmental skills
6) Consistently unilateral pain
7) Cranial bruit
8) HTN
9) Papilloedema
10) Fall-off in growth

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12
Q

Describe the character of a tension headache

A

Constricting and band-like

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13
Q

When is a tension headache worst?

A

Towards the end of the end of the day

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14
Q

Are there any associated symptoms or findings on examination of a tension headache?

A

No

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15
Q

Describe the character of a migraine

A

Throbbing, unilateral

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16
Q

What features are associated with a migraine?

A

N&V
Aura
Photophobia
FH

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17
Q

Are there any findings on physical examination of a migraine?

A

No

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18
Q

Describe the character of a headache due to raised ICP

A

Worse on lying down, may be localised to the site of the lesion

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19
Q

Describe the timing of a headache due to raised ICP

A

Worse early morning

Wakes child at night

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20
Q

List some associated features of raised ICP

A

Vomiting without nausea

Other features depend on site of lesion

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21
Q

What may be found on physical examination of a child presenting with a headache due to raised ICP

A
Slow pulse
High BP
Papilloedema
Enlarged head circumference
Focal signs
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22
Q

What should be included on physical examination of a child presenting with a headache?

A

Is the child ill?
- Fever, meningeal signs and reduced level of consciousness indicate meningitis / meningoencephalitis

HTN

Signs of raised ICP
- Slow pulse, high BP, papilloedema and enlarging head circumference (preschool child)

Focal neurological signs

  • Cranial nerve palsies and cerebellar signs suggest infratentorial tumour
  • Focal spasicity indicate cerebral lesion
  • Delayed growth and puberty indicate pituitary tumour
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23
Q

What investigations are done for a child presenting with a headache?

A

Rarely done unless evidence of raised ICP or neurological signs

CT / MRI

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24
Q

What is the management of a tension headache?

A

Reassurance

Avoid medications as they are unlikely to help

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25
Q

What is thought to be the pathophysiology of a migraine?

A

Constriction followed by vasodilation of the intracranial arteries

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26
Q

What is the typical age of onset of a migraine in a child?

A

Late childhood or early adolescence

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27
Q

How does a migraine present?

A

Preceded by aura (vasoconstriction)

Within a few minutes a throbbing, unilateral headache occurs accompanied by n&v

Last 1-72hrs

Sleep usually ends the attack

Rarely, complicated migraine occurs when focal neurological symptoms and sign are present

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28
Q

How does a migraine present in younger children?

A

Attack often bilateral with no aura, nausea or vomiting

29
Q

What is the management of migraine?

A

1st line: rest with simple analgesia

Avoid triggers eg chocolate, cheese, citrus fruits, nuts, caffeine

If frequent, prophylaxis with propanolol or pizotifen

Sumatriptan (5-hydroxy-tryptamine agonist) can help abort acute migraine in adolescents but is not recommended in childhood

30
Q

List some causes of hydrocephalus

A

Obstruction of CSF flow:

  • Congenital abnormality of the brain eg aqueductal stenosis
  • Acquired as a result of intracranial haemorrhage, infection or tumour

Failure to resorb CSF

31
Q

What group of children are particularly at risk of hydrocephalus?

A

Premature babies with severe intracranial haemorrhage

32
Q

What is hydrocephalus associated with?

A

NTD

Occurs in 80% of children with spina bifida

33
Q

How does hydrocephalus present?

A

Accelerated head growth:

  • Anterior fontanelle is wide open and bulging
  • Sutures separated
  • Scalp veins dilated
  • Forehead is broad and eyes deviated down = ‘setting sun’ sign

Irritability, lethargy, poor appetite and vomiting common

Spasticity, clonus and brisk deep tendon reflexes

34
Q

How does hydrocephalus present in older children?

A

More subtle

Headache and deterioration in school performance

35
Q

How is hydrocephalus managed?

A

Urgent

Cranial USS or MRI used to determine most appropriate neurological procedure

Most commonly an extracranial shunt put in to drain CSF
- Most shunts are ventriculoperitoneal = CSF drains from lateral ventricle into peritoneal cavity (ventriculoatrial rarely used)

36
Q

What are some complications of shunt placement in hydrocephalus?

A

Blockage and infection

Parents must be educated to recognise the features of raised ICP - which indicate these problems

37
Q

What is the prognosis of children with hydrocephalus?

A

At risk of developmental disabilities and LD, esp relating to performance tasks and memory

Visual problems common

Important to receive long term follow up

38
Q

What is hydrocephalus?

A

Obstruction of CSF flow leads to dilatation of ventricles

When skulls are not fused this causes grossly enlarged head and raised ICP

39
Q

What is a non-communicating vs a communicating cause of hydrocephalus?

A

Non-communicating (obstructive) - CSF blocked along one/more of the narrow passages connecting the ventricles:

  • Aqueduct stenosis
  • Posterior fossa
  • Tumours

Communicating - CSF blocked after it exits the ventricles, but CSF can still flow between ventricles:

  • Meningitis
  • SAH
  • IVH
40
Q

What is a tic?

