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Child Neurology > Paediatric Epilepsy > Flashcards

Paediatric Epilepsy Flashcards

1
Q

what is the most common neurological condition in children

A

epilepsy

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2
Q

prevalence of epilepsy

A

1 in 200

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3
Q

what percentage of children outgrow epilepsy by adulthood

A

50-60%

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4
Q

causes of epilepsy

A
  • Genetic disorders.
  • Structural lesions.
  • Previous brain injury.
  • Epilepsy syndromes (genetically influenced).
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5
Q

definition of epilepsy

A

tendency to recurrent unprovoked seizures

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6
Q

what is a seizure

A

physical manifestation of abnormal electrical brain activity

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7
Q

types of seizures

A
  • Focal (affecting one part of the brain).
  • Generalised (affecting the whole brain).
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8
Q

causes of seizures

A
  • Febrile seizures (high temperature in children).
  • Hypoglycaemia (low blood sugar).
  • Trauma (head).
  • Symptomatic causes.
  • Epileptic.
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9
Q

what to do if someone is having a tonic-clonic seizure

A
  • Time the siezure
    • Most stop within 5 mins.
    • Any longer than 30 mins is concerning in relation to brain damage.
  • Check the person for epilepsy ID.
  • Remove any harmful objects.
  • Cushion the head.
  • Don’t put anything in their mouth.
  • Son’t restrain the person unless they’re in danger.
  • Place them into the recovery position when the seizure ends.
  • Stay with the person until the seizure ends and offer assistance.
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10
Q

what to do if someone is having a focal impaired awareness seizure

A
  • Time the seizure.
  • Don’t restrain or grab them.
  • Guide them gently from danger.
  • Calmly reassure.
  • Stay until seizure ends.
  • Explain what happened.
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11
Q

when to call an ambulance for someone having a seizure

A
  • The seizure is longer than 5 minutes.
  • One seizure follows another without stopping.
  • It’s the person’s first seizure.
  • The person is injured.
  • You are in any doubt.
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12
Q

what testing occurs for a child with their first seizure (primary care/ED)

A
  • History → consider differential diagnosis.
  • Examination with vitals, and lying and standing BP.
  • ECG.
  • Blood glucose and electrolytes.
  • Consider neuroimaging if appropriate.
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12
Q

what happens after first seizure

A
  • EEG as outpatient
  • General paediatrics follow up.
  • Consider rescue plan if longer than 5 mins (e.g. medication).
  • Give advice regarding how to recognise another seizure, first aid, reducing risk of second seizure, how to make contact if further seizure occurs, video further events.
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13
Q

what happens if there is a subsequent seizure of if there is an abnormal EEG

A
  • Classification of seizure type/ epilepsy type/ epilepsy syndrome.
  • Decision regarding commencement of anti-siezure medication.
  • General paediatric follow up if appropriate.
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14
Q

when to refer a child with a seizure to paediatric neurology

A
  • Drug resistance.
  • Diagnostic doubt.
  • Specialised treatments are required.
  • Aged under 3 years.
  • Aged under 4 years with with myoclonic seizures.
  • EEG positive absence seizures < 4 years.
  • infantile spasms/ epileptic encephalopathies.
  • Unilateral structural lesion.
  • Metabolic condition.
  • Deterioration in behaviour, speech or learning or developmental regression.
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15
Q

what does EEG stand for

A

electroencephalogram

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16
Q

EEG testing

A
  • Tests electrical activity of the brain.
  • Non-invasive, painless.
  • Process:
    • Electrodes placed on scalp.
    • +/- sleep deprivation.
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17
Q

activation procedures for EEGs

A
  • Hyperventilation.
  • Photic stimulation.
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18
Q

