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Year 4 - Paediatrics > Neurology > Flashcards

Neurology Flashcards

1
Q

Pathophysiology of vasovagal syncope.

A

Strong stimulus to the vagus nerve causes parasympathetic activation.

Parasympathetic activation counteracts the sympathetic nervous system.

As a result, cerebral blood pressure drops leading to hypoperfusion, so the patient loses consciousness.

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2
Q

Symptoms of syncope.

A

Prodromal period:
- clammy
- sweaty
- heavy
- dizzy
- vision going blurry
- headache

Collateral history may reveal:
- sudden loss of consciousness
- unconscious for a few minutes
- twitching, shaking

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3
Q

Causes of primary syncope.

A
  • dehydration
  • missed meals
  • extended standing in a warm environment
  • vasovagal response to stimuli (e.g. surprise, pain, blood)
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4
Q

Causes of secondary syncope.

A
  • hypoglycaemia
  • dehydration
  • anaemia
  • infection
  • anaphylaxis
  • arrhythmias
  • valvular heart disease
  • HOCM
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5
Q

Syncope vs Seizure.

A
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6
Q

What examinations are required following syncope?

A
  • secondary survey looking for injuries
  • neurological examination
  • cardiac examination (pulses, heart rate, rhythm, mumur)
  • lying and standing BP
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7
Q

Syncope investigations.

A
  • ECG
  • 24hr ECG
  • echocardiogram
  • FBC (?anaemia)
  • U&Es (?arrythmia ?seizure)
  • blood glucose (?diabetes)
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8
Q

Management of fainting in children.

A

Common and usually resolve by the time they reach adulthood.

Once a simple vasovagal episode is diagnosed, simple advice can be given:
- avoid dehydration
- avoid missing meals
- avoid standing for long periods

Secondary syncope may require further investigation and referral to specialist.

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9
Q

What is a generalised tonic-clonic seizure?

A

Loss of consciousness with:
- tonic (muscle tensing)
- clonic (muscle jerking)

There may be associated tongue-biting, incontinence, groaning and irregular breathing.

After the seizure there is a prolonged postictal period where the person is confused, drowsy and irritable.

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10
Q

Management of tonic-clonic seizures.

A

First line: sodium valporate

Second line: lamotrigine or carbamazepine

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11
Q

What area of the brain is affected in focal seizures?

A

Start in the temporal lobes, therefore affect:
- hearing
- speech
- memory
- emotions

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12
Q

Presentations of focal seizures.

A
  • hallucinations
  • memory flashbacks
  • déjà vu
  • doing strange things on autopilot
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13
Q

Management of focal seizures.

A

First line: carbamazepine or lamotrigine

Second line: sodium valporate

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14
Q

What are absence seizures?

A

Patient becomes blank, stares into space and then abruptly returns to normal after around 20 seconds.

During these episodes they are unaware of their surroundings.

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15
Q

Management of absence seizures.

A

First line: sodium valporate or ethosuximide.

Most common in children; 90% stop having absence seizures as they get older.

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16
Q

What are atonic seizures?

A

Brief lapses in muscle tone causes the patient to collapse.

May be indicative of Lennox-Gastaut syndrome.

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17
Q

Management of atonic seizures.

A

First line: sodium valporate

Second line: lamotrigine

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18
Q

What are myoclonic seizures?

A

Sudden brief muscle contractions, causing a sudden ‘jump’.

The patient usually remains awake during the episode.

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19
Q

Management of myoclonic seizures.

A

First line: sodium valporate

Second line: lamotrigine

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20
Q

What are infantile spasms?

A

A rare syndrome starting in infancy, characterised by clusters of full body spasms.

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21
Q

Prognosis of infantile spasms.

A

1/3 die by age 25.

1/3 are seizure free at age 25

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22
Q

Management of infantile spasms.

A

Difficult to manage:
- prednisolone
- vigabatrin

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23
Q

What age child is typically affected by febrile convulsions?

A

6 months to 5 years

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24
Q

Differentials to epilepsy.

A
  • vasovagal episodes
  • febrile convulsions
  • encephalitis
  • meningitis
  • sepsis
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25
Q

How are seizures investigated?

A
  • EEG
  • MRI brain (?structural problems)
  • ECG (?heart defects)
  • U&Es (Na, K, Ca, Mg)
  • blood glucose (?hypoglycaemia)
  • blood culture, urine culture & LP (?sepsis, encephalitis, meningitis)
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26
Q

What general advice should be given to families presenting with seizures?

A
  • showers instead of baths
  • supervised if swimming
  • cautious with heights
  • cautious with traffic
  • cautious with heavy, hot or electrical equipment

Older teenagers must avoid driving unless they meet specific criteria regarding control of their epilepsy.

