Lecture 6 - Epilepsy Paeds Flashcards Preview

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Flashcards in Lecture 6 - Epilepsy Paeds Deck (62):
1

Broadly how does epilepsy have such a big impact on children who have it.

Children with epilepsy most commonly have slowed cognitive progression which can impact daily life (in the home, school, and socially) resulting in widespread psychological and cognitive impact.

2

Does epilepsy make a child less smart?

Cognitively epilepsy has been associated with a downward trend in IQ. Where the normal range is 80-115+, the mean IQ for children with epilepsy has often been found to fall within the lower end of this 'normal' range (85-90).

However, IQ is a gross and insensitive measure, it does not necessarily offer a complete picture of the difficulties faced by children with epilepsy. There are commonly subtle deficits (E.g., memory, executive, language, processing speed and behavioural symptoms) that result either DIRECTLY from the seizure activity or INDIRECTLY from the impact of epilepsy on the child's home, school, and social functioning/life.

In many forms of epilepsy, there is slowed cognitive progress, that is rather then a decline in previously acquired abilities, though this can also occur particularly in epileptic encephalopathies.

It is also important to note that a 'mean IQ' does not represent everyone equally. Children are likely to have greater intellectual impairment when:

- Seizures have an early onsets, and the disorder is ongoing.
- Frequent seizures.
- Requires polytherapy
- Treatment resistant seizures (intractable), particularly if they are generalised.
- Status epilepticus/non-convulsive state

3

What is the difference between a decline and delay of cognitive development? Define both.

b

4

In epilepsy, are declines or delays in development more common?

b

5

Name the types of epilepsy associated with DECLINE (not just delay) in development/cognitive abilities.

Encephalitic encephalopathies (e.g., west syndrome and lennox-gastaut)

6

What is the lifetime prevalence of having a seizure? and during what years of life are they more likely to occur?

5%. Infants

7

Approx how many people in 1000 have epilepsy?

3-8 per 1000

8

Why might infants have a particular vulnerability to seizures?

Immature brain - GADA theory
or secondary to underlying abnormality:
- encephalopathy
-haemorrhage
-metabolic disorders
- congenital cerebral malformation
- neuronal migration disease (cortical dysplasia)

9

What neuropathological finding characterises Tuberous Sclerosis?

calcified lesions ('tubers') in the brain, often also have lesions on the skin. Sometimes tubers are static, other time new ones grow. May have a genetic component.

10

What is polymicrogyria?

Hyper-folding of the gyri

11

What is the definition of a 'seizure'?

(ILAE and IBE) definition:

An epileptic seizure is a transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain.

Or SIMPLIFIED:

A period of abnormal electrical activity in the brain, usually with associated clinical symptoms.

12

What is the current (2014) operational definition of epilepsy? (ILAE)

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

1. At least 2 unprovoked seizures occurring >24h apart;
2. One unprovoked seizure and probability of further future seizure (at least 60% risk) occurring in the next 10 years.
3. Diagnosis of an epilepsy syndrome.

13

What is an ictal event?

Seizure event, an active seizure

14

What is another name of a seizure event?

Ictal event

15

What does 'interictal' mean?

Period between seizure events

16

What is the name for the period between seizures?

Interictal

17

What does 'post-ictal' mean?

Period immediately following a seizure event

18

What is the name for the period immediately following a seizure event

Post-ictal

19

What is an Aura?

A subjective phenomenon just prior to, or simultaneous with , the start of a seizure that can act as a warning.

These experiences rarely last more than a few seconds, but the nature of the Aura can be diagnostically relevant.

For example, Nausea/epigastric aura can indicate a temporal lobe epilepsy.

20

What are the three TIERS of epilepsy classification?

TIER 1: Seizure type (generalised, localised, undetermined)
TIER 2: Seizure aetiology (idiopathic, symptomatic, cryptogenic)
TIER 3: Syndrome (specific epilepsy syndromes, a cluster of signs & symptoms)

21

What are the 2 MAIN categories of type of seizure (TIER 1)?

1. Partial seizure (AKA focal or local seizures, affecting only part of the brain)
2. Generalised seizures (where clinical and EEG data indicate aberrant activity in both cerebral hemispheres simultaneously at onset.

