Epilepsy Flashcards

1
Q

Focal vs generalised seizures

A

Focal:
- limited to one hemisphere
- divided into those with retained awareness or impaired awareness

Generalised:
- bilateral networks
- divided into motor and non-motor (absence) seizures

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2
Q

Define epilepsy

A

> =2 unprovoked seizures occurring > 24h apart (recurrent risk > 60%)

or 1 unprovoked seizure and probability of further seizures occurring >=60% over next 10 years

or diagnosis of an epilepsy syndrome

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3
Q

Convulsive status epilepticus

A

Prolonged seizure >= 5 minutes or recurrent seizures without recovery in between

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4
Q

Causes of epilepsy and incidence

A

Cause only found in 1/3

1) Structural: stroke, congenital, acquired

2) Genetic eg. Dravet syndrome

3) Infectious: TB, cerebral malaria, HIV, Zika

4) Metabolic: porphyria, pyridoxine deficiency

5) Immune: anti-NMDA receptor encephalitis

Highest risk in infants and > 50y
Lifetime risk of epilepsy = 3%, 5-10/1000
Lifetime risk of single seizure = 10%

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5
Q

Prognosis in epilepsy

A

80% with childhood absence epilepsy will be in remission by adulthood

In children and adults 70%% will be in remission - seizure free for 5y on or off treatment

Predictors :
- number of seizures in first 6 months
- response to antiepileptic treatment

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6
Q

When can you consider withdrawing antiepileptic medication?

A

Seizure free for > 2 years - reduce over 3 months (longer if bentos / barbiturates), if on multiple do one at a time.

  • can;t drive for 6 months after stopping treatment or if seizures recur
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7
Q

When is epilepsy considered resolved?

A

Seizure free for past 10y with no antiepileptic treatment for past 5y

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8
Q

Epilepsy risk factors

A

1) Premature
2) Complicated febrile seizures
3) Genetic condition associated with epilepsy (tuberous sclerosis/neurofibromatosis)
4) Brain development malformations
5) FH
6) Cerebrovascular disease or stroke
7) Dementia and neurodegenerative disorders - Alzheimers 10x increased risk

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9
Q

Seizure investigations

A

1) ECG for all to r/o cardiac condition that could mimic epilepsy

2) Bloods: FBC, U&E, LFT, BP, glucose

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10
Q

Management of suspected epilepsy

A

1) First fit clinic - seen within 2 weeks

2) Advice:
- stop driving
- avoid working with heavy machinery, heights, swimming, baths
- lifestyle factors that may lower seizure threshold
- contact GP if further episodes whilst waiting for specialist
- record seizures
- keep diary
- cushion head & recovery position after
- time seizure - 999 if > 5 minutes / recurrent without recovery

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11
Q

Todd’s paralysis

A

Temporary weakness after seizure, lasting from a few minutes to 36h before resolving

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12
Q

Managing an epileptic seizure

A

1) Cushion head, remove jewellery and glasses

2) Recovery position once stopped

3) Admit if 1st seizure/recurs after shotrt time/injured/difficult to wake

Tonic-clonic > 5 minutes or > 3 seizures/hour:
1) Buccal midazolam (not licensed < 3m) / rectal diazepam.(not licensed < 1y)

2) 999 if does not respond if does respond and:
- prolonged before treatment given
- history of repeated seizures/Status
- difficulties monitoring
- 1st seizure

3) Specialist review - provide carers with buccal midazolam or rectal diazepam if not already provided

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13
Q

Annual epilepsy review

A

1) Seizure control
- if seizure recurrence after period of remission -urgent referral within 2 weeks

2) Long-term carbamazepine - risk of osteoporosis - calcium + vitamin D supplements

3) contraception

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14
Q

Contraception options if on enzyme-inducing drugin epilepsy

A

If on enzyme inducing drug:

1) Medroxyprogesterone acetate injections

2) IUD/levonorgestrel-IUS

If on lamotrigine:
1) Oestrogens may reduce effectiveness of lamotrigine
2) Progestogen only contraceptives can be used without restriction