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27
Q

MOA of sodium valporate.

A

Increasing the activity of GABA, having an inhibitory effect on the brain.

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28
Q

Sodium valporate SEs.

A
  • teratogenic*
  • liver damage and hepatitis
  • hair loss
  • tremor

*avoided in girls unless there are no suitable alternatives and strict criteria are met to ensure they do not get pregnant.

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29
Q

SEs of carbamazepine.

A
  • agranulocytosis
  • aplastic anaemia
  • CYP P450 inducer
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30
Q

Phenytoin SEs.

A

Folate deficiency:
- megaloblastic anaemia

Vitamin D deficiency:
- osteomalacia

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31
Q

Lamotrigine SEs.

A
  • Stevens-Johnson syndrome
  • leukopenia
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32
Q

Management of seizures (general).

A
  • put patient in a safe position
  • place in the recovery position
  • put something soft under patient’s head
  • remove obstacles that could lead to injury
  • time the seizure
  • call an ambulance if lasting >5 minutes or if this is their first seizure
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33
Q

What is status epilepticus?

A

Seizure lasting longer than 5 minutes

or

2 or more seizures without regaining consciousness

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34
Q

Management of status epilepticus.

A
  • A-E
  • high-concentration oxygen
  • IV lorazepam
  • rpt IV lorazepam after 5 mins
  • IV phenobarbital / phenytoin after 5 mins
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35
Q

What are simple febrile convulsions?

A

Generalised, tonic clonic seizures.

Last less than 15 minutes.

Only occur once during a single febrile illness.

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36
Q

What are complex febrile convulsions?

A
  • partial seizure
  • focal seizure

Last more than 15 minutes.

Occur multiple times during the same febrile illness.

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37
Q

Differentials for febrile convulsions.

A
  • epilepsy
  • meningitis
  • encephalitis
  • sepsis
  • space occupying lesions
  • syncope
  • electrolyte abnormalities
  • trauma (NAI)
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38
Q

Management of febrile convulsion.

A
  • identify and manage underlying source of infection
  • control fever with paracetamol / ibuprofen
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39
Q

Prognosis of a simple febrile convulsion.

A

No increased risk of epilepsy compared to normal population.

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40
Q

Prognosis of complex febrile convulsion.

A

Around 10% of children go on to develop epilepsy.

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41
Q

What are breath holding spells?

A

Involuntary episodes where a child holds their breath, usually triggered by something upsetting or scary.

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42
Q

What aged children are usually affected by breath holding spells?

A

6 to 18 months of age.

Most children outgrow them by 4 to 5 years.

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43
Q

What are the types of breath holding spells?

A
  • cyanotic breath holding spells
  • reflex anoxic seizures
44
Q

What are cyanotic breath holding spells?

A

When a child is upset, they let out a long cry then stop breathing.

This causes cyanosis and loss of consciousness.

Within a minute or so, they regain consciousness and start breathing - they can be tired and lethargic after an episode.

45
Q

What are reflex anoxic seizures?

A

When a child is startled, the vagus nerve sends a strong stimulus to the heart and causes it so stop breathing.

The child will suddenly go pale, lose consciousness and may start to have some seizure-like muscle twitching.

Within 30 seconds, the heart restarts and the child becomes conscious again.

46
Q

What is the management of breath holding spells?

A

Reassure parents that breath holding spells are not harmful in the long term, and most children grow out of them by 4 or 5 years.

They have been linked with iron deficiency anaemia; treatment of anaemia can help minimise further episodes.

47
Q

What are the causes of headaches in children?

A
  • tension headache
  • migraine
  • medication overuse headache
  • ENT infection
  • problems with vision
  • raised ICP
  • brain tumours
  • meningitis
  • encephalitis
  • carbon monoxide poisoning
48
Q

Features of tension headache.

A

Mild ache across the forehead and a pain or pressure in a band-like pattern around the head.

Tension headaches come on and resolve gradually, and do not produce visual changes or pulsating sensations.

49
Q

Tension headache triggers.

A
  • stress, fear or discomfort
  • skipping meals
  • dehydration
  • infection
50
Q

Management of tension headaches.

A
  • reassurance
  • analgesia
  • regular meals
  • avoid dehydration
  • reduce stress
51
Q

Features of migraines.

A
  • unilateral
  • severe
  • throbbing

Migrains can be associated with:
- visual aura
- photophobia
- phonophobia
- N+V
- abdominal pain*

*when a patient presents with possible migraines, ask about recurrent central abdominal pain as a child. They may have a history of abdominal migraine that started before the headaches.

