Paeds Neuro + misc Flashcards

1
Q

How old does a child need to be to get a intramedullary nail?

A

4

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2
Q

What is a reflex anoxic seizure?

A

Episodes of tonic clonic fitting due to cardiac asystole secondary to vagal inhibition

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3
Q

What are the triggers of reflex anoxic seizure?

A

Pain
Head trauma
Cold food (ice cream)
Fright
Fever

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4
Q

What feature of a reflex anoxic seizure differentiates it from epilepsy?

A

No tongue biting

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5
Q

What is a febrile convulsion?

A

A seizure + fever in the absence of CNS infection

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6
Q

What is the age group that can be affected by febrile convulsions?

A

6m to 6y
Commonest form of childhood seizure up to age 2y

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7
Q

Give 4 features of simple febrile seizures

A

Generalised tonic clonic seizure
<15 mins
Complete recovery within 1h
DONT recur within 24h/ same febrile illness

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8
Q

What is a generalised tonic clonic seizure?

A

Muscle stiffening followed by rhythmical jerking or shaking of the limbs, which may be asymmetrical
Rolling back of eyes
Staring
Loss of consciousness

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9
Q

Give 4 features of complex febrile seizures

A

Partial (Focal) seizure

> 15 mins

Recurrence within 24h/ in same illness

Incomplete recovery wthin 1h

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10
Q

What is a partial onset/ focal seizure?

A

movement limited to 1 side of body/ 1 limb

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11
Q

What investigations should be done following a febrile seizure?

A

Exclude RFs: CNS infection

Hx of illness + identify cause

No other main Ix (don’t do an EEG)

Consider: blood glucose, urine dip + MC+S if no clear focus

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12
Q

How should febrile convulsion be managed during the seizure?

A

Start timer

Protect from injury + do not restrain

Check airway + recovery position when stopped

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13
Q

If a febrile convulsion lasts longer than 5 minutes, what should be done?

A

Call an ambulance
OR
If drugs are available: buccal midazolam or PR diazepam

If ongoing after 10 mins, repeat dose + cal ambulance

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14
Q

Which children should be admitted to hospital following a febrile convulsion? (5)

A

1st febrile seizure
<18 months old
Diagnostic uncertainty about cause
Features of recurrent Complex febrile seizure
Currently on Abx (may mask CNS infection)

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15
Q

What advice should be provided following a febrile seizure?

A

Risk of developing epilepsy is low

1/3 will have another febrile seizure (risk reduces with age)

Antipyretics don’t prevent recurrence

Ensure child completes immunisations (even if these were followed by seizure)

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16
Q

What system is used to classify seizures?

A

International League Against Epilepsy 2017

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17
Q

What are the different types of seizure?

A

Generalised
Non-motor: absence
Motor: Tonic clonic, Myoclonic, Atonic, Tonic

Focal
Aware: simple partial. Conscious, aware + remember
Impaired awareness: complex partial. Consciousness affected, may hear but not understand/ respond

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18
Q

What is the alternate name for an absence seizure? What are these characterised by?

A

Generalised non-motor seizure

person becomes blank + unresponsive for a few seconds

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19
Q

What is the difference in symptoms between tonic-clonic and myoclonic?

A

Tonic clonic: violent muscle contractions, eyes rolling back, tongue biting, incontinence and both aura + post-ictal phenomena

Myoclonic = brief arrhythmic muscular jerking movement

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20
Q

What is the most common form of childhood epilepsy?

A

Benign Rolandic Epilepsy - myoclonic seizures in 3-12y

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21
Q

What are the 3 different types of myoclonic epilepsy

A

Benign rolandic (3-12y)
Juvenile myoclonic epilepsy (12-18y)
Progressive myoclonic epilepsy

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22
Q

What is the other name for benign rolandic seizures?

A

Sylvian seizures

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23
Q

What are the signs and symptoms of benign rolandic epilepsy?

A

Often occur at night
Typically partial seizures involving face + UL with hypersalivation

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24
Q

What is seen on EEG in benign rolandic epilepsy?