A

A rapid, repetitive, brief, involuntary movement such as blinking, jerking or facial grimacing

41
Q

What can precipitate a tic? Who gets them?

A

Anxiety, fatigue or excitement

Common in school children
Peak age 8-11yrs
M>F

42
Q

What can tics resemble and how can they be differentiated?

A

Simple or complex partial (focal) seizures

Differentiated by the fact that tics can be controlled voluntary and are not associated with alteration in consciousness

43
Q

What features indicate an immediate CT with a child presenting following a head injury? (10)

A

1) Witnessed LOC >5 mins
2) Amnesia >5mins
3) >2 discrete episodes of vomiting
4) Clinically suspected NAI
5) Post traumatic epilepsy
6) GCS <14 or <15 for an infant
7) Suspected open or depressed skill injury or tense fontanelle
8) Focal neurological deficit
9) Swelling, bruising or laceration >5cm
10) Dangerous mechanism of injury

44
Q

What is plagiocephaly?

A

Common asymmetry of the skull

Normal head circumference

Usually improves over the first few months

45
Q

How does plagiocephaly present?

A

Flat spot on the back or one side of the head

Cuased by remaining in supine position for too long (sleeping position)

No associated symptoms

Does not affect the brain

46
Q

How is plagiocephaly managed?

A

Early recognition
‘Tummy time’
Altering positioning of toys
Alleviate pressure from head whilst in car seat
Physiotherapy if difficulty turning to one side?

?Helmets and bands - controversial

47
Q

What syndrome has motor and vocal tics (rarely tic free)?

A

Tourett’s syndrome

48
Q

How is Tourett’s syndrome managed?

A

Clonidine / risperidone

49
Q

What is the commonest neuromuscular disorder of childhood?

A

Duchenne muscular dystrophy (DMD)

50
Q

What are the genetics involved in DMD?

A

X linked recessive condition caused by a mutation in dystrophin gene on Chr X(q28) - a protein which keeps muscle cells in tact

51
Q

How does DMD present?

A

Typically presents by 4yr with motor and speech development delay

  • Waddling gait
  • Gower’s sign
  • Hyporeflexia
  • Calf pseudohypertrophy (muscle replaced with fat)
  • Sparing of facial, extraocular and bulbar muscles
52
Q

What investigations are done for DND?

A

Bloods show raised serum creatinine kinase

Measure all boys not walking by age 18m

53
Q

What is the management of DMD?

A

Prophylactic - genetic counselling

Supportive - physiotherapy, mobility aids

54
Q

What is the prognosis of DMD?

A

Affected children develop progressive cardiomyopathies

Ability to walk lost by age 10

Death by cardiorespiratory failure by early 20s

55
Q

What is Gower’s sign?

A

Using hands and arms to “walk” up their own body from a squatting position

Due to lack of hip and thigh muscle strength

56
Q

What is spina bifida?

A

Failure of closure of the posterior neuropore

57
Q

How is spina bifida prevented?

A

Periconceptual folic acid supplementation (reduced incidence by 75%)

5mg

58
Q

What monitoring is done throughout pregnancy to check for spina bifida?

A

USS or alpha-fetoprotein (raised in spina bifida)

59
Q

List 4 types of spina bifida

A

1) Anencephaly
2) Myelomenigocoele
3) Meningocoele
4) Spina bifida occulta

60
Q

What is anencephaly?

A

Most severe form

Complete failure of the development of the cranial part of the neural tube and the brain does not develop

61
Q

What is myelomeningocoele?

A

Open lesion with the malformed and exposed spinal cord (myelocoele) being covered in a thin membrane of meninges (meningoecele)

62
Q

What is myelomeningocoele associated with?

A

Severe neurological abnormality of the LL, bladder and anal innervation together with hydrocephalus in 90%

Surviving children have major disabilities requiring lifelong supervision

63
Q

What is meningocoele?

A

Spinal cord is intact and functions normally but defect involves an exposed bag of meningeal membranes which rupture easily

64
Q

What are major risks in meningocoele?

A

Meningitis and hydrocephalis

Rapid surgical closure necessary to avoid infection

65
Q

What is spina bifida occulta (SBO)?

A

‘hidden’ abnormality of the developing neural tube which, if unrecognised and untreated, may later cause serious neurological disability

SBO may be the only visible feature of tethering of the spinal cord within the spinal canal, with eventual stretching of the cord

66
Q

What features are associated with SBO?

A

Bladder dysfunction and pyramidal tract sings in lower limbs as child growths

Subtle abnormality of midline over spine:

  • Deep pit over lower back
  • Tuft of hair
  • A naevus
  • A lipoma at or near the midline
67
Q

How SBO investigated?

A

US

68
Q

How does spina bifida present?

A
Can occur at any spinal level
Swelling over spine 
Spinal cord exposed 
Causes paralysis below lesion 
Hydrocephalus
Neuropathic bladder - incontinence
Faecal incontinence