22

Describe what a 'generalised' seizure is and outline the types of generalised seizures.

Generalised seizures are where clinical and EEG data indicate aberrant activity in both cerebral hemispheres simultaneously at onset.

TYPES:
Absence seizures ("petit mal") - characterised by EEG activity with minimal or no outward signs. Child will look phased out, for a brief period (1-2 --30 seconds), may have mild motor signs (e.g., humming or eye-deviation)

Tonic-Clonic seizures ("grand mal") - The 'typical' seizure, combinations of muscle (tonic) tensing and jerking (clonic) movements.

Myoclonic seizures - Brief shock like twitching , which can be restricted to specific muscle groups (E.g., eye lid)

Clonic seizures - Small jerking movements (Without tonic rigidity)

Tonic seizures - Tensing/flexing, rigidity, increased tone of muscle

Atonic Seizures - Bodies muscles relax/go floppy, can occur in only some body parts, or everywhere as in a 'drop attack' (drop attacks may also occur in tonic)

23

Describe absent seizures

Absence seizures ("petit mal") - characterised by EEG activity with minimal or no outward signs. EEG of 3hz with spikes-waves pattern. Child will look phased out, for a brief period (1-2 --30 seconds), may have mild motor signs (e.g., humming or eye-deviation)

24

What is 'status epilepticus?'

A medical emergency, recurrent tonic-clonic without recovery of consciousness between attacks. EEG will show continuous, abnormal activity.

Most commonly occurs in severe epilepsy where there is poor medication adherence and/or epilepsy of encephalitic origins.

Repeated attacks of status epilepticus can affect cognition (highly damaging to brain). high morbidity.

25

What are the three types of aetiology of seizures? (TIER 2)
(also include other/old names for these)

1. "Unknown" (formerly 'idiopathic' and 'primary' before that) - Arising spontaneously from an obscure or unknown cause.

2. "Structural/Metabolic" (formally known as 'symptomatic' and 'secondary' before that) - Arising as a symptom of a known brain abnormality

3. Genetic (new as of 2016 paper)

There USED to be a 'cryptogenic' aetiology = presumed neuropathological cause, but lack of evidence (E.g., MRI normal) - but these now come under 'unknown'.

26

What is an EEG?

Measures electrical activity of the cortex via 10-20 electrodes places on the scalp. Each electrode measures electric activity in the are beneath it (though it is not a perfect/exact measure) and can give an idea of the nature of the seizure as well as the location/origin.

27

What type of seizure has the characteristic EEG presentation of a generalised 3Hz spike-wave complex?

Absence seizures.

28

What signs and symptoms are used to define epileptic syndromes (TIER 3)?

Seizure type
Precipitants
EEG/Imaging characteristics
Pathological information
Age of onset
Duration
Frequency, severity
Aetiology and prognosis

Many signs and symptoms occur uniquely together to form a recognisable 'syndrome'. However, this is not always the case, especially in kids. Therefore a neurologist may give a preliminary diagnosis and list the TIER 1 and TIER 2 features instead as the nature and locus of the seizures are the most important information.

29

What are the primary features of 'West Syndrome"?
- Age of onset, type of epilepsy, aetiology, presenting symptoms.

West syndrome, also known as "infantile spasms", as the typical onset is between 3-12 months, is an epileptic encephalopathy (global brain dysfunction) of varied aetiology (can be secondary to a brain abnormality or cryptogenic).

Associated with deterioration of sensory, motor, and/or cognitive functions due to epileptic activity, including frequent seizures and /or prominent interictal paroxysmal activity.

It is characterised by a triad of symptoms:
1. Infantile spasms - clusters of sudden brief movements of the head, neck, and/or limbs.
2. Psychomotor deterioration (loss of motor skills)
3. Hypsarrhythmia on EEG - Inter-icatal high voltage, slow-wave and spikes and sharp waves (basically just a really 'messy' pattern, that is present on EEG in between seizures that is indicative of chaotic neuronal firing.

50-70% will develop other seizure types

30

Describe the neuropsychological/cognitive features associated with the continuation of "West Syndrome".