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15
Q

Anti-epileptic drugs

A

Cat 1: ensure maintained on specific manufacturers product - phenytoin, carbamazepine, phenobarbital, primidone

Cat 2: clinical judgement depending on seziure frequency and treatment history - valproate, lamotrigine, clonazam, topiramate

Cat 3: can switch manufacturer - levetiracetam, gabapentin, pregabalin, vigabatrin

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16
Q

Anti-epileptic enzyme inducers

A
  • carbamazapine
  • phenobarbital
  • phenytoin
  • primidone
  • topiramate
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17
Q

Anti-epileptic non-enzyme inducers

A

aetazolamide
-clobazam
-gabapentin / pregabalin
-lamotrigine
-levetiracetam
- valproate
- topiramtte

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18
Q

Driving in epilepsy

A

Inform DVLA of any seizures / blackouts

G1:
1) Epileptic seizures whilst awake and lost consciousness - licence taken away, can reapply when seizure free for 1 year

2) Seizure due to medication change/reduction - reapply when last seizure 6m ago + back on previous medication for 6m

3) First seizure whilst awake and lost consciousness - licence taken away, reapply when no seizure for 6m and DVLA medical advisor indicated there isn’t a high risk of a further seizure

4) Seizures whilst asleep and awake - if seizures for past 3y only when asleep DVLA may allow you to have licence

5) Only had seizures whilst asleep - if > 12m sine first seizure, DVLA may allow you to have license.

6) Seizures that don’t affect consciousness - if only type of seizure has been when fully conscious and aware and able to move and first seizure > 12m ago, may get licence back.

G2:
1) > 1 seizure - need to not have had epileptic seizure for 10y, taken any anti-epileptic drug for 10y and have < 2% risk of anther seizure according to DVLA - reapply

2) One off seizure.- no epileptic sezizure for 5y and no medication for 5y and been assessed by neurologist in past 12m and DVLA agree

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19
Q

What factors increase risk of second seizure following first?

A

1) Mental health problem - depression, anxiety, psychosis, alcohol/substance misuse

2) Vascular risk factors: DM, HTN, AF

3) Sepsis

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20
Q

Which features of febrile seizure increase risk of epilepsy later on?

A

1) > 10 minutes

2) Associated with features eg. weakness

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21
Q

When should EEG be offered?

A

1) History and examination suggests epilepsy - routine EEG while awake

2) Cannot be used to exclude epilepsy

3) If EEG offered after first seizure, should be within 72h

4) Discuss risks vs benefit of using provoking manoeuvres during EEG

5) If EEG normal consider sleep-deprived EEG

6) If routine & sleep-deprived EEG are normal and diagnostic uncertainty, consider 48h EEG

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22
Q

MRI in epilepsy

A

Offer to all unless idiopathic generalised epilepsy of self-limited epilepsy with centrotemporal spikes

Should be done within 6 weeks of referral

If CI offer CT

Repeat MRI if initial scan suboptimal, new features to epilepsy, idiopathic generalised epilepsy or with centrotemporal spikes which has not responded to 1st- line treatment or if surgery being considered

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23
Q

Who should be offered genetic testing?

A

1) < 2 years when epilepsy started

2) Clinical features suggestive of syndrome

3) Associated with learning disability, ASD, structural abnormality, unexplained cognitive decline

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24
Q

When should patients be referred to a tertiary service?

A

1) All within 4 weeks if:
- uncertainty about Dx
- has epilepsy syndrome likely to be drug resistant
- for further assessment or treatments
- wants to participate in research

2) Children, within 2 weeks if:
- < 3 years old
- < 4 years old and have myoclonic seizures.
- unilateral structural lesion
- deterioration in behaviour, speech or learning

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25
Q

Principles of epilepsy treatment

A

Use mono therapy if possible.

If 1st line monotherapy fails and epilepsy Dx confirmed - try alternative mono therapy - increase dose of 2nd drug slowly whilst maintaining dose of 1st, then if 2nd drug successful slowly wean 1st. If 2nd unsuccessful wean this one and try alternative.

