Fits faints and funny turns

Question 1

What does good management of fits faints and funny turns depend on?

Answer 1

Are the attacks epileptic or non-epileptic?
Consider differential diagnosis
If they are epileptic -
What kind of seizure(s)?
What type of epilepsy (i.e. what epilepsy syndrome)?
What is the cause of the seizures?

Question 2

Epilepsy vs non-epilepsy

Answer 2

Misdiagnosis of epilepsy in ~25% patients
Causes of misdiagnosis
Inadequate history
Undue reliance on EEG
Diagnosis may be difficult!

Epilepsy is a clinical diagnosis

Question 3

How do we take a history of a child who has had a “seizure” or “fit”?

Answer 3

From eye witness
Entire attack from normality to normality
Context
Onset
Nature of attack
Offset
Recovery
Repeated history
Question the child

Question 4

What are the key differential of a child having a fit or seizure?

Answer 4

Syncope (including breath holding)
Staring episodes
Paroxysmal events in sleep
Behavioural
Psychogenic/dissociative
“Neurological”
Metabolic
Toxic

Question 5

What are the characteristics of an Anoxic seizure - reflex asystolic syncope?

Answer 5

Onset precipitated by noxious stimulus
Pallor
Floppy -> stiff
Jerks
May bite tongue
May be incontinent of urine
Relatively rapid recovery

Question 6

What is syncope?

Answer 6

abrupt cessation of delivery of energy substrates to
cerebral cortex
Reduction in perfusion, oxygenation or both

Question 7

What is an anoxic seizure?

Answer 7

clinical or electroclinical event resulting from
cessation of energy to most metabolically active cortical neurons

Question 8

What are some sleep events that could happen to a child?

Answer 8

Sleep myoclonus
Night terrors
Nightmares
Sleep walking
Hypnagogic/hypnapompic phenomena

Question 9

What are the type of blank spells children can have?

Answer 9

Attentional lapse/”daydreaming”
Behavioural
Psychogenic non-epileptic seizure
Absence
Focal dyscognitive seizure

Question 10

What are some behavioural issues children could have?

Answer 10

Day dreaming
Self gratification
Tics
Stereotyped movements – more commonly(but not exclusively) seen in children with development disorders such as Autism Spectrum Disorder

Question 11

What are some neurological issues children can have?

Answer 11

Migraine
Paroxysmal choreoathetosis
Acute dystonia
Benign paroxysmal vertigo
Benign paroxysmal torticollis of infancy
Narcolepsy/cataplexy
etc

Question 12

What are some psychiatric issues children could have?

Answer 12

Anxiety
Panic attacks
Acute confusional states
Psychogenic/dissociative seizures
Hyperventilation

Question 13

What are the causes of misdiagnosis in children?

Answer 13

Inadequate history
Undue reliance on EEG
Diagnosis may be difficult

Question 14

What should an EEG in children 6-13 years show

Answer 14

131/3726 (3.5%) abnormal
4 3Hz spike/ slow wave
37 polyspike/ slow wave
79 focal spikes
11 multifocal spikes

Question 15

What is epilepsy defined as?

Answer 15

Epilepsy is a disease of the brain defined by any of the following conditions:

At least two unprovoked seizures occurring more than 24 hours apart.
One unprovoked seizure and a probability of further seizures similar to the general recurrence risk after two unprovoked seizures (approximately 75% or more).
At least two seizures in a setting of reflex epilepsy.

Question 16

What is epilepsy remission?

Answer 16

Epilepsy is considered to be no longer present for individuals who had an age-dependent epilepsy syndrome but are now past the applicable age or those who have remained seizure-free for at least 10 years off anti-seizure medicines, provided that there are no known risk factors associated with a high probability (>75%) of future seizures.

Question 17

Epidemiology of epilepsy?

Answer 17

Incidence varies between 1st and 3rd world countries
Recent evidence of reduction in incidence in UK
Probably mainly due to improvements in diagnosis
Occurs in approximately 1:200 – 1:100

Question 18

What are the seizure types

Answer 18

Focal
Generalised
Unclassifiable

Question 19

What are the types of focal seizures?

Answer 19

motor, sensory, autonomic, psychic ± automatisms ± GTCS

Question 20

What are the types of generalised seizures?

Answer 20

absence ± clonic/atonic/tonic/automatism atypical absence
myoclonic
clonic, tonic, tonic-clonic atonic

Question 21

Features of focal seizures

Answer 21

Manifestations depend on the area of the brain affected
Twitching, jerking or posturing if arise in the motor areas of the brain
Visual phenomena if arise in the occipital lobes
Abnormal smell or experiential symptoms if arise in temporal lobes

Question 22

Features of generalised seizures?

Answer 22

Absence – may include features of increase or loss of muscle tone,
jerks, etc
Myoclonic
Tonic, clonic, tonic-clonic
Atonic

Question 23

Seizure types which could lead to epilepsy syndrome

Answer 23

Childhood absence epilepsy
Juvenile myoclonic epilepsy
Benign focal epilepsies
Localisation related epilepsies - temporal
- frontal
Lennox Gastaut syndrome

Question 24

What are some aetiological diagnosis?