EEG duration

A

1 hour

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19
Q
A

normal/organised

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20
Q
A

slow and disorganised

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21
Q
A

discontinuous or burst supression

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22
Q
A

supressed and featureless

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23
Q

MRI testing

A
  • Used to assess structural abnormalities in the brain
  • I.e. where is the activity coming from.
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24
for what type of seizure are MRIs particularly relevant for
focal
25
possible causes of structural abnormalities revealed by MRI testing
- Under development of brain matter. - Tumour. - Small stroke in the past.
26
types of genetic testing in epilepsy
- karyotype - maicroarray - single gene testing - gene panel - whole exome/genome sequencing
27
karyotype
examines all 23 pairs of chromosomes
28
microarray
examines specific chromosomes for small sections which are deleted or duplicated
29
singe gene testing
examines a single gene in detail
30
gene panel
examines a number of genes in detail (e.g. all genes known to cause epilepsy)
31
whole exome/genome sequencing
examines all genes in detail
32
clinical freatures of seizures
- Variety of transitory abnormal events including: - Altered consciousness - Motor, sensory, autonomic, or cognitive events
33
tonic-clonic seizure features
- Often starts with a cry - Sudden fall, bilateral stiffening, jerking movements - Possible tongue biting or incontinence
34
absence seizure features
- Sudden onset of staring - Lasts **5-10 seconds** - May involve **eye blinking, lip smacking**
35
myoclonic seizure features
Sudden brief muscle contractions
36
tonic seizure features
Sudden stiffening of the body
37
atonic seizure features
Sudden loss of muscle tone ("drop attack")
38
epilepsy syndromes
- Defined by **distinct clinical and EEG patterns** - May have **genetic, structural, metabolic, immune, or infectious causes**
39
what is SeLECTS
Self-limited epilepsy with centro-temporal spikes
40
brain area affected by SeLECTS
central/temporal lobes
41
age of onset for SeLECTS
4-10 years
42
is development and cognition typical in children with SeLECTS
yes
43
SeLECTS features
- **Nocturnal focal clonic or tonic seizures**: - Involves activity of throat/tongue and lower face. - May progress to **bilateral tonic-clonic seizures**
44
EEG features of SeLECTS
- Normal background - High amplitude centro-temporal sharp and slow wave complexes (activated in sleep)
45
prognosis of SeLECTS
Seizures are infrequent and resolve by puberty
46
what does SeLEAS stand for
Self-limited epilepsy with autonomic seizures
47
what part of the brain is affected by SeLEAS
occipital lobe
48
SeLEAS features
- Focal autonomic seizures (e.g., nausea, vomiting, pallor) - Often prolonged
49
SeLEAS prognosis
- 25% have only a single seizure - Remission within a few years is typical
50
examples of generalised epilepsy syndromes
- **CAE**: Childhood Absence Epilepsy - **JAE**: Juvenile Absence Epilepsy - **JME**: Juvenile Myoclonic Epilepsy - **GTCA**: Epilepsy with Generalised Tonic-Clonic Seizures Alone
51
how are generalied epilepsy syndromes categorised
by most commonly occuring type of seizures, a child with CAE could also have a tonic-clonic seizure
52
age of onset for CAE
4-10 years
53
is development typical in children with CAE
yes
54
CAE features
- **Frequent episodes at diagnosis** (>40 per day) - **Seizure duration**: 3-20 seconds
55
EEG findings of CAE
3Hz spike-wave
56
triggers for CAE
hyperventilation
57
treatment for CAE
ethosuximide
58
age of onset for juvenline myoclonic epilepsy
10-24 years
59
what do people with juvenile myoclonic epilepsy have higher rates of
ADHD
60
types of seizures present in juvenile myoclonic epilepsy
- Myoclonic seizures on waking - Also absence and GTCS
61
triggers of juvenile myoclonic epilepsy
Sleep deprivation, photic stimulation
62
prognosis of juvenile myoclonic epilepsy
Generally worse and can be a lifelong diagnosis.
63
what is developmental epileptic encephalopathy
"Epileptic activity itself contributes to severe cognitive and behavioural impairments"
64
causes of developmental epileptic encephalopathy
- Monogenic - Structural - Metabolic - Secondary to insult (e.g., prematurity)
65
example of a developmental epileptic encephalopathy
lennox gastaut syndrome
66
onset of infantile spasms
1-24 months
67
features of infantile spasms
- Sudden **brief neck flexion, arm extension** - Often in clusters upon waking