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26
Q

When to start antiseizure medication

A

1) Once diagnosis of epilepsy confirmed

2) After first unprovoked seizure if:
- neurological deficit
- EEG - unequivocal epileptic activity
- risk of further seizures not acceptable to patient
- structural abnormality on imaging

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27
Q

Safety considerations for phenytoin

A

Increased risk of serious skin reactions in Han Chines or Thai

28
Q

Safety considerations for carbamazepine

A

Increased risk of serious skin reactions in Han Chinese, Thai, European, Japanese

29
Q

Which anti-seizure medications are associated with reduced bone mineral density when used long term?

A

Carbamazepine, phenytoin, primidone and sodium valproate

30
Q

Anti-seizure medications for women and girls - advice

A

Sodium valproate - increased risk with increased dose and polytherapy

Carbamazepine phenytoin and topiramate can impair effect of hormonal contraceptives

Oestrogen can impair effectiveness of lamotrigine

Breastfeeding for most is safe

After birth should return to preconception dose within days

31
Q

Who needs annual review in epilepsy?

A

1) LD

2) Drug-resistant

3) High risk of SUDEP

4) Complex psychosocial, cognitive or mental health problems

5) On meds associated with long-term effects/drug interactions

6) On valproate and of child-bearing age.

7) Child

32
Q

When would you monitor anti seizure medication levels?

A

1) uncontrolled.

2) side effects

3) Pregnancy/renal failure.

4) Poor adherence

5) Planning pregnancy and considered at risk of seizures worsening

6) When starting medication in women planning pregnancy - baseline level and monitor to check adherence

33
Q

Treatment of generalised tonic-clonic seizures

A

1st line for boys/men (likely to change if < 55y) / girls < 10y who unlikely to need treatment when of child-bearing potential) / women who can’t have children
- sodium valproate monoRx
- offer monoRx with lamotrigine or levetiracetam if it doesn’t work

1st line for women and girls of child bearing age or likely to need treatment when of child-bearing age
- lamotrigine monoRx (off-label < 13y) or levetiracetam (offer the other if one doesn’t work)

Add-on treatment if monoRx unsuccessful:
- clobazam (off-label < 6m)
- lamotrigine (off-label < 2y)
- levetiracetam (off-label < 12y)
- perampanel (off-label < 7y)
- sodium valproate
- topiramate (off-label < 2y)

2nd add on options:
- brivacetam
- lacosamide
- phenobarbital
-primidone
- zonisamide

34
Q

Conditions for prescribing valproate

A

1) Female < 55y - 2 specialists independently report no other effective or tolerated treatment

AND

Conditions of prevent are fulfilled if of childbearing potential

2) Men < 55y - 2 specialists independently report no other effective or tolerated treatment unless vasectomy/infertile due to other causes.

35
Q

Prevent - sodium valproate

A

GP - review female patients are sodium valproate initiated (unless post menopause/hysterectomy) to ensure conditions of prevent fulfilled.

1) Patient guide and copy of signed annual risks acknowledgement form done with specialist

2) Effective contraception - IUD/implant or 2 complimentary forms inc. barrier method

3) Female child - must inform GP at menarche - refer back to specialist

4) Annual specialist review

36
Q

Teratogenic effects of sodium valproate

A

1) Congenital malformations:
- 11% on mono therapy - major malformation (2-3% in general population), increases with polytherapy and dose
- neural tube defects facial dysmorphism, cleft lip & palate, craniostenosis, heart, renal, urogenital defects, limb defects (BL aplasia of radius) , deafness, eye malformations

2) Neurodevelopmental disorders:
- 30-40% delayed development, ASD, ADHD

37
Q

Sodium valproate - effects on men

A

1) Testicular degeneration/atrophy, spermatogenesis abnormalities

38
Q

Which antiepileptics may exacerbate seizures in people with absence or myoclonic seizures including juvenile myoclonic epilepsy?