A

Centrotemporal spikes

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25
Q

What is the prognosis for benign rolandic epilepsy?

A

seizures stop by adolescence

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26
Q

What are the signs and symptoms of juvenile myoclonic epilepsy?

A

Myoclonic seizures involving upper body just after waking up + beginning after puberty

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27
Q

What is progressive myoclonic epilepsy?

A

Rare syndromes of combination of myoclonic + tonic-clonic seizures, with patient deteriorating over time

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28
Q

For what type of epilepsy is treatment not usually given?

A

Benign Rolandic Epilepsy

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29
Q

What type of antiepileptic exacerbates myoclonic seizures?

A

Lamotrigine

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30
Q

What type of antiepileptic actually exacerbates absence seizures?

A

Carbamazapine

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31
Q

What is the 1st line for rescue therapy for prolonged epileptic seizures?

A

Buccal midazolam

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32
Q

Which antiepleptic drug requires monitoring?

A

Carbamazapine

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33
Q

After how long seizure-free can antiepileptic therapy be stopped?

A

2 years

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34
Q

Recall 2 Side effects of valporate

A

Weight gain
Hair loss

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35
Q

How is status epilepticus defined?

A

1 seizure lasting >5 mins OR
>2 seizures within a 5 min period without the person returning to normal between them

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36
Q

What is the age range of peak incidence for infantile spasm?

A

3-8 months

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37
Q

What is West syndrome?

A

Specific type of epilepsy characterised by infantile spasms, age of onset + EEG pattern

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38
Q

What is an infantile spasm?

A

Sudden, rapid, tonic contraction of trunk + limb muscles with gradual relaxation over 0.5-2s

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39
Q

Give 3 characteristics of West syndrome

A

‘salaam’ attacks: flexion of the head, trunk + arms followed by extension of the arms

Lasts only 1-2s but may be repeated up to 50x

Progressive mental handicap

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40
Q

What is a ‘Salaam’ attack?

A

Head goes down + arms go up in the air

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41
Q

What is seen on EEG in West Syndrome?

A

Hypsarrhythmia

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42
Q

What is the prognosis for West syndrome?

A

Poor
Most have learning difficulties
Most go onto to develop Lennox-gastaut syndrome

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43
Q

How should West syndrome be managed?

A

Guidance from paediatric neurologist within 24h
Urgent assessment by paediatric neurologist
Vigabatrin + Prednisolone

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44
Q

What investigations should be done for vasovagal syncope?

A

LSBP with ECG if indicated

FBC (to r/o anaemia/ bleeding)

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45
Q

What are the indications for CT head in children?

A

Head injury + at least 1 of:
- suspected NAI
- post-traumatic seizure
- GCS <14
- Suspected skull fracture
- Focal neurology
OR
2 other risk factors
(LOC >5 mins, abnormal drowsiness, >3 episodes of vomiting, high-impact injury, amnesia > 5 mins)

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46
Q

Describe the pathophysiology of extradural haemorrhage

A

Low impact trauma to pterion overlying the middle meningeal artery

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47
Q

How does extradural haemorrhage manifest in a patient?

A
  1. Initial LOC
  2. Briefly regains consciousness= ‘Lucid interval’
  3. LOC due to expanding haematoma + brain herniation
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48
Q

Give 2 signs of tear of the MMA

A

Battle sign
Racoon eyes

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49
Q

What are the signs of extradural haemorrhage on MRI?

A

Biconvex (or lentiform), hyperdense collection around surface of the brain
Limited by suture lines of skull.
“Lemon”

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50
Q

How should extradural haemorrhage be managed?

A

Fluid resuscitation to correct hypovolaemia
Evacuation of haematoma + arrest bleeding

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51
Q

What is the cause of subdural haemorrhage?

A

Shearing in vein as it crosses subdural space
Bleed into space between dura + arachnoid membrane

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52
Q

What are 8 symptoms of subdural in a child?

