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Paediatrics > 1: Neurology + Development - Down's Syndrome, Cerebral Palsy, Status Epilepticus, Febrile Convlusions, Seizures, Seizure Disorders > Flashcards

1: Neurology + Development - Down's Syndrome, Cerebral Palsy, Status Epilepticus, Febrile Convlusions, Seizures, Seizure Disorders Flashcards

1
Q

What is downs syndrome

A

Where there are three copies of chromosome 21 (Trisomy 21)

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2
Q

What is the most common chromosomal aberration

A

Downs Syndrome

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3
Q

What is the main risk factor for Down’s syndrome

A

Maternal Age

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4
Q

What is the risk of DS at maternal age 20y

A

1:1500

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5
Q

What is the risk of DS at maternal age 30y

A

1:800

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6
Q

What is the risk of DS at maternal age 40y

A

1:100

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7
Q

What is the risk of DS at maternal age 45y

A

1:50

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8
Q

What is the risk of DS at maternal age 50y

A

1:6

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9
Q

What is the most common cause of trisomy 21

A

Meiosis Non-Dysfunction

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10
Q

What is non-dysfunction

A

Failure of homologous chromosomes to separate during meiosis

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11
Q

Where does the majority of non-dysfunction occur

A

During meiosis I

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12
Q

What is the risk of recurrence in future pregnancies if DS is due to non-dysjunction

A

<1:100 if <35

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13
Q

What is a balanced robertsonian translocation

A
  • Where the long arm of chromosome 14 combines with long arm of chromosome 21. And, short-arm is deleted
  • Individuals possess only 45 chromosomes but have all the genetic material to give a normal phenotype
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14
Q

What is an unbalanced translocation

A
  • Where individuals inherited one ‘balanced translocated’ chromosome and one normal chromosome
  • This gives three copies of chromosome 46XY ± 21 ± t(21:14)
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15
Q

What % of DS is due to unbalanced translocation

A

5

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16
Q

What is mosaicism

A

Where there are two cell lines present. In DS, will be one normal cell line and one DS cell line. Phenotype depends on balance.

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17
Q

What are two risk factors for DS

A
  • Maternal age

- First-degree relative with DS

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18
Q

How will a foetus with DS present on 10-13+6W scan

A

Raised nuchal translucency

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19
Q

What are the 4 heart structural abnormalities seen in DS and their frequency

A
  • AVSD (40%)
  • VSD (30%)
  • TOF (5%)
  • Patent ductus arteriosus (5%)
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20
Q

What are the 2 GI tract abnormalities seen on anomaly scan in foetus with DS

A
  • Hyperechogenic bowel

- Duodenal atresia

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21
Q

What are the craniofacial dysmorphic features of a child with DS

A

Eyes:
Upward slanting
Large epicanthic folds
Brushfield spots

Nose:
Broad nose

Ears: Small round low-set ears

Mouth: small oral cavity, high arched palate

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22
Q

What are brushfield spots

A

aggregation of connective tissue in the peripheral iris

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23
Q

In the extremities, what are 5 features of DS

A
  • Single palmar crease
  • Sandal gap
  • Increase risk inguinal hernia
  • Increase risk umbilical hernia
  • Atlanto-axial instability
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24
Q

What is the sandal gap

A

Increased gap between big toe and second toe

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25
Q

What are two GI tract features seen in neonates with DS

A

Hirschsprungs

Imperforate anus

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26
Q

What is the GU features seen in neonates with DS

A

Cryptochidism

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27
Q

What are the aesthetic features of an older child with DS

A

Short stature

Obese

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28
Q

What are cognitive features of a child with DS

A

Lower IQ

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29
Q

What are the haematological symptoms of a child with DS

A

ALL

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30
Q

What are the endocrine features in DS

A

Hypothyroidism

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31
Q

What are the GU features in an adult with DS

A

Subfertility in females

Infertility in males

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32
Q

What are the respiratory features of someone with DS

A
  • Recurrent infections and hearing impairment: glue ear

- OSA

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33
Q

What are the long-term neurological symptoms of someone with DS

A

Increased risk of pre-mature Alzheimer’s disease

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34
Q

What are the neonatal developmental features of a baby with DS

A
  • Hypotonia

- Developmental delay

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35
Q

What are the two main types of screening test used for DS

A
  1. Combined Test

2. Quadruple test

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36
Q

When is the combined test performed

A

10-13W+6

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37
Q

When is the quadruple test performed

A

15-20W

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38
Q

What are the four components of the combined test

A
  1. PAPP-A
  2. Nuchal Translucency
  3. serum bHCG
  4. Maternal Age
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39
Q