A

1) Carbamazepine
2) Gabapentin
3) Lamotrigine - myoclonic
4) Oxcarbamazepine
5) Phenytoin
6) Pregabalin
7) Tiagabine
8) Vigabatrin

39
Q

Management of focal seizures without evolution of bilateral tonic clinic seizures

A

1st line monotherapy: lamotrigine (off-label < 13y) or levetiracetam (off-label < 16y)

2nd line mono therapy: carbamazepine, ox carbamazepine, zonisamide

3rd line mono therapy: lacosamide

1st line Add on:
- carbamazepine
-lacosamide
- lamotrigine
- levetiracetam
- oxcabramazline
- topiramate
- zonisamide

40
Q

Management of absence seizures

A

1st line: ethosuximide

2nd line: sodium valopriate

3rd line: lamotrigine or levetiracetam

If associated with other seizure types:
- consider sodium valproate 1st line (unless < 55 etc) or lamotrigine or levetiracetam

41
Q

Management of myoclonic seizures

A

1st line: sodium valproate

Alternative 1st line: levetiracetam (off-label)

2nd line: alternative 1st line

Then add-on treatments:
- brivacetam
- clobazam
- clonezapam
- lamotrigine
- piracetam
-topiramate
- zonisamide

LAMOTRIGINE can exacerbate myclonic seizures

42
Q

Management of tonic or atonic seizures

A

See specialist to diagnose the syndrome and guide management

1st line: sodium valproate

Alternative 1st line: lamotrigine

43
Q

Management of idiopathic generalised epilepsy

A

1st line: sodium valproate

Alternative 1st line: lamotrigine or levetiracetam

3rd line add-on : perampanel or topiramate

44
Q

Dravet syndrome

A

Mutation SCN1A

Starts < 1 year (3-8% who have seizure < 1y have Dravet)

Often initial seizure is febrile seizure

Seizures triggers by slight changes in body temperature eg. warm bath/heat , flashing lights/patterns, stress

Seizure features: > 10 minutes, one side of body and triggers as above

> 2 years can loose developmental milestones

Management:
1st line: sodium valproate even in females
Then triple therapy: + stiripentol + clobazam
2nd line > 2y: cannabidiol + clobazam + SV
3rd line or 2nd line and < 2y: add on ketogenic diet, levetiracetam, topiramate
4th line: potassium bromide

45
Q

Lennox-Gastaut syndrome

A

Seizures start 3-5 y old (18m-10y)

1 in 5 with infantile epileptic spasm syndrome will develop LGS

Developmental delays before seizures

All have tonic seizures and can also have other types

Causes: genetic, tuberous sclerosis, malformation during pregnancy, lack of oxygen, meningitis, heard injury

1st line: SV even if female
2nd line: lamotrigine

46
Q

Infantile spasms syndrome (West syndrome)

A

Begins 4m-8m old - clusters of spasms lasting several minutes, often own waking, developmental delay

Causes: structure of brain, injury, meningitis, mutation

< 2 years - review weekly during treatment and repeat EEG 2 weeks after starting treatment

1st line: high dose prednisolone + vigabatrin (if not due to tuberous sclerosis). If TS give vigabatrin alone and if ineffective after 1w add prednisolone

Before starting steroid check antibodies to VZV and give steroid and

47
Q

Self-limited epilepsy with centrotemporal spikes (benign Rolandic epilepsy)

A

Usually begins 6-8y

Most children will outgrow seizures by adolescence

When awake - twitching/tingling of one side of face/tongue < 2 minutes

When asleep - twitching one side of face, but often progress to tonic-clonic

Normal development

1st line: lamotrigine or levetiracetam

2nd line: carbamazepine, oxcarbamazepine, zonisamide

48
Q

Myotonic atonic epilepsy (Doose syndrome)

A

First seizure 2-6y
- > 50% generalised TC +/- fever, then within days drop seizures begin
- can also have absence/myoclonic
- respond poorly to medication

2/3 will outgrow

1st line: levetiracetam or SV
2nd line: ketogenic diet

49
Q

SUDEP:

Risk factors

A

Risk factors:
- non-compliance
- alcohol/substances
- focal to bilateral tonic-clinci seizures or generalised tonic clonic seizures
- uncontrolled seizures
- living or sleeping alone
-previous brain injury / CNS infection /metastatic cancer / CVA / abnormal neurological findings

50
Q

Which antiepileptic is associated with peripheral neuropathy?