West Syndrome is an epileptic encephalopathy (global brain dysfunction), the diffuse seizure activity and disrupted between seizure neuronal activity (hypsarrhythmia) is highly damaging to the brain and this is, therefore, one of the epileptic syndromes that results in a DECLINE in cognitive abilities (rather than just a delay). With deterioration of sensory, motor, and/or cognitive functions.

Prognosis is POOR. 71-80% will have an intellectual disability, severe in more than half the cases. Wide-ranging cognitive deficits and/or cognitive decline is commong.

50-70% will develop other seizure types.

31

How does a child/adolescent's epilepsy impact them in the home? and how does this further increase the negative impact of epilepsy on development?

- Child fails to meet parental expectations, e.g., grades drop and child is unable to explain why --> family conflict and psychological distress.

- Child's/adolescents activities and independence may be restricted for safety (e.g., no swimming, sleepover, school camps, contact sports AND require observation at bath time --> Increase isolation, reduced quality of life

Psychological distress and reduced quality of life has many flow on effects including:
- Depression and mood disturbances, reduced motivation, low self-esteem
- Poor social development
- Missing out on valuable experiences

32

How does a child/adolescent's epilepsy impact them at school? and how does this further increase the negative impact of epilepsy on development?

Slowed cognitive progression & regular school disruptions results in academic problems (e.g., falling behind class-mates). The child can become frustrated and they may develop a fear or dislike for school --- > truancy (non-attendance/avoidance) or behavioural issues ("acting out" and delinquency).

ALSO - May develop self-esteem or mood disturbances. May have trouble meeting parental expectations.

33

How does a child/adolescent's epilepsy impact them socially? and how does this further increase the negative impact of epilepsy on development?

Epilepsy/seizures are highly misunderstood and poorly studied. Lots of stigma and kids may get bullied or teased (e.g., "slow" "Stupid"), rejection by peers leads to feelings of isolation. This, combined with being disappointed with falling behind peers academically (and also possibly conflict at home) ---> reduced self-esteem --> depression, anxiety, and anger.

34

How is West Syndrome related to Lennox-Gastaut Syndrome?

Both are:
- Epileptic encephalopathies

Approximately 40% of Lennox-Gastaut epilepsy follow West Syndrome (Infantile Spasms).

35

__________ also known as _______ is an epileptic encephalopathy (global brain dysfunction) of varied aetiology (can be secondary to a brain abnormality or cryptogenic) with onset typically 3-12 months.

Associated with deterioration of sensory, motor, and/or cognitive functions due to epileptic activity, including frequent seizures and /or prominent interictal paroxysmal activity.

It is characterised by a triad of symptoms:
1. ____ spasms - clusters of sudden brief movements of the head, neck, and/or limbs.
2. Psychomotor deterioration (loss of motor skills)
3. Hypsarrhythmia on EEG - Inter-icatal high voltage, slow-wave and spikes and sharp waves (basically just a really 'messy' pattern, that is present on EEG in between seizures that is indicative of chaotic neuronal firing.

50-70% will develop other seizure types

West Syndrome; Infantile Spasms

36

What are the primary features of 'Lennox-Gastaut" Syndrome?
- Age of onset, type of epilepsy, aetiology, presenting symptoms.

Is a epileptic encephalopathy with an age of onset 1-8 years (peaking between ages 3-5), 40% of cases follow from West Syndrome and are considered 'symptomatic' (i.e., structural/metabolic); in 'unknown' (i.e., idiopathic) cases, development is typically normal prior to onset. More males than females are affected.

Multiple seizure types occur including: tonic, atonic, tonic-clonic, and absence. EEG is characterised by an abnormal, diffuse, slow spike-wave discharges.

As with West Syndrome (Infantile Spasms) (and where there is DIFFUSED SLOWING on EEG - cognitive impairment is usually severe.

37

What pattern on EEG is associated with the worst cognitive outcomes?

DIFFUSE SLOWING.

38

_________ is a epileptic encephalopathy with an age of onset 1-8 years (peaking between ages 3-5), 40% of cases follow from West Syndrome and are considered 'symptomatic' (i.e., structural/metabolic); in 'unknown' (i.e., idiopathic) cases, development is typically normal prior to onset. More males than females are affected.