A

Bruises, esp to head + neck
Apnoea or Irregular breathing
Seizures
Altered GCS
Lethargy
Poor feeding
Cardiorespiratory collapse or sudden death.
Vomiting

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53
Q

What is often the cause of subdural in infants?

A

NAI

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54
Q

Which infants are most at risk of intraventricular haemorrhage?

A

Premature babies due to VLBW

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55
Q

What are 2 causes of intraventricular haemorrhage in infants?

A

ECMO in preterm babies with ARDS
Congenital CMV infection

ECMO = extracorporeal membrane oxygenation

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56
Q

Recall 5 signs and symptoms of intraventricular haemorrhage

A

Sleepiness + lethargy
Apnoea
Reduced Moro reflex
Hypotonia
Tense fontanelle

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57
Q

Which investigation is used to diagnose intraventricular haemorrhage in a baby?

A

Trans-fontanelle USS

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58
Q

How should intraventricular haemorrhage be treated in a baby?

A

Fluids
Anticonvulsant
Acetazolamide (to reduce CSF) and LP
Ventriculo-peritoneal shunt if hydrocephalus

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59
Q

What is the difference between communicating and non-communicating hydrocephalus?

A

Communicatig: CSF flow obstructed after it enters the ventricles
Non-communicating: flow of CSF is obstructed WITHIN the ventricles

60
Q

What are the 2 causes of communicating hydrocephalus?

A

Meningitis
SAH

61
Q

What are the possible causes of non-communicating hydrocephalus?

A

Aqueduct stenosis:
Congenital causes: Dandy-Walker malformation or Chiari malformation

Acquired: IVH/ tumour

62
Q

What is the sunset sign?

A

Eyes appear to be driven down bilaterally - sign of raised ICP

63
Q

What are the 2 key investigations for hydrocephalus?

A

Cranial USS
Measurement of head circumference

64
Q

What is the first line management for hydrocephalus?

A

Ventriculoperitoneal shunt

65
Q

What is the second line management for hydrocephalus?

A

Furosemide - to inhibit CSF production

66
Q

What % of migraines have aura?

A

10%

67
Q

Is migraine likely to be UL or BL?

A

Could be either

68
Q

What are the symptoms of cluster headaches?

A

UL (eyes/ side of face) sharp throbbing pain, causing swelling/ watering inclusters

69
Q

What are some signs that migraine is secondary to something more sinister?

A

Visual field defects
Gait or CN abnormalities
Growth failure
Papilloedema
Early morning headache

70
Q

If simple analgesia doesn’t work, what is the 2nd line pain med in migraines?

A

Nasal sumatriptan

71
Q

Recall 2 prophylactic medications for migraine

A

Topiramate
Propranolol

72
Q

What is Tourette’s?

A

Chronic + multiple tics: starting before 18y + persisting >1y

73
Q

What are the 3 types of tic?

A

Motor, vocal or phonic

74
Q

Recall 2 therapies that are useful in tics

A
  1. Habit reversal therapy (learn movements to ‘compete’ with tics)
  2. Exposure with response prevention - help the child get used to the unpleasant feeling before a tic so that they can stop the tic occuring
75
Q

What is the first line medication for tic disorder?

A

Antipsychotics - eg risperidone

76
Q

What is the inheritance pattern of Duchenne muscular dystrophy?

A

x-linked recessive

77
Q

At what age does duchenne muscular dystrophy present, and at what age can it be diagnosed?

A

1-3y presentation
Can be diagnosed at 5y

78
Q

Which gene is deleted in Duchenne muscular dystrophy?

A

Dystrophin

79
Q

Describe the pathophysiology of duchenne muscular dystrophy?

A

Dystophin gene connects cytoskeleton to muscle fibres to ECM through membrane

Where deficient –> influx of Ca –> calmodulin breakdown –> excess free radicals –> myofibre necrosis

80
Q

What type of gait is seen in Duchenne muscular dystrophy?

A

Waddling

81
Q

Other than gait, what else is affected in early DMD?