A

Phenytoin

51
Q

Adverse effects of phenytoin

A

Acute:
- dizziness, diplopia, nystagmus, slurred speech, ataxia
- confusion, seizures

Chronic:
- gingival hyperpleasia, hirsuitism, coarsening of facial features , drowsiness
- megaloblastic anaemia (alters folate metabolism)
- peripheral neuropathy
- enhanced vit D metabolism - osteomalacia
- LN
- dyskinesia

Idiosyncratic:
- fever
- rashes, including TEN
- hepatitis
- Dupytrens contracture
- aplastic anaemia
- drug induced lupus

Teratogenic:
- cleft palate, congenital heart disease

52
Q

Monitoring of phenytoin

A

Not routine

Check torugh levels immediately before dose if adjustment to dose, suspected toxicity or non-adherence

53
Q

Driving after first unprovoked/isolated seizure

A

No driving for 6 months if no relevant structural abnormalities on imgaing and no definite epileptiform activity on EEG - if these conditions not met, no driving for 12 months

54
Q

Driving after established epilepsy or multiple unprovoked seizures

A

Need to be seizure free for 12 months to qualify for driving licence

If no seizures for 5y a ‘til 70 licence is usually restored

55
Q

Driving when withdrawing epislepy medication

A

No driving for 6m

56
Q

Dose of rectal diazepam to give during prolonged seizure

A

1m-1y - 5mg

2y-11y - 5 -10mg

12y - 17y - 10mg

Adult - 10 - 20mg

Elderly - 10mg (max 15mg)

Can repeat dose at 10-15 minutes if needed

57
Q

Dose of buccal midazolam to give during prolonged seizure

A

1 - 2 months - 300mcg/kg, max 2.5mg

3 - 11 months - 2.5mg

1 - 4 years - 5mg

5 - 9 years - 7.5mg

10 -17 years - 10mg

Adult - 10mg (unlicenced)

58
Q

West syndrome

A

Infantile spasms

Features:
- infantile spasms in first few months of life
- flexion of head, trunk, limbs –> extension of arms (Salaam attack), lasting 1-2 seconds, repeats up to 50x
- usually secondary to serious neurological abnormality eg. tuberous scelerosus, encephalitis, birth asphyxia)

Management:
- vigabatrin
- steroids

Prognosis: poor

59
Q

Typicsl (petit mal) absence seizures

A

Onset: 4-8y

Duration ofa few-30 seconds, no warning, quick recovery, many/day

EEG: 3Hz generalised, symmetrical

Management:
- sodium valproate
- Ethosuxamide

Prognosis: 90-95% seizure free in adolescence

60
Q

Lennox-Gautault syndrome

A

May be an extension of infantile spasms

Onset: 1-5y

Features:
- atypical absences, falls, jerls
- 90% moderate-severe handicap

EEG: slow spike

Management:
- ketogenic diet

61
Q

Benign rolandic epilepsy

A

Childhood, males

Parasthesia (unilateral face) usually on waking

62
Q

Juvenile myoclonic epilepsy (Janz syndrome)

A

Onset: teenagers, girls

Features:
- initially present with absences or intermittent myoclonus of upper limbs
- Within a few months - infrequent generalised tonic-clonic seizures, often in morning/following sleep deprivation
- daytime absences
- sudden, shock like myoclonic seizure (these may develop before seizures)

Management:
- referral to paediatric neurology
- sodium valproate

63
Q

GCS

A

M:
1 - none
2 - extending to pain
3 - flexing to pain - decorticate
4 - withdraws from pain
5- localises to pain
6 - obeys commands

V:
1 - none
2 - sounds
3 - words
4 - confused
5 - oriented

64
Q

Which anti-epileptic is associated with Stephen Johnson Syndrome?

A

Lamotrigine

Flu-like symptoms
Painful erythema, blisters, ulcerations

65
Q

Which are the most important anti-epileptic drugs to prescribe by brand?

A

Phenytoin, carbamazepine, phenobarbital, primidone