Multiple seizure types occur including: tonic, atonic, tonic-clonic, and absence. EEG is characterised by an abnormal, diffuse, slow spike-wave discharges.

As with West Syndrome (i.e., Infantile Spasms) (and where there is DIFFUSED SLOWING on EEG) - cognitive impairment is usually severe.

Lennox-Gastaut Syndrome

39

___________ is a rare epileptic syndrome that is well studied due to it's unique effects on cognition and behaviour. Peak age of onset is between 5-7 years old and slightly more males than females are affected. Aetiology is symptomatic (Structural/metabolic) in 1/3 of cases and idiopathic ('unknown') elsewise and development is usually normal prior to onset.

By definition, EEG must show generalised (Diffuse) spike-wave discharges occupying 80% of slow wave sleep -- this is a form of 'electrical status epilepticus'. (EEG needs to be conducted during sleep).

Seizures are not easily controlled with AEDS - steroids can be used short-term with mixed effectiveness. A ketogenic diet may also help.

CSWS - Continous Spikes and Waves during Slow Wave Sleep

40

How is Llandau-Kleffner syndrome (LKS) related to CSWS? How is it distinguished

BOTH:
- Show Electrical stats epilepticus during slow wave sleep and there is debate whether they are separate syndromes or different variants.

ONLY Llandau-Kleffner also includes prominent epileptiform activity in the temporal or temporal-parietal regions. It has a primarily language presentation and is regarded as an acquired epileptiform aphasia.

41

What are the neuropsychological and cognitive sequelae of CSWS?

- Developmentally normal prior to onset
- Sudden and marked deterioration, particularly of behaviour (e.g., poor self-regulation, poor inhibition, but NOT defiant)

Reduced:
- global intellectual functioning
- language
- temporal spatial orientation
- motor function
- behaviour (temper outbursts, reduced frustration tolerance, short attention span, poor self-monitoring, disinhibition, perseveration)

Behaviour symptoms are often severe making neuropsych Ax difficulty.

Seizures often non-responsive to AEDS, steroids can be used short term and seizures may remit in adolescence. Steroids often result in improvement in behaviour and cognition. But significant executive and cog impairments likely to persist (part direct, part indirect due to missed development opportunity).

42

What is CSWS Syndrome (Continuous Spike-and-Waves during slow wave Sleep)?

A rare epileptic syndrome that is well studied due to it's unique effects on cognition and behaviour. Peak age of onset is between 5-7 years old and slightly more males than females are affected. Aetiology is symptomatic (Structural/metabolic) in 1/3 of cases and idiopathic ('unknown') elsewise and development is usually normal prior to onset.

By definition, EEG must show generalised (Diffuse) spike-wave discharges occupying 80% of slow wave sleep -- this is a form of 'electrical status epilepticus'. (EEG needs to be conducted during sleep).

Seizures are not easily controlled with AEDS - steroids can be used short-term with mixed effectiveness. A ketogenic diet may also help.

BUT prognosis is POOR, seizures can remit spontaneously in adolescence.

43

What does CSWS stand for?

Continuous Spike-and-Waves during slow wave Sleep

44

What are the primary features of 'Llandau-Kleffner Syndrome" Syndrome?
- Age of onset, type of epilepsy, aetiology, presenting symptoms.

Very rare - regarded as an acquired epileptiform aphasia - due to its effect on language.

Generally onset is after language acquisition but before 6 years (M=5 years). Strong male gender bias 2:1.

As in CSWS EEG shows electrical status eplilepticus during slow wave sleep. + prominent epileptiform activity in temporal or temporal-parietal regions. DESPITE abnormal EEG, 30% do not present with seizures.

45

What are the neuropsychological and cognitive symptoms of Llandau-Kleffner syndrome?

Regarded as an acquire epileptiform aphasia.

- Regression of language skills (both expressive and receptive), child may become mute.
- Also Auditory verbal agnosia (word deafness) followed by complete auditory agnosia in some.
- Behavioural abnormalities common, but more similar to autism (receptive, stereotyped movements)

Prognosis variable, seizures responsive to medication, and often spontaneously remit before age 15y but permanent language impairment common.
Better language outcome associated with earlier remission of electrical status. Steroids often used in the early stage.