A

Language delay

82
Q

What is Gower’s sign?

A

The need to turn prone to rise - seen in DMD

83
Q

How is the heart affected by Duchenne muscular dystrophy?

A

Primary dilated cardiomyopathy

84
Q

What is elevated in the plasma in Duchenne muscular dystrophy?

A

CK (creatine kinase) due to myofibre necrosis

85
Q

How should Duchenne muscular dystrophy be managed?

A

No cure: often Mx is to alleviate the Sx
Physiotherapy to clear lungs + exercise to help prevent contractures
Medical:
- CPAP (due to weakness of intercostals)
- Glucocorticoids (to delay need for wheelchair)
- Cardioprotective drugs

86
Q

What is the difference between Beck’s MD and DMD?

A

Same signs + Sx but often less severe + progresses at a slower rate

87
Q

What is the genetic cause of myotonic muscular dystrophy?

A

Autosomal dominant trinucleotide repeat disorder

88
Q

When does myotonic muscular dystrophy present?

A

20s to 30s

89
Q

In what way is myotonic muscular dystrophy the reverse of duchenne muscular dystrophy?

A

Affects small muscles more than large muscles

90
Q

Which form of MEN is NF1 associated with?

A

MEN2

91
Q

Recall 4 signs and symptoms of NF1

A

Café au lait spots
Neurofibroma
Axillary freckles
Lisch nodule

92
Q

What is tuberous sclerosis?

A

Rare genetic condition that causes mainly benign tumours to develop in different parts of the body

93
Q

Recall 3 cutaneous features of tuberous sclerosis

A

“Ash leaf” patch
Shagreen patches (rough skin on lumbar spine)
Angiofibromata (butterfly facial distribution)

94
Q

Recall some neurological features of tuberous sclerosis

A

Infantile spasms
Focal epilepsy
Intellectual disability (often with ASD)

95
Q

Why is there a risk of hydrocephalus in tuberous sclerosis?

A

Development of subependymal giant cell astrocytoma

96
Q

Which investigations are useful for diagnosing tuberous sclerosis?

A

CT/ MRI

97
Q

What is the layman’s term for the haemangioma in Sturge Weber syndrome?

A

Port wine stain

98
Q

Where does the haemangiomatous facial lesion present in Sturge Weber Syndrome?

A

Trigeminal nerve distribution

99
Q

Recall 5 signs and symptoms of Sturge Weber Syndrome

A

Epilepsy
Contralateral hemiplegia
Phaeochromocytoma
Intellectual disability
Glaucoma

100
Q

How should Sturge Weber Syndrome diagnosis be confirmed?

A

MRI

101
Q

What are the 3 symptoms that may present via somatisation?

A

Abdo pain
Reucrrent headaches
Limb pain

102
Q

What is Apley’s rule?

A

The further the pain is from the umbilicus, the more likely the pain is of an organic nature

103
Q

What is the 1st line management for somatisation?

A

Promote communication between family + children
Pain-coping skills ie relaxation techniques for headaches

104
Q

What should be done if 1st line treatments for somatisation fail?

A

Referral to CAMHS

105
Q

Which type of developmental delay has the best prognosis?

A

Isolated delay (global has a/w syndromes that have poorer prognosis)

106
Q

How does the aetiology of conjunctivitis differ between children and adults?

A

In adults is usually viral or allergic, in children is more likely to be bacterial

107
Q

What is the most likely cause of sticky eyes in a <48 hours neonate?

A

Gonorrhoea

108
Q

What is the most likely cause of sticky eyes in a neonate in first 1-2w?

A

Chlamydia: often co-presents with pneumonia

109
Q

How should neonatal gonorrhoeal infection be investigated and treated?

A

Gram stain + culture
3rd gen cephalosporin (eg ceftriaxone)

110
Q

How should neonatal chlamydia infection be investigated and treated?

A

Immunofluorescent staining
Oral erythromycin

111
Q

Which is more common in children out of hypermetropia and myopia?