What do low levels of PAPP-A reflect

A

Poor Placentation

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40
Q

What is a low level of PAPP-A

A

<0.4

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41
Q

If a monochorionic multiple pregnancy how is the risk of DS calculated

A

Per pregnancy

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42
Q

If a dichorionic multiple pregnancy how is the risk of DS calculate

A

Per foetus

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43
Q

Explain what will happen at the end of screening

A

Mum is given a score of ‘high risk >1:150’ or low risk ‘<1:150’.

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44
Q

What are the two non-invasive pre-natal testing methods for DS

A
  • Amniocentesis

- Chorionic Villus Sampling

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45
Q

What is SAFE

A

Checking karyotype of foetal blood cells in maternal blood

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46
Q

If SAFE test is positive what does this mean

A

It is still a screening test and would indicate need for diagnostic test

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47
Q

Who is the IONA test offered to for free

A

If high-risk of DS. More than 1:150.

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48
Q

If IONA test is positive what does this mean

A

It is still a screening test and would indicate need for diagnostic test

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49
Q

What are the two methods of diagnostic testing for DS

A
  1. Chorionic Villus Sampling

2. Amniocentesis

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50
Q

What gestation can chorionic villus sampling be performed

A

First trimester.

10-13W

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51
Q

Explain the procedure of CVS

A

Needle placed trans-abdominally to take trans-placental sample

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52
Q

What is performed on sample from CVS

A
  • Karyotype (2d)

- FISH (3W)

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53
Q

What is the advantage of CVS

A

Within termination limits

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54
Q

What is the disadvantage of CVS

A
  • 2%. miscarriage risk
  • Infection risk
  • Placental mosaicism
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55
Q

When can amniocentesis be performed

A

Second trimster 15-20W

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56
Q

Explain the procedure for amniocentesis

A

Needle placed through abdomen to take sample from baby.

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57
Q

What is performed on amniocentesis

A
  • Karyotype (3W)

- FISH and PCR (3d)

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58
Q

What is the advantage of amniocentesis

A

Miscarriage rate 1% - lower than CVS

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59
Q

What is the disadvantage of amniocentesis

A

Miscarriage rate (1%)

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60
Q

What 4 post-natal tests re recommended for DS baby

A
  1. Karyotype
  2. ECHO
  3. Hip US
  4. Audiology
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61
Q

What is the average prognosis of DS

A

55y

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62
Q

What do the majority of people with DS develop

A

early-onset Alzhiemer’s disease

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63
Q

What is the aim of treatment for DS

A

Supportive. No curative treatment.

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64
Q

What should be offered to DS for management

A
  • Paediatrician follow-up

- Physio to improve tone

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65
Q

What follow-up is recommended in DS patients

A
  • Annual TFTs

- Audiology and Ophthalmology every 1-2Y

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66
Q

What is cerebral palsy

A

Chronic neurodevelopment disorder that presents with abnormalities in movement and posture that start early and continue through life

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67
Q

How can the aetiology of cerebral palsy be divided

A

Cerebral palsy is caused by repeated brain insult prior to 2-years. Its aetiology can be divided into antenatal, perinatal and postnatal.

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68
Q

What percentage of cerebral palsy is antenatal in origin

A

80%

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69
Q

What are three causes of antenatal cerebral palsy

A
  1. TORCH Infections
  2. Cerebral malformation
  3. Placental Insufficiency
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70
Q

What are the TORCH infections

A 
Toxoplasmosis 
'Other'
Rubella 
CMV
Herpes
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71
Q

What percentage of cerebral palsy is peri-natal in origin

A

10%

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72
Q

What causes peri-natal cerebral palsy

A

Birth asphyxia - resulting in hypoxic-ischaemic injury and encephalopathy

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73
Q

What percentage of cerebral palsy is post-natal in origin

A

10%

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74
Q

What causes post-natal cerebral palsy

A
  • IV haemorrhage
  • Trauma
  • Kernicterus
  • Meningitis
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75
Q

What are two risk factors for cerebral palsy

A

Low birth weight

Premature!