Answer 24

Imaging (esp. MRI)
Genetics
Pathology
etc.

Question 25

Genetics and epilepsy

Answer 25

Explosion of genetic input into diagnosis of epilepsies
- Familial epilepsies – e.g. benign - familial neonatal convulsions (KCNQ2, KCNQ3)
- Specific genetic mechanisms – e.g. SCN1A mutation in Dravet, GEFS+
. Genetic conditions characterised by epilepsy
- ARX
- PDCH19
- Ring chromosome 20
. Genetic conditions complicated by epilepsy
- Rett syndrome (MECP2, CDLK5)
- Angelman syndrome

Question 26

What is the mgmt types of epilepsy?

Answer 26

Drug therapy
Surgery
Service delivery
Co-morbidities

Question 27

What does drug therapy consist of?

Answer 27

~ 70% of people with epilepsy will achieve seizure control with a
single appropriate drug
10 - 20% will achieve reasonable control with 2 (or more) drugs
10 - 20% will not have adequate seizure control

Question 28

What are the principles of drug therapy?

Answer 28

Single drug (or rational polypharmacy) is desirable
Careful attention to side effects
Tailoring of drug therapy to epilepsy syndrome (as far as possible)
Drugs suppress seizures - they do not “cure” epilepsy

Question 29

What do you need to consider for drug options?

Answer 29

Requires clear identification of basic underlying seizure types
Focal
Generalised
Syndrome diagnosis ideal
Tailor drugs to seizures (add-on vs substitute)
Numerous drugs available – need for data

Question 30

What is the NICE - new drugs appraisal?

Answer 30

Before combination therapy is considered, people should be given a
trial of appropriate monotherapy
Caution needed during changeover periods between drugs.
Not possible to determine whether any one drug was more likely to
bring about seizure freedom over the longer term than any other

Question 31

What medications (AEDs) can be used for epilepsy/ epileptic seizures?

Answer 31

Gabapentin1 Lamotrigine1 Zonisamide4 Oxcarbazepine2 Tiagabine1 Vigabatrin1 Levetiracetam3 Topiramate1

Question 32

What does management of epilepsy include?

Answer 32

Effective drug therapy
Avoidance of side effects
Good service delivery
Life style issues
Aim is optimal quality of life

Question 33

How do we define refractory epilepsy?

Answer 33

Epilepsy can be considered to be refractory to medical treatment when there are continued epileptic seizures despite a trial of two appropriate anti-epileptic drugs given to tolerance

The drugs should have been administered in a dose which is
considered to be within the therapeutic target range

Accounts for 10-20% childhood epilepsy

Question 34

How do we approach refractory epilepsy?

Answer 34

Medical issues
Not epilepsy
Mis-diagnosis of epilepsy syndrome
Wrong choice of drug
Inappropriate drug dosage
“True” refractory epilepsy
Patient related issues
Non-compliance
Lifestyle issues

Question 35

What are the causes of refractory epilepsy?

Answer 35

Structural pathology
Progressive disease
Neurodegenerative disorder e.g. Batten’s
Tumour
Recognised refractory epilepsy syndrome
SMEI
Lennox Gastaut syndrome
Etc
Not known

Question 36

What is the clinical management of refractory epilepsy?

Answer 36

Re-evaluate history and all investigations
Review previous drug therapy
Confirm compliance
THEN:
Consider next drug options
Consider referral to paediatric neurologist
Possibility of epilepsy surgery

Question 37

What are the complications of epilepsy?

Answer 37

Education
Behaviour
Social stigma
Lifestyle issues
Driving, etc
Death
Accident
Status epilepticus
SUDEP

Question 38

Issues with education for those with epilepsy?

Answer 38

50% children achieve below level expected by IQ
Attentional difficulties
Specific learning difficulties
1/6 have frank learning difficulties

Question 39

Causes of learning disabilities

Answer 39

Underlying cause of epilepsy
Seizures
Sub-clinical discharges
Transient cognitive impairment
Drugs
Teacher/parental attitudes
Depression, etc.

Question 40

Complications of epilepsy?

Answer 40

Education
Behaviour
Social stigma
Lifestyle issues
Driving, etc
Death
Accident
Status epilepticus
SUDEP

Question 41

Behavioural issues and epilepsy

Answer 41

Children with epilepsy are consistently more behaviourally disturbed and have lower self-esteem than children with diabetes

Question 42

Causes of behavioural problems in epilepsy

Answer 42

Neurobiological factors
“non-epileptic” factors
effects of treatment
direct epileptic mechanisms
effects of epileptic activity on the brain
External factors
stigma
restrictions due to seizure unpredictability

Question 43

Treatment for epilepsy

Answer 43

No studies on intervention in behaviour difficulties
Careful assessment of possible causes of behaviour difficulties in each child
Education of child/family may be of value cf. Lewis et al, 1990
Attention to family interactions
Formal psychiatric intervention
Maximise seizure control
Active treatment of interictal abnormalities in Landau Kleffner
Avoidance of known AED precipitants
Low threshold for AED change
Avoidance of polypharmacy
Role of early surgery