A

Hypermetropia

112
Q

What is the cause of Retinopathy of Prematurity?

A

Vascular proliferation leads to retinal detachment –> fibrosis and blindness

113
Q

What is the main RF for retinopathy of prematurity?

A

Uncontrolled use of high concentrations of oxygen

114
Q

What are the 2 key signs of retinopathy of prematurity?

A

Unusual eye movements
White pupils

115
Q

What is the first line treatment of retinopathy of prematurity?

A

Laser photocoagulation

116
Q

What is strabismus?

A

Abnormal alignment of eyes

117
Q

At what age is strabismus diagnosed?

A

1-4 years

118
Q

What are the 2 types of strabismus?

A

Non-paralytic (refractive error in one or more eyes)
Paralytic (squinting eye could be caused by motor nerve paralysis or SOL - 3rd nerve palsy)

119
Q

How should strabismus be managed?

A

1st line = glasses
2nd line = eye patching
3rd line = eye drops
4th line = eye muscle surgery

120
Q

Which burn pattern is typical of NAI?

A

Glove and stocking

121
Q

Which type of long bone fracture is indicative of NAI?

A

Spiral fractures

122
Q

What is the classic triad of features in shaken-baby syndrome?

A
  1. Retinal haemorrhages
  2. CT showing brain swelling/ encephalopathy
  3. CT showing subdural haematoma
123
Q

What needs to be ruled out in suspected NAI?

A

Leukaemia, ITP + haemophilia

124
Q

What is the definition of SIDS?

A

Deaths which remain unexplained after a post-mortem

125
Q

What is the peak age for SIDS?

A

2-4 months

126
Q

Recall 6 important risk factors for SIDS

A

Front-sleeping baby
Prematurity
LBW
Male
Maternal smoking
Microenvironment (pillow, heat)

127
Q

For how long should parents share a room with baby?

A

6 months

128
Q

Which organisation provides support for SIDS?

A

Lullaby Trust

129
Q

For how long should a child be excluded from school if they have scarlet fever?

A

24 hours after antibiotics

130
Q

For how long should a child be excluded from school if they have whooping cough?

A

48 hours after Abx

131
Q

For how long should a child be excluded from school if they have measles?

A

4 days from onset of rash

132
Q

For how long should a child be excluded from school if they have rubella?

A

4 days from onset of rash

133
Q

For how long should a child be excluded from school if they have chickenpox?

A

Until all lesions crusted over

134
Q

For how long should a child be excluded from school if they have impetigo?

A

Until all lesions crusted over

135
Q

For how long should a child be excluded from school if they have mumps?

A

5 days from onset of swollen glands

136
Q

For how long should a child be excluded from school if they have influenza?

A

Until recovered

137
Q

When do fontanelle close by?

A

1 year usually, can be as late as 2

138
Q

What should be given before buccal midazolam in a fitting child?

A

Oxygen

139
Q

What infections are children with DiGeorge syndrome particularly at risk of and why?

A

Candidiasis
No thymus –> no T cells

140
Q

Give 2 causes of evanescent salmon pink rash

A

Listeriosis (neonate)
Juvenile idiopathic arthritis

141
Q

Why shouldn’t metoclopramide be given to children?

A

Can give oculogyric crises

142
Q

Recall 3 conditions that cause rigors in children?

A

Pyelonephritis
Influenza
Malaria

143
Q

How should burns be managed medically?

A

Cover for potential toxic shock syndrome with ceftriaxone and clindamycin

144
Q

How can the % of Total Burn Surface Area be measured OE?

A

Hand is 1% as a rough guide

145
Q

Recall the 5 points of the Fraser guidelines

A
  1. YP understands professional’s advice
  2. YP cannot be persuaded to inform parents
  3. YP is likely to begin/ continue having sex regardless of contraception
  4. Physical/ mental wellbeing likely to suffer from lack of contraception
  5. YP’s BI require them
146
Q

What % of children who have a febrile convulsion will have one again?

A

33-50%