46

______ is very rare and regarded as an acquired epileptiform aphasia - due to its effect on language.

Generally onset is after language acquisition but before 6 years (M=5 years). Strong male gender bias 2:1.

Llandau-Kleffner syndrome

47

What is the main take home message for "Benign Rolandic Epilepsy?

AKA benign childhood epilepsy with centro-temporal spikes

- it is the most common form
- there is not enough research on it
- but it probably isn't actually benign, subtle exec and language deficits.
- IQ deficits correlate with frequency of EEF spikes, not frequency of seizures, cognitive problems correlate with frequency of nocturnal spikes.

OTHER INFO ABOUT IT (was not covered in lecture)

- Related to CSWS
- Onset 3-13 years, peak at 8-10 years.
- Seizures: brief, simple, partial hemifacial (motor) seizures, usually in sleep or upon waking; often infrequent.
- Seizures can secondarily generalise
- EEG high voltage centrotemporal spikes followed by slow waves.
- Seizures and abnormal electrical activity usually resolve by mid-adolescence.

48

What are the primary features of 'Childhood Absence Epilepsy and Juvenile Abscence Epilepsy?
- Age of onset, type of epilepsy, aetiology, presenting symptoms.

Age of onset for CAE is 3year-puberty. Age of onset for JAE is puberty onwards.

Main features is frequent absence seizures which can occur multiple times/day and can go on to develop into tonic-clonic seizures.

Aetiology is presumed to be idiopathic ('unknown'), though some evidence of a genetic component. Disruption to the thalamocortical circuits are implicated.

EEG: bilateral synchronous 3hz spike and wave (the typical absence seizure pattern).

49

What are the neuropsychological and cognitive symptoms of CAE/JAE?

Good prognosis in that 80% of CAE cases remit. HOWEVER, 40% that do not remit will go on to develop Juvenile Myoclonic Epilepsy. Prognosis is worse with concomitant tonic-clonic seizures.

Despite typically good prognosis - psychosocial outcome is relatively poor - more likely to drop out of school, have drug/alcohol problems and have anxiety disorders than partial epilepsy and normal controls.

50

What are the primary features of 'Juvenile Myoclonic Epilepsy?
- Age of onset, type of epilepsy, aetiology, presenting symptoms.

MOST COMMON idiopathic (unknown cause) generalised epilepsy syndrome in adolescence (but limited literature on cog impairment)

Onset between 12-18 years, requires LIFELONG treatment.

Seizures are characterised by myoclonic jerks of head and neck; most also have tonic-clonic seizures. Up to 40% experience absence seizures. Seizures often well-controlled with medication (up to 90% of patients).

identified triggers include: sleep deprivation, stress, alcohol, illicit drug use. Photosensitivity is also common.

EEG: high amplitude generalised symmetrical 4-6Hz polyspike-wave complexes. (this is just for your own interest!).

51

Diagnose:
A 7YO child with a 6 week history of deteriorating speech and a 12 month history of trouble hearing/processing what others were saying. Prior normal development.

EEG continuous spike and long wave during sleep. (subclinical during sleep)











Llandau-Kleffner Syndrome

52

__________ it is the most common form
- there is not enough research on it
- but it probably isn't actually benign, subtle exec and language deficits.
- IQ deficits correlate with frequency of EEF spikes, not frequency of seizures, cognitive problems correlate with frequency of nocturnal spikes.

OTHER INFO ABOUT IT (was not covered in lecture)

- Related to CSWS
- Onset 3-13 years, peak at 8-10 years.
- Seizures: brief, simple, partial hemifacial (motor) seizures, usually in sleep or upon waking; often infrequent.
- Seizures can secondarily generalise
- EEG high voltage centrotemporal spikes followed by slow waves.
- Seizures and abnormal electrical activity usually resolve by mid-adolescence.

Benign Rolandic Epilepsy AKA benign childhood epilepsy with centro-temporal spikes

53

______ is the MOST COMMON idiopathic (unknown cause) generalised epilepsy syndrome in adolescence (but limited literature on cog impairment)

Onset between 12-18 years, requires LIFELONG treatment.