68
conditions associated with infantile spasm
T21, tuberous sclerosis
69
EEG findings of infantile spasms
Hypsarrhythmia
70
treatment of infantile spasms
Steroids, Vigabatrin
71
prognosis of infantile spasms
associated with poor development, developmental regression and poor outcome - children with T21 tend to have better prognosis.
72
is landau-kleffner syndrome common or rare
rare
73
at what age does landau-kleffner syndrome occur
school-age
74
features of landau kleffner syndrome
- Acquired aphasia with seizures - Verbal auditory agnosia - Epileptiform activity during sleep - Seizures are **infrequent,** language issues are **more significant.**
75
two mechanisms of action for anti-seizure medications
decrease excitation or enhance inhibition of neurons
76
when to start anti-seizure medicaton
- After **two unprovoked seizures** - After **one if recurrence is highly likely** - **Consideration for infrequent seizures**, especially in: - SeLECTS - SeLEAS
77
when to wean from anti-seizure medication
Generally after two years of seizure freedom
78
how are anti-seizure medications taken
generally taken 2x a day, can be liquid or tablets.
79
for whom should emergency seizure medications be considered
those with convulsive seizures lasting longer than five mins.
80
example of emergency anti-seizure medication
Buccal midazolam.
81
three examples of non-pharmacological seizure treatments
- Ketogenic Diet - Vagus Nerve Stimulation - Epilepsy Surgery
82
theory behind ketogenic diet for those with epilepsy
to give the brain a different source of fuel
83
aim of vagus nerve stimulation for epilepsy
for QOL improvements not seizure freedom
84
co-morbidities in epilepst
- mental health disorders - behavioural issues - intellectual disabilities - sleep disturbances - bone health concerns
85
examples of mental health disorders co-morbid with epilepsy
anxiety and depression
86
examples of behavioural issues co-morbid with epilepsy
ADHD, ASD, aggression
87
what does SUDEP stand for
sudden unexpected death in epilepsy
88
what is SUDEP
Sudden, unexpected, non-traumatic, non-drowning death in epilepsy
89
risk factors for SUDEP
- >3 GTCS per year - Nocturnal seizures - Intellectual disability - Polytherapy - Treatment-resistant epilepsy - Subtherapeutic drug levels
90
examples of epilepsy mimics
- Febrile seizures - Syncope - Functional Neurological Disorder - Tics - Panic attacks - Stereotypies - Migraine - Infantile gratification
91
what age group do febrile seizures occur in
6 months to 6 years
92
what are febrile seizures
- Seizures exclusively occur in the context of a high temperature - Generally short, GTCS, <5 minutes
93
are children who experience febrile seizures developmentally normal
yes
94
what age group does syncope often occur in
teenagers
95
symptoms of syncope
- Feel lightheaded - Loss of vision - Loud ringing in ears - Collapse to the ground - Can have some stiffening with it
96
how is syncope assesed
standing and lying BP
97
staring in ASD considerations
- Very common reason for referral - Rule out absence seizures - Frequency, length of events, clinical course - Most will not tolerate EEG
98
other names for Functional Neurological Disorder
Dissociative seizures / Non Epileptic Attack Disorder / Psychogenic
99
clues to a diagnosis of FND
- eyes often closed - hip thrusting - no injuries - always witnessed - no post ictal phase - asynchronous movements - side to side head movements - ictal crying
100
age range for people affected with FND
from about age 8 to elderly
101
testing in FND
MRIs and EEG won’t reveal seizures
102
cause of FND
Brain functions normally but the body doesn’t interpret signals correctly (interaction between brain and the mind). Influence of biopsychosocial factors
103
treatment for FND
psychology
104
what are tics
- sudden and repetitive movements and/or sounds that people make involuntarily. - Suppressibility - Change over time - Worse if stressed or distracted
105
what age group do tics commonly affect
school-aged children, often starting around early primary school age.
106
what is another name for infantile gratification
infantile masturbation
107
what is infantile gratification
- a self-stimulatory psychological behaviour - Often associated with perspiration, irregular breathing and grunting, and leg stiffness, but no loss of consciousness.
108
is infantile gratification more common in girls or boys
girls
109
at what age does infantile gratification usually occur
2-3 years

Child Neurology (5 decks)

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