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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
Principles of epilepsy treatment
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.
26
When to start antiseizure medication
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
27
Safety considerations for phenytoin
Increased risk of serious skin reactions in Han Chines or Thai
28
Safety considerations for carbamazepine
Increased risk of serious skin reactions in Han Chinese, Thai, European, Japanese
29
Which anti-seizure medications are associated with reduced bone mineral density when used long term?
Carbamazepine, phenytoin, primidone and sodium valproate
30
Anti-seizure medications for women and girls - advice
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
Who needs annual review in epilepsy?
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
When would you monitor anti seizure medication levels?
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
Treatment of generalised tonic-clonic seizures
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
Conditions for prescribing valproate
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
Prevent - sodium valproate
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
Teratogenic effects of sodium valproate
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
Sodium valproate - effects on men
1) Testicular degeneration/atrophy, spermatogenesis abnormalities
38
Which antiepileptics may exacerbate seizures in people with absence or myoclonic seizures including juvenile myoclonic epilepsy?
1) Carbamazepine 2) Gabapentin 3) Lamotrigine - myoclonic 4) Oxcarbamazepine 5) Phenytoin 6) Pregabalin 7) Tiagabine 8) Vigabatrin
39
Management of focal seizures without evolution of bilateral tonic clinic seizures
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
Management of absence seizures
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
Management of myoclonic seizures
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
Management of tonic or atonic seizures
See specialist to diagnose the syndrome and guide management 1st line: sodium valproate Alternative 1st line: lamotrigine
43
Management of idiopathic generalised epilepsy
1st line: sodium valproate Alternative 1st line: lamotrigine or levetiracetam 3rd line add-on : perampanel or topiramate
44
Dravet syndrome
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
Lennox-Gastaut syndrome
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
Infantile spasms syndrome (West syndrome)
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
Self-limited epilepsy with centrotemporal spikes (benign Rolandic epilepsy)
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
Myotonic atonic epilepsy (Doose syndrome)
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
SUDEP: Risk factors
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
Which antiepileptic is associated with peripheral neuropathy?
Phenytoin
51
Adverse effects of phenytoin
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
Monitoring of phenytoin
Not routine Check torugh levels immediately before dose if adjustment to dose, suspected toxicity or non-adherence
53
Driving after first unprovoked/isolated seizure
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
Driving after established epilepsy or multiple unprovoked seizures
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
Driving when withdrawing epislepy medication
No driving for 6m
56
Dose of rectal diazepam to give during prolonged seizure
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
Dose of buccal midazolam to give during prolonged seizure
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
West syndrome
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
Typicsl (petit mal) absence seizures
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
Lennox-Gautault syndrome
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
Benign rolandic epilepsy
Childhood, males Parasthesia (unilateral face) usually on waking
62
Juvenile myoclonic epilepsy (Janz syndrome)
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
GCS
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
Which anti-epileptic is associated with Stephen Johnson Syndrome?
Lamotrigine Flu-like symptoms Painful erythema, blisters, ulcerations
65
Which are the most important anti-epileptic drugs to prescribe by brand?
Phenytoin, carbamazepine, phenobarbital, primidone

Neurology (16 decks)

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