Epilepsy

Question 1

Define epilepsy

Answer 1

Recurring tendency to have seizures
- At least 2 unprovoked seizures >24 hours apart
- 1 unprovoked seizure + probability of further seizures similar to general recurrence risk (60%) after 2 unprovoked seizures occurring over the next 10 years

Question 2

Define seizure

Answer 2

Excessive, abnormal and synchronised electrical activity that leads to external manifestations. Can be provoked or unprovoked

Question 3

What defines a prolonged and pseudo-seizure

Answer 3

Prolonged seizure = seizure activity ≥5 minutes
Pseudo-seizure= The patient believes to be having a seizure

Question 4

What is the cause of epilepsy

Answer 4

Most epilepsy (70-80%) is genetic/idiopathic with complex inheritance
Primary epilepsy syndromes e.g. idiopathic generalised, temporal lobe, juvenile myoclonic
Secondary: tumours, CNS infection, vasculitis, electrolyte abnormalities, drugs, haemorrhage/infarction, malignant hypertension, TBI

Question 5

What are the childhood epileptic syndromes

Answer 5

Infantile spasms (West syndrome)
Lennox-Gastaut
Childhood absence epilepsy
Epilepsy with centro-temporal spikes (Childhood rolandic epilepsy)
Panayiotopoulos syndrome (early-onset benign occipital epilepsy)
Juvenile absence epilepsy
Juvenile myoclonic epilepsy

Question 6

What is the difference between generalised and focal epilepsy and which types of epilepsy fall in each group

Answer 6

Generalised: Discharge arises from both hemispheres
- Convulsive: tonic, clonic, tonic-clonic, myoclonic
- Non-convulsive: absent, atonic

Focal: Seizures arise from one or part of one hemisphere. Each one will produce different symptoms. Originates in a relatively small group of dysfunctional neurones
- Simple partial: no loss of consciousness
- Complex partial: May have a loss of consciousness

Question 7

What is the classification of epilepsy according to the International League Against Epilepsy 2017

Answer 7

1. Where they begin: focal, unknown, generalised, focal to bilateral
2. Level of awareness: focal aware, focal impaired, awareness unknown, generalised
3. other features
   - Focal: motor onset (twitching, jerking, stiffening, automatisms) or non-motor (cognitive, emotional, sensory)
   - Generalised onset: motor (tonic clonic) or non-motor (absence, awareness changes, automatisms)

Question 8

What is the aetiology and risk factors of epilepsy

Answer 8

Most genetic/idiopathic (70-80%)

Family history
Perinatal asphyxia
Metabolic disorders
Febrile seizure
Head trauma
CNS infection or tumour
Cerebral palsy
Learning disabilities
Down’s syndrome

Question 9

What is the epidemiology of epilepsy

Answer 9

0.05% incidence (less common during the first year of life) and a prevalence of 0.5%
Peak age of onset in early childhood or in the elderly <20, >60
Males > females

Question 10

What are the signs and symptoms that suggest a generalised seizure

Answer 10

Before: Epileptic aura, triggers e.g. flashing lights
During: stiffness, jerking, incontinence, tongue-biting, sweating, palpitations, mouth frothing, pallor, cyanosis
After: Muscle ache, post-ictal confusion, drowsiness

Question 11

Describe absence seizures

Answer 11

Transient loss of consciousness
Abrupt onset and termination
May see flickering of the eyelids and minor alteration in muscle tone, otherwise NO motor phenomena
May be precipitated by hyperventilation

Question 12

Describe myoclonic seizures

Answer 12

Brief, repetitive, jerking movements of the limbs, neck or trunk
Includes benign rolandic epilepsy and juvenile myoclonic epilepsy

Question 13

Describe the types of childhood myoclonic epileptic disorders

Answer 13

Benign Rolandic Epilepsy (BRE) (most common childhood epilepsy) / Sylvian seizures
3-12yo
S/S: seizures of face / upper limbs during sleep with hypersalivation & speech arrest
Childhood (age 3-12yo) seizures – outgrown at end of puberty

Juvenile myoclonic epilepsy
12-18yo
Usually involving neck, shoulders, upper arms, most occur after waking up
Begin around puberty

Question 14

Describe tonic epileptic seizures

Answer 14

Generalised increase in tone

Question 15

Describe tonic-clonic seizures

Answer 15

1. Tonic phase - children may fall to the ground, sometimes injuring themselves
   - May not breathe → cyanosis
2. Clonic phase - Rhythmical contraction of muscle groups → jerking of limbs
   - Breathing may be irregular as cyanosis persists and saliva accumulates in the mouth
   - May show biting of the tongue and incontinence of urine
3. Post-ictal phase:
   - followed by unconsciousness or deep sleep for up to several hours

Question 16

Describe atonic seizures

Answer 16

Often combined with myoclonic jerk, followed by a transient loss of muscle tone causing a sudden fall to the floor or drop of the head

Question 17

Describe frontal focal epileptic seizures

Answer 17

Behavioural changes
Motor: posturing, peddling, motor arrest (dysphasia, speech arrest)