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76
Q

In which population is the incidence of cerebral palsy higher

A

Those born 22-27W

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77
Q

How is cerebral palsy categorised

A

It is categorised by the type of movements present into two main categories:

  1. Spastic CP
  2. Non-Spastic CP
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78
Q

What are the two categories of cerebral palsy

A
  1. Spastic Cerebral Palsy

2. Non-Spastic Cerebral Palsy

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79
Q

What percentage of cerebral palsy is spastic

A

70%

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80
Q

What percentage of cerebral palsy is non-spastic

A

30%

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81
Q

What are the two-types of non-spastic cerebral palsy

A
  1. Ataxic

2. Dyskinetic

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82
Q

What is dyskinetic non-spastic cerebral palsy

A

Presence of involuntary movements. Including choreoarthroid movements and dystonia

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83
Q

How does ataxic cerebral palsy present

A

Intention tremor
Poor balance
Poor co-ordination

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84
Q

How will individuals with spastic cerebral palsy present in general

A
  • Hypotonic at birth until 6-12 months when they become spastic
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85
Q

what reflexes may remain in spastic cerebral palsy

A

Primitive reflexes remain

Upgoing Plantar Reflex

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86
Q

what type of gait may be present in cerebral palsy

A
  • Tip toe walking

- Scissoring gait due to weakness in abductors

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87
Q

What are three complications of spastic cerebral palsy

A
  1. Hip dislocation
  2. Scoliosis
  3. Hearing and visual impairment
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88
Q

What are the three types of spastic cerebral palsy

A
  1. Hemiplegic
  2. Quadriplegic
  3. Diplegic
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89
Q

What is hemiplegic CP

A

Weakness in arm and leg on the same side

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90
Q

Are the arms of legs affected more in hemiplegic CP

A

Arms

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91
Q

How will the arms appear in hemiplegic spastic CP

A
  • Hands in fists
  • Pronated
  • Hand preference before 12m
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92
Q

What is a classic indictor of hemiplegic CP and why

A

Hand preference before 12m. In hemiplegic CP, one side will be weak causing the infant to use the other side giving hand preference

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93
Q

How will legs present in hemiplegic CP

A

Tip toe walk

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94
Q

What is diplegic CP

A

When all four limbs are affected

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95
Q

Are legs or arms affected more in diplegic CP

A

Legs

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96
Q

What is quadriplegic CP

A

When arms and legs are affected

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97
Q

How will quadriplegic CP present early-on

A

Poor head control!!

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98
Q

What conditions is quadriplegic CP associated with

A
  • Microcephaly
  • Intellectual impairment
  • Epilepsy
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99
Q

Explain abnormal involuntary movements in non-spastic CP

A

Worsen with stress and improve with sleep

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100
Q

What percentage of CP is dyskinetic

A

10%

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101
Q

How will tone present in dyskinetic CP

A

fluctuating tone leading to abnormal movements

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102
Q

What are three types of movement in dyskinetic CP

A
  • Dystonia
  • Chorea
  • Athetosis
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103
Q

What is chorea

A

Non-Repetitive, sudden movements

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104
Q

What is dystonia

A

Contraction of agonist and antagonist muscles of the proximal limb

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105
Q

What is athetosis

A

Contraction of agonist and antagonist muscles of the distal limb

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106
Q

What percentage of CP is ataxic hypotonic

A

10%

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107
Q

How does ataxic hypotonic CP present

A 
Ataxia
Intention tremor 
Hypotonia 
Poor balance 
Un co-ordination
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108
Q

What are 7 problems associated with CP

A
  • Epilepsy
  • Intellectual disability
  • Developmental motor delay
  • ADHD
  • Squint
  • Feeding difficultly
  • Joint contractures
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109
Q

Which type of CP is more associated with intellectual disability

110
Q

Why will children with CP struggle with feeding

A

Struggle with oro-motor co-ordination leading to gagging

111
Q

What causes spastic CP

A

Damage to pyramidal tract. Damages GABAergic neurons, leading to hypertonia.

112
Q

What causes dyskinetic CP

A

Damage to basal ganglia which controls initiation of movement. Disinhibiting movement

113
Q

What causes ataxic CP

A

Damage to cerebellum - resulting in ataxia.

114
Q

What is the term cerebral palsy used to describe

A

CP is used to describe the disability not the cause - therefore need to find underlying neurological insult.