Seizures are characterised by myoclonic jerks of head and neck; most also have tonic-clonic seizures. Up to 40% experience absence seizures.

identified triggers include: sleep deprivation, stress, alcohol, illicit drug use. Photosensitivity is also common.

EEG: high amplitude generalised symmetrical 4-6Hz polyspike-wave complexes. (this is just for your own interest!).

Juvenile Myoclonic Epilepsy

54

Age of onset for ______ is 3year-puberty. Age of onset for _______ is puberty onwards.

Main features is frequent absence seizures which can occur multiple times/day and can go on to develop into tonic-clonic seizures.

Aetiology is presumed to be idiopathic ('unknown'), though some evidence of a genetic component. Disruption to the thalamocortical circuits are implicated.

EEG: bilateral synchronous 3hz spike and wave (the typical absence seizure pattern).

CAE - Childhood Absence Epilepsy
JAE - Juvenile Absence Epilepsy

55

What are the neuropsychological and cognitive symptoms of Juvenile Myoclonic Epilepsy?

Traditionally, cognitive functioning reported as normal. BUT more recent studies report cognitive dysfunction (particularly executive), including high levels of impairment in:

-concept formation
-speed of processing
-planning and organisation
-mental flexibility
-attention span
-Inhibitory control

Some reports of memory difficulties, but these may be attributable to executive deficits rather than memory dysfunction per se e.g.,
- difficulty with independent organisation of information
- difficulty with independent retrieval of information from memory
- reduced verbal fluency.

Mood disorders also reported to be at a slightly higher rate than general population - but not a defining feature.

56

Diagnose:
17 YO girl, referred due to academic concerns.

Onset at age 12 (year 7) with collapse one night at a disco, subsequent arm jerks in the mornings (infrequent). age 13, tonic-clonic seizure.

Impairments in WM, planning, organising information, mental flexibility, expressing & comprehending complex information.

Juvenile Myoclonic Epilepsy

57

Executive dysfunction in epilepsy often associated with....

- Absence seizures
- Generalised seizures
- Specific syndromes (JME, CSWS, BRE(?), FLE

58

Outline the pre-surgical work up prior to epilepsy surgical.

- Neurological exam (Hx and nature of seizures; neurological functioning)

- Neuroimaging:
---Telemetry (video-continous EEG)
---MRI (results often normal, can detect lesions or cortical abnormalities or WM changes);
---fMRI (less invasive than WADA for language lateralisation, but still new tech);
---SPECT (measures blood flow, usefull in identifying areas of reduced/increased flow - ictal & post-ictal. Timing of injection must be precise (tricky);
---PET (tends to be used in cases where localisation difficult e.g., abnormality unclear on MRI)

-Electro-cortical stimulation
-Neuropsychological assessment

59

What are the broad outcomes of epilepsy surgery in children?

- 50-80% of children will become seizure free
- Further 20% will have a significant reduction in seizures
- Surgery in children generally reported to result in better cognitive outcome than in adults
Earlier the surgery --> Better cognitive outcome than later (though not always)

BUT

- Long-term outcome of early vs delayed surgery not established
- Good quality childhood surgical outcome studies lacking.

60

Why is neuropsychology important in epilepsy treatment and pre-surgery?

- Helping with lateralisation and/or localisation i.e., identifying areas of deficit consistent with suspected epileptic-focus.
- Alerting medical team to possible -ve impact of surgery on post-op cognitive functioning.
-- e.g., martin LH boy, right temporal focus, terrible verbal memory, intact visual memory (so need to investigate laterality, suggests some reorganisation has already occured)

OR

--- e.g., Louise: RH girl, left temporal focus, superior intellectual and memory skills (verbal and visual), outstanding academics, with ambition to become journalist (so lets them know this is a very high functioning girl with still high level functioning).

61

Describe the nature of localisation in children.

Localisation of function similar to that in adults. E.g., Temporal lobe --> memory impairment. Frontal lobe --> Executive impairments.

62

Describe the nature of lateralisation in children.

Lateralistion of function MUCH LESS CLEAR CUT, especially:
- language
- verbal/non-verbal distinction in memory..