± Jacksonian March: As the seizure increases in size, more of the homunculus becomes affected i.e. hand shaking → elbow shaking → Arms shaking
± Todd’s paralysis: Post-ictal flaccid weakness

Question 18

Describe temporal focal epileptic seizures

Answer 18

Aura (visceral and psychic symptoms - fear or deja-vu sensation)
Hallucinations (smell, taste)
Emotional disturbance
Depersonalisation
Automatisms e.g. lip-smacking, fumbling, fiddling, chewing, singing, kissing)

Question 19

Describe parietal focal epileptic seizures

Answer 19

Sensory disturbances: tingling, numbness
Motor symptoms: abnormal movements, rhythmic muscle contractions

Question 20

Describe occipital focal epileptic seizures

Answer 20

Visual phenomena: spots, lines, flashes

Question 21

What investigations should be done for epilepsy

Answer 21

Bedside: ECG, urine dip, BM
Bloods: Glucose, U&Es, FBC, genetic testing, toxicology, serum prolactin, CRP/ESR
Other: MRI brain, EEG (inter-ictal, ictal, sleep-deprived), /CT head/PET/SPECT, ± LP

Question 22

What is the management for a child with a first fit

Answer 22

Urgent referral to hospital for paediatric assessment
Referral to first fit clinic

Question 23

What is the long term management for epilepsy

Answer 23

Advice and safety netting
MDT management (paeds, neuro, school nurse, epilepsy nurse, GP)
Anti-epileptic monotherapy (usually valproate)
Rescue therapy (seizures >5mins): Buccal midazolam

Question 24

What anti-epileptics are indicated in tonic-clonic seizures

Answer 24

First line: sodium valproate, carbamazepine
Second line: clozabam, levetiracetam, Topiramate

Question 25

What anti-epileptics are indicated in myoclonic seizures

Answer 25

First line: sodium valproate, levetiracetam
Second line: clozabam, clonazepam

Question 26

What anti-epileptics are indicated in absence seizures

Answer 26

First line: valproate, ethosuximide
Second line: clozabam, lecetiracetam, topiramate

Question 27

What anti-epileptics are indicated in focal seizures

Answer 27

First line: carbamazepine, valproate, levetiracetam, lamotrigine
Second line: clozabam, topiramate, gabapentin

Question 28

What are the treatment options for intractable epilepsy

Answer 28

Ketogenic (low-carb, fat-based) diets
Vagus nerve stimulation or responsive neurostimulation system
- delivered using externally programmable stimulation of a wire implanted around the afferent (left) vagus nerve
Resective epilepsy surgery (lobectomy, hemispherotomy
- For epilepsy has a well-localised structural cause or epileptogenic zone
- Demonstrated by good concordance between ictal EEG, MRI, functional imaging findings

Question 29

What advice should be given for children with epilepsy

Answer 29

How to recognise a seizure
Video record future seizure
Avoid dangerous activities (i.e. swimming or deep baths) unsupervised
Ensure school is informed
Support: epilepsy action
Adolescents → 1 year free of seizures is safe to drive, may interact with contraception, affects pregnancy outcomes
Avoid alcohol, drugs, poor sleep

Question 30

Define status epilepticus

Answer 30

Continuous seizure activity ≥ 30 minutes or repetitive seizures with no intervening recovery of consciousness

Question 31

What is the management for status epilepticus

Answer 31

0mins:
- A-E
- High flow oxygen if available
- Check BM and confirm it is epileptic seizure

5mins:
- Buccal midazolam / rectal diazepam
- IV established: IV lorazepam

15mins:
- IV lorazepam
- Call senior
- Re-assess, confirm epilepsy

25mins:
- IV phenytoin/IV phenobarbitone (if already on phenytoin)
- Call anaesthetics and inform ICU

45mins: Rapid sequence induction of anaesthesia with thiopental, PICU transfer

Question 32

What are the complications of epilepsy

Answer 32

Falls and injuries → Fractures
Aspiration
Status epilepticus - respiratory arrest
SUDEP - sudden death in epilepsy
Depression and anxiety disorders
Absence from school, learning difficulties

Question 33

What is the prognosis for epilepsy

Answer 33

50% remission at 1 year
Mortality 2 in 100,000/year
Related to seizure or secondary to injury
Children with epilepsy do less well educationally, with social outcomes and future employment compared to others with chronic conditions e.g. diabetes
2/3 of children go to mainstream school but some may need educational help for associated learning difficulties.
1/3 go to special schools but often have multiple disabilities and their epilepsy is part of a severe brain disorder.
Few children require residential schooling

Question 34

What are the side effects of anti-epileptics

Answer 34

Valproate: weight gain, hair loss, rare idiosyncratic liver failure, teratogenic (DO NOT give to female children/adolescents, women of childbearing potential or pregnant women

Carbamazepine: rash, neutropoenia, hyponatraemia (SIADH), ataxia, inducer

Lamotrigine: severe skin rash (SJS)

Levetiracetam: sedation (rare)