115
Q

What causes cerebral palsy

A

repetitive neurological insult before 2-years. Therefore investigation focuses on finding sources of insult

116
Q

What is first-step in investigating CP

A

Detailed history to try and identify episodes of birth asphyxia and HIE

117
Q

What imaging is used to investigate cause of CP in neonates

A

Cranial US

118
Q

What can cranial US identify

A

HIE and structural abnormalities

119
Q

What imaging is used in older children to identify CP

120
Q

Explain aims of treatment in CP

A

Treatment is not curative - but aims to relieve symptoms

121
Q

What is used to manage posture and movement in CP

A

Posture + Movement:

  • Exercises
  • Orthoses
  • Wheelchair
  • Management of spasticity
122
Q

What can be used to treat spasticity in CP

A
  • Botox of gastrocnemius = prevents toe walking
  • Oral diazepam
  • Oral/Intrathecal Baclofen
123
Q

What is used to manage communication in CP

A

SALT referral

124
Q

What is used to manage congnition in CP

A

Tailored Educational Program

125
Q

Why is CP classified as non-progressive

A

As it does not get worse

126
Q

Define status epileptics

A
  • Seizure persisting beyond 30-minutes

- Or, recurrent seizures with no return to baseline between

127
Q

What are 5 causes of status epileptics

A
  1. SOL
  2. Epilepsy
  3. Metabolite disturbances
  4. CNS Infection
  5. Febrile convulsion
  6. Head Injury
128
Q

What is a risk factor for status epilepticus

A

Seizures persisting beyond 5 minutes

129
Q

Why is status epileptics protocol started after 5-minutes

A

Seizures beyond 5 minutes have a significant risk of transforming into status epilepticus. Therefore risk is avoided by introducing treatment early.

130
Q

How can status epilepticus be divided

A
  • Convulsive

- Non-Convulsive

131
Q

How is non-convulsive status epilepticus identified

132
Q

What investigations are performed for status epilepticus

A
  • Blood Glucose
  • U+Es
  • CRP/ESR
  • FBC
  • Toxicology screen
  • CT
133
Q

When should a CT scan be considered

A

Focal neurological signs or symptoms. Head trauma

134
Q

How should status epilepticus be managed in general

A

A-E approach (Emergency)

135
Q

How is status epilepticus managed in 0-5m

A
  • ACTION - for seizure management.
  • Call For Help
  • IV Access
  • 100% Oxygen
  • Monitor glucose
136
Q

How is status epilepticus managed in 5-15m

A

IV Lorazepam OR Rectal Diazepam

137
Q

When is IV lorazepam given

A

If IV access obtained

138
Q

What should be done at 15-20m if individual is still in status

A

Repeat dose of IV lorazepam or rectal diazepam (up to maximum of 4mg for lorazepam. Or, 10 for diazepam)

139
Q

What is maximum dose of IV lorazepam

140
Q

What is maximum dose of rectal diazepam

141
Q

What medication is given 15-35 minutes if still in status

A

IV phenytoin

142
Q

If individual is normally on phenytoin, but still not responding to lorazepam or diazepam, what should be given as an alternative

A

IV Phenobarbitone

143
Q

If status epilepticus has not responded to lorazepam or second-line what is it called

A

Refractory

144
Q

What should be done for refractory seizures

A

Contact ITU for admission

  1. Intubated
  2. Thiopentone given
  3. IV midazolam

EEG monitoring

145
Q

What is thiopentone

A

Muscle relaxant

146
Q

What is IV midazolam used for

A

Terminate seizure activity

147
Q

Explain medications given in order for status epileptics

A
  1. IV lorazepam or Rectal diazepam
  2. Repeat dose IV lorazepam or rectal diazepam
  3. IV phenytoin (or If regularly on phenytoin, IV phenobarbitone)
148
Q

What is the risk following status epilepticus in a child

A

High risk of entering respiratory arrest following status

149
Q

What should be done to prevent children going into respiratory arrest following status epilepticus

A
  • Oxygen
  • Observe SpO2
  • Recovery position
150
Q

When may a LP be performed if required following a status

A

Wait for GCS > 9

151
Q

What is a febrile convulsion

A

Single generalised tonic-clonic seizure lasting less than 15-minutes associated with a temperature >38.

152
Q

In which age group do febrile convulsions occur

A

6m -5 years.