52
Q

Management of migraines in children.

A
  • rest, fluids and low stimulus environment
  • paracetamol
  • ibuprofen
  • sumatriptan
  • antiemetics
53
Q

Migraine prophylaxis options.

A
  • propranolol
  • pizotifen
  • topimarate (teratogenic)
54
Q

Infective causes of headache.

A
  • viral URTIs
  • otitis media
  • sinusitis
  • tonsillitis
  • meningitis
  • encephalitis
55
Q

What is cerebral palsy?

A

A permanent neurological issue resulting from damage to the brain around the time of birth.

56
Q

Causes of cerebral palsy.

A

Antenatal:
- maternal infections
- trauma during pregnancy

Perinatal:
- birth asphyxia (HIE)
- pre-term birth

Postnatal:
- meningitis
- severe neonatal jaundice
- head injury

57
Q

What are the features of spastic cerebral palsy?

A

Damage to the upper motor neurones leads to hypertonia and reduced function.

58
Q

What are the features of dyskinetic cerebral palsy?

A

Damage to the basal ganglia results in problems controlling muscle tone, with hypertonia and hypotonia.

This leads to athetoid movements and oro-motor problems.

59
Q

What are the features of ataxic cerebral palsy?

A

Damage to the cerebellum causes problems with coordinated movement.

60
Q

What are the features of mixed cerebral palsy?

A

A combination of:
- spastic
- dyskinetic
- ataxic

features

61
Q

Patterns of spastic cerebral palsy.

A
  • monoplegia (one limb affected)
  • hemiplegia (one side of the body affected)
  • diplegia (four limbs are affected, but mostly the legs)
  • quadriplegia (four limbs are severely affected, often with seizures, speech disturbance and other impairments)
62
Q

Presentation of cerebral palsy.

A

Children at risk of developing cerebral palsy need to be followed up to identify any signs and symptoms that develop:
- failure to meet milestones
- increased or decreased tone
- hand preference below 18 months
- problems with coordination, speech or walking
- feeding or swallowing problems
- learning disability

63
Q

UMN vs LMN lesions.

A
64
Q

Complications of cerebral palsy.

A
  • learning disability
  • epilepsy
  • kyphoscoliosis
  • muscle contractures
  • hearing and visual impairment
  • reflux
65
Q

Management of cerebral palsy.

A

Manage via MDT:
- physiotherapy
- occupational therapy
- SALT
- dieticians
- orthopaedic surgeons
- paediatricians
- social workers
- charities
- support groups

66
Q

What is squint?

A

Misalignment of the eyes, meaning images on the retina do not match and the person experiences double vision.

67
Q

Define strabismus.

A

Misalignment of the eyes.

68
Q

Define amblyopia.

A

Affected eye becomes passive and has reduced function compared to the dominant eye.

69
Q

Define esotropia.

A

Inward positioned squint (affected eye towards the nose)

70
Q

Define exotropia.

A

Outward positioned squint (affected eye towards the ear).

71
Q

Define hypertropia.

A

Upward moving affected eye.

72
Q

Define hypotropia.

A

Downward moving affected eye.

73
Q

Causes of squint.

A

Usually idiopathic, but exclude secondary causes:
- hydrocephalus
- cerebral palsy
- space occupying lesions
- trauma

74
Q

Examination of Squint.

A
  • general inspection
  • eye movements
  • fundoscopy
  • visual acuity
75
Q

Hirschberg’s test for squint.

A

Shine a pen-torch at the patient from 1 meter away.

Observe the reflection of the light source on their cornea.

The reflection should be central and symmetrical - deviation from the centre will indicate a squint.

76
Q

Cover test for squint.

A

Cover one eye and ask the patient to focus on an object infront of them.

Move the cover across to the opposite eye and watch the movement of the previously covered eye.

If the eye moves inwards, it has drifted outwards when covered (exotropia); if it moves outwards, it has drifted inwards when covered (esotropia).

77
Q

Management of squint.

A

No treatment until 8 years as visual fields are still developing.

Refer to opthalmologist.

Occlusive patch can be used to cover the good eye and force the weaker eye to develop.

Atropine drops can be used in good eye to blur the vision, and force the weaker eye to develop.

78
Q

What is hydrocephalus?

A

Cerebrospinal fluid (CSF) builds up abnormally within the brain and spinal cord, either as a result of:
- over-production of CSF
- problem with draining or absorbing CSF

79
Q

Normal CSF physiology.

A

Choroid plexuses produce CSF within the ventricles.

CSF is absorbed into the venous system by the arachnoid granulations.

80
Q

Causes of hydrocephalus.