153
Q

What is peak age for febrile convulsions

A

12-18 months

154
Q

What are 5 risk factors for febrile convulsions

A 
1.  HHV6 Infection 
(Roseola Infantum) 
2. Influenza virus 
3. MMR vaccine 
4. FH 
5. Rapid rise in temperature
155
Q

What are the three categories of febrile convulsions

A
  • Simple Febrile Convulsion
  • Complex Febrile Convulsion
  • Convulsive Status Epilepticus
156
Q

What are the 4 criteria for a simple febrile convulsion

A
  • < 15m
  • Generalised Tonic-Clonic
  • Quick return to baseline
  • No recurrence in 24h
157
Q

What are three criteria for a complex febrile seizure

A
  • Focal
  • <15 minutes
  • May recur in 24h
158
Q

What is febrile status epileptics

A

Seizure persisting longer than 30-minutes

159
Q

What are the three names of theories for aetiology of febrile convulsions

A
  1. Fever rises body temperature
  2. Fever causes hyperventilation
  3. Cytokine theory
160
Q

Explain fever rises core body T theory

A

Fever increases core body T - increasing neuronal excitability

161
Q

Explain fever causes hyperventilation theory

A

Fever increases RR, this causes respiratory alkalosis. Respiratory alkalosis leads to increased excitability of neurons

162
Q

Explain fever, cytokine theory

A

Cytokines act on NMDA receptors increase excitability

163
Q

Explain investigations for febrile convulsions

A

If simple, febrile convulsions may not need to be investigated

164
Q

What investigations may be performed in febrile convulsions

A 
FBC
CRP
Blood Glucose 
U+E 
Urinalysis 
Urine Culture 
LP
EEG
165
Q

When should children with febrile convulsions be admitted to paediatrics

A
  1. First presentation of febrile convulsion
  2. Complex febrile convulsions
  3. Under 18m with febrile convulsion
166
Q

What is the three criteria for complex febrile convulsions

A
  1. <15m
  2. Focal neurological signs and symptoms
  3. May recurrent in 24h
167
Q

What is first-line management for febrile convulsions

A

ACTION management

168
Q

What is the ACTION approach

A 
Assess situation
Call for help 
Time 
Identity 
Over - once finished put in recovery position
Never restrain their head
169
Q

If a simple febrile convulsion lasting more than 5 minutes what should be done

A

Benzodiezapine rescues medication

170
Q

What is given as benzodiezapine rescue medication

A

Buccal midazolam

Or, rectal diazepam

171
Q

What dose of buccal midazolam is given if under 11 months

172
Q

What dose of buccal midazolam is given if 1-4 years

173
Q

What dose of buccal midazolam is given if 5-9 years

174
Q

What is an alternative to buccal midazolam for febrile convulsions

A

Rectal diazepam

175
Q

Aside from midazolam/diazepam what may be given in febrile convulsions

A

Anti-Paretics

176
Q

What are criteria for admitting children with febrile convulsions

A
  1. <18m
  2. Complex febrile convulsion
  3. First presentation
177
Q

Discuss long-term management of febrile convulsions

A

At start of fever parent’s may given paracetamol of wet flannel to cool the child.

Teach parents how to use benzodiezapine rescue pack

178
Q

What have anti-pyretics NOT been shown to do

A

reduce recurrence of febrile convulsions

179
Q

What is the risk of another seizure in febrile convulsions

180
Q

Explain link between febrile convulsions and epilepsy

A

There are three risk factors for epilepsy:

  1. FH
  2. Complex febrile convulsion
  3. Background neurodevelopment issue
  • If not factors = 2.5% risk
  • Multiple factors = 50% risk
181
Q

Define a seizure

A

Irregular electrical activity due to hyper-activity of neurons

182
Q

What defines a neonate

183
Q

What are four causes of seizure in neonate

A
  1. Peri-natal Infection
  2. Peri-natal asphyxia
  3. Metabolite abnormalities
  4. Congenital Syndromes
184
Q

What is the most common trigger of seizures in infants

A

Febrile convulsions

185
Q

What age are febrile convulsions most common

186
Q

What are 5 causes of seizures in infants

A
  1. Malformaiton
  2. TBI
  3. Metabolic
  4. Fever
  5. Infection
187
Q

What are three causes of seizures in adolescents

A
  1. TBI
  2. Illicit substance
  3. Encephalitis
188
Q

What are 4 causes of seizure in young adults

A
  1. Alcohol withdrawal
  2. Illicit substances
  3. TBI
  4. Brain Tumours
189
Q

What are the two main broad categories of seizures

A
  1. Generalised

2. Focal

190
Q

What is a generalised seizure

A

Electrical seizure activity originates in bilateral networks

191
Q

What is used to classify seizure type

A

International League Against Epilepsy

192
Q

In ILAE classification, how are seizures first classified

A
  1. Generalised

2. Focal

193
Q

If a focal seizure how are they first classified

A
  1. Awareness Impaired

2. Awareness Un-Impaired

194
Q

How are focal seizures classified following awareness impaired/unimpaired

A

Motor

Non-Motor

195
Q

What can focal seizures become

A

Focal Seizures can progress to bilateral tonic-clonic seizures

196
Q

How are generalised seizures classified

A
  1. Motor = tonic clonic

2. Non-Motor = absence

197
Q

What is a generalised motor seizure called

A

Tonic Clonic Seizure

198
Q

What is a generalised non-motor seizure called

A

Absence Seizure

199
Q

How are ‘other’ seizures classified

A

Into motor and non-motor

200
Q

What are four types of generalised seizure

A
  1. Tonic-Clonic
  2. Myoclonic
  3. Atonic
  4. Absence
201
Q

Explain clinical presentation of Tonic Clonic Seizures

A
  • Pro-drome
  • Individual first goes stiff and then limbs move symmetrically, bilaterally giving a rhythmic appearance.
  • Individual may have tongue biting and incontinence during the seizure
202
Q

What age group are myoclonic seizures more common

203
Q

How do myoclonic seizures present

A
  • LOC

- individual is thrown to the ground where they start violently jerking

204
Q

What is best medication to manage myoclonic seizures

A

Sodium Valproate

205
Q

How does atonic seizures present

A

Sudden loss of tone - causes person to fall to the ground

206
Q

In which age-group is absence epilepsy more common

207
Q

What is absence epilepsy

A

Brief loss of consciousness for 5-20 seconds. Eyes may roll up. Individual is unaware

208
Q

What are focal seizures

A

More commonly due to focal structural damage - occur on one hemisphere. Anatomical location indicates seizure symptoms.

209
Q

What are the two types of focal seizure

A
  1. Consciousness unimpaired = simple partial seizure

2. Consciousness impaired = complex partial seizure

210
Q

If a simple partial seizure where may it have arisen

A
  • Frontal Lobe
  • Parietal Lobe
  • Occipital Lobe
211
Q

How will a frontal focal seizure present

A
  • Jacksonian March: Individual starts with twitching of the hand progresses to twitching of the face
  • Head/Facial movements
  • Todd’s paralysis
212
Q

How will occipital focal seizure present

A

Flashers and Floaters in Vision

213
Q

How will focal seizure of the parietal lobe present

A

Parasthesia

214
Q

Where do complex focal seizures originate

A

Temporal Lobe

215
Q

What % of focal seizures occur in temporal lobe

216
Q

What are 6 features of temporal lobe seizures

A
  • Aura
  • Deja Vu
  • Jamais Vu
  • Automatisms (Lip Smacking)
  • Behavioural arrest
  • Impaired consciousness
217
Q

Child presents with lip smacking, where has the seizure likely originated

A

Temporal Lobe

218
Q

What age does West Syndrome Occur

A

4-6 months

219
Q

What is West Syndrome also referred to as

A

Infantile spasms

220
Q

What gender is West Syndrome more common

221
Q

How does West Syndrome present

A

Head nodding followed by arm jerks for 3-30s

222
Q

What is head nodding in West syndrome called

A

Salaam attack

223
Q

What is a reflex-anoxic attack

A

Paraoxysmal self-limiting asystole triggered by pain or fear

224
Q

How will a child present in reflex-anoxic attacks (6 Features)

A
  • Pale
  • Hypotonic
  • Rigid
  • Upward eye deviation
  • Clonic
  • Urinary incontinence
225
Q

What investigation should be ordered if a reflex-anoxic attack is suspected

A

Vagal excitation tests during EEG and ECG monitoring

226
Q

What age does panayiotopolous syndrome occur

227
Q

What is panayiotopoulos syndrome

A

Benign Focal Seizure Disorder affecting ~5years. Occurs at night, Autonomic symptoms predominate, with eye deviation and vomiting. Seizures can last 30 minutes. Self-resolves within 2 years

228
Q

Explain age-dependent epileptic encephalopathy

A

Starts with Otahara syndrome (Tonic spasms) Progresses to West Syndrome then Lenox-Gustalt.