A
  • stenosis of cerebral aqueduct
  • arachnoid cysts
  • chromosomal abnormalities
81
Q

Presentation of hydrocephalus.

A
  • rapidly increasing head circumference
  • bulging anterior fontanelle
  • poor feeding and vomiting
  • poor tone
  • sleepiness
82
Q

How is hydrocephalus treated?

A

Place a ventriculoperitoneal shunt (VP) - allows the CSF to drain from the ventricles into the peritoneal cavity, where it is reabsorbed.

83
Q

VP shunt complications.

A
  • infection
  • blockage
  • excessive drainage
  • intraventricular haemorrhage
  • outgrowing the shunt*

*needs replacement around every 2 years as the child grows.

84
Q

What is craniosynostosis?

A

Skull sutures close prematurely - resulting in abnormal head shapes and restriction to growth in the brain.

If left untreated it will lead to raised intracranial pressure.

85
Q

Presentation of craniosynostosis.

A
  • abnormal head shape
  • anterior fontanelle closure before 1 year of age
  • small head in proportion to body
86
Q

Head shape in saggital synostosis.

A

Long and narrow from front to back.

87
Q

Head shape in coronal synostosis.

A

Bulging on one side of the forehead.

88
Q

Head shape in metopic synostosis.

A

Pointy triangular forehead.

89
Q

Head shape in lambdoid synostosis.

A

Flattening on one side of the occiput.

90
Q

How is craniosynostosis managed?

A

First line: skull xray

CT head to confirm the diagnosis.

91
Q

Management of craniosynostosis.

A

Surgical reconstruction of the skull.

Prognosis usually good with proper management.

92
Q

What is plagiocephaly?

A

Flattening of one area of the baby’s head, occurring where a baby had a tendency to rest their head on a particular point.

This results in the skull bones and sutures moulding with gravity to create an abnormal head shape.

93
Q

What is brachycephaly?

A

Flattening of the back of the head, resulting in a short head from back to front.

This results in the skull bones and sutures moulding with gravity to create an abnormal head shape.

94
Q

Presentation of plagiocephaly.

A

Baby aged 3-6 months with an abnormal head shape.

Often have a history of preferring to sleep on one side of their head.

95
Q

Management of plagiocephaly.

A

Exclude craniosynostosis with a thorough history.

Look for congenital muscular torticollis (CMT), which is shortening of SCM on one side. Physiotherapy can help treat this.

Reassurance is key - in most cases the head shape will return to normal as the child grows.

Simple measures can be taken:
- position them on rounded side for sleep
- supervised tummy time
- using rolled towels or other props
- minimising time in pushchairs and car seats

96
Q

What is muscular dystrophy?

A

An umbrella term for genetic conditions that cause gradual weakening and wasting of muscles.

97
Q

What is Gower’s sign?

A

Children with proximal muscle weakness use a specific technique to stand up from a lying position.

98
Q

What is the management of muscular dystrophy.

A

There is no curative management of muscular dystrophy - management is aimed at allowing the person to have the highest quality of life for the longest time possible.

This involves an MDT approach:
- occupational therapy
- physiotherapy
- medical appliances

99
Q

Inheritance pattern of Duchennes muscular dystrophy.

A

X-linked recessive disorder causing a defective gene on for dystrophin.

100
Q

Presentation of Duchennes muscular dystrophy.

A

Present at around 3-5 years:
- weakness in pelvic muscles
- progressive

Usually wheelchair bound by the time they become a teenager.

101
Q

Prognosis of Duchennes muscular dystrophy.

A

25-35 years.

102
Q

Management of Duchennes muscular dystrophy.

A
  • oral steroids slow progression of weakness
  • creatine supplementation can give slight improvement in genetic strength.
103
Q

Inheritance pattern of spinal muscular atrophy (SMA).

A

Autosomal recessive condition that causes a progressive loss of motor neurones, leading to progressive muscular weakness.

Affects the lower motor neurones in the spinal cord.

104
Q

Categories of SMA.

A

SMA 1: onset within the first few months of life, usually progressing to death within 2 years.

SMA 2: onset within the first 18 months. Most never walk, but survive into adulthood.

SMA 3: onset after the first year of life. Most walk without support, but subsequently loose that ability. Respiratory muscles are unaffected, and life expectancy is close to normal.

SMA 4: onset in the 20s. Most retain the ability to walk short distances, but require a wheelchair for mobility. Everyday tasks can lead to significant fatigue.

105
Q

Management of SMA.

A

MDT:
- physiotherapy
- respiratory support with non-invasive ventilation
- PEG feeding when a weak swallow makes swallowing unsafe

Year 4 - Paediatrics (17 decks)

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