These are age-specific syndrome reactions to a non-specific exogenous source

229
Q

How does Rolandic epilepsy present

A

Parasthesia of unilateral face worse on waking

230
Q

What is Rolandic epilepsy also known as

A

Benign epilepsy with centrotemporal spikes

231
Q

What is associated with west syndrome

A

Low IQ (70%)

232
Q

What is the management of west syndrome

A
  1. Prednisolone

2. Vigabatrin

233
Q

What are bedside investigations following seizure

A
  • Neurological Exam

- Cardiac Exam

234
Q

What are blood investigations for seizure

A
  • FBC (Infection)
  • U+E (Metabolic)
  • Serum Calcium
  • Blood Glucose (Hypoglycaemia)
  • Blood Gas ( Hypoxic)

Blood and Urine MC+S

235
Q

What imaging may be ordered following paediatric seizure

A

MRI
ECG
EEG

236
Q

When are three indications for MRI following a seizure

A
  1. Focal Seizure
  2. Children who develop epilepsy before age 2
  3. Seizures continue despite first-line treatment
237
Q

When is an ECG performed following a seizure

A

All seizures

238
Q

When is an EEG performed following a seizure

A

To SUPPORT diagnosis of epilepsy following second-seizure

239
Q

What conservative management is offered for seizures

A

Advice:

  • Avoid triggers
  • Avoid dangerous situations such as bathing or swimming alone
240
Q

What is used to manage epilepsy

A

anti-epileptic drugs

241
Q

When should AEDs be used

A

following a second-seizure

242
Q

What population should NOT be offered sodium valproate and why

A

young girls of reproductive age (from 8 years) due to high teratogenicity

243
Q

What is first-line for focal seizures

A

lamotrigine or carbamezapine

244
Q

How can you remember first-line for focal seizures

A

foCaL = has a C for carbamezapine and L for lamotrigine

245
Q

What is first line for generalised TC seizures

A

Sodium valproate.

246
Q

If a young female with generalised tonic-clonic seizures what medication should be given

A

Lamotrigine

247
Q

How are absence seizures managed

A 
Boys = Sodium Valproate 
Girls = Ethosuximide
248
Q

How are myoclonic seizures managed

A 
Boys = Sodum Valproate 
Girls = Levetiracetam
249
Q

What are the two types of generalised epilepsy syndrome

A

Idiopathic epilepsy syndrome

Symptomatic epilepsy syndrome

250
Q

Explain idiopathic epilepsy syndromes

A

Most common (25%). Children are generally healthy

251
Q

Explain symptomatic/cryptogenic epilepsy syndromes

A

structural or metabolic abnormality. Cryptogenic means seizures of unknown origin. Generally this group respond poorly to anti-epileptics. Associated with developmental delay, cognitive impairment or motor impairment

252
Q

What age does childhood absence epilepsy syndrome occur

253
Q

Which gender is childhood absence epilepsy syndrome more common

254
Q

How will children appear in childhood absence epilepsy syndrome

A

Absence seizures where children may have lip smacking, eye fluttering

255
Q

How will EEG appear in childhood absence epilepsy syndrome

A

3Hz spikes

256
Q

What is used to treat childhood absence epilepsy syndrome

A

Ethosuximide

257
Q

What age is juvenile myoclonic epilepsy syndrome more common

258
Q

What is juvenile myoclonic epilepsy syndrome also referred to as

A

Janz Syndrome

259
Q

What can cause west syndrome

A
  • Hypoxic- Ischaemic Injury

- Perinatal infection

260
Q

How will West Syndrome present

A

Sudden spasms involving flexion of the head (Salaam attack) and extension of the arms

261
Q

What is used to manage West Syndrome

A
  • Prednisolone

- Vigabatrin

262
Q

What is west syndrome associated with

A

Neurodevelopment delay

263
Q

What age does Lennox Gastaut Syndrome occur

264
Q

Which gender is Lennox-Gastaut more common in

265
Q

What do 50% of children with Lennox - Gastaut have

A

West Syndrome

266
Q

How will Lennox-Gastaut present clinically

A
  • Frequent episodes of status epilepticus

- Atypical absence seizures

267
Q

How will Lennox-Gastaut present on EEG

A

Slow-spikes

268
Q

What is used to manage Lennox-Gastaut

A

Sodium Valproate

Ketogenic diet

269
Q

What is Lennox Gastaut associated with

A

Neurodevelopment delay

270
Q

What gene is mutated in Dravet syndrome

Paediatrics (61 decks)

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