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A - MSRA Paediatrics [15] > [34] Epilepsy > Flashcards

[34] Epilepsy Flashcards

1
Q

What is epilepsy?

A

A group of neurological disorders characterised by epileptic seizures

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2
Q

What are epileptic seizures?

A

Episodes that vary from brief, nearly undetectable periods, to long periods of vigorous shaking

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3
Q

What is a seizure?

A

A paroxysmal abnormality of motor, sensory, autonomic, and/or cognitive function due to transient brain dysfunction

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4
Q

What does the term ‘seizure’ include?

A
  • Epileptic
  • Syncopal (anoxic)
  • Brainstem
  • Emotional
  • Functional
  • Undetermined
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5
Q

What are the types of brainstem seizures?

A
  • Hydrocephalic

- Coning

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6
Q

What are functional seizures also known as?

A

Psychogenic pseudo-seizures

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7
Q

What makes a seizure epileptic?

A

The nature of the underlying electrical activity in the brain, especially the cerebral cortex

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8
Q

What is the problem with the thing that makes seizures epileptic being the nature of the underlying electrical activity in the brain?

A

They can be difficult to distinguish from a non-epileptic seizure clinically

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9
Q

What are epileptic seizures due to?

A

Excessive and hypersynchronous electrical activity, typically in neural networks in all or part of the cerebral cortex

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10
Q

When can an epilepsy generally be recognised?

A

After two or more unprovoked epileptic seizures

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11
Q

What is the incidence of epilepsy in childhood?

A

0.05%

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12
Q

How does the incidence of epilepsy in children compare to other age groups?

A

It affects infants and children more than any other age group

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13
Q

What are seizures, in terms of pathophysiology?

A

A paroxysmal manifestation of the electrical properties or the cerebral cortex

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14
Q

When does a seizure result?

A

When there is a sudden imbalance between the excitatory and inhibitor forces within the network of cortical neurones in favour of sudden-onset net excitation

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15
Q

What % of epilepsies are genetic?

A

70-80%

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16
Q

What is meant by ‘genetic’ epilepsies?

A

Idiopathic syndromes with complex inheritance

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17
Q

What are the other causes of epilepsy?

A
  • Cerebral dysgenesis/malformation
  • Cerebral vascular occlusion
  • Cerebral damage
  • Cerebral tumour
  • Neurodegenerative disorders
  • Neurocutaneous syndromes
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18
Q

Give two examples of causes of cerebral damage that can lead to epilepsy

A
  • Congential infection

- Hypoxic-ischaemic encephalopathy

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19
Q

Give an example of a neurocutaneous syndrome that can cause epilepsy

A

Tuberous sclerosis

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20
Q

What is the main risk factor for epilepsy?

A

Family history

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21
Q

What can epilepsies be broadly classified as?

A
  • Generalised

- Focal

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22
Q

When do focal seizures occur?

A

When the seizures arise from one or part of the hemisphere

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23
Q

What does the manifestation of focal seizures depend on?

A

The part of the brain where the discharge originates and moves too

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24
Q

What happen to the level of consciousness during focal seizures? `

A

May be retained or lost

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25
Q

How can a focal seizure progress?

A

May evolve to a generalised secondary tonic-clonic seizure

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26
Q

What region of the brain do frontal seizures affect?

A

The motor or pre-motor cortex

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27
Q

How might frontal seizures present?

A
  • Clonic movements, which may travel proximally (Jacksonian march)
  • Tonic seizure with both upper limbs raised high for several seconds
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28
Q

How might temporal seizures present?

A
  • Aura
  • Smell or taste abnormalities
  • Distortion of sound and shape
  • Automatisms, e.g. lip smacking, plucking at one’s clothing
  • Deja-vu
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29
Q

How might occipital seizures present?

A
  • Stereotyped visual hallucinations
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30
Q

How might parietal seizures present?

A
  • Contralateral dysesthesias

- Distorted body image

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31
Q

What is dysesthesias?

A

Altered sensation

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32
Q

What are generalised epileptic seizures?

A

Where discharges arise from both hemispheres

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33
Q

What are the types of generalised epileptic seizures?

A
  • Absence
  • Myoclonic
  • Tonic-clonic
  • Atonic
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34
Q

How do absence seizures present?

A

Transient loss of consciousness, with abrupt onset and termination unaccompanied by motor phenomena apart from some flickering of eyelids and minor alteration in muscle tone

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35
Q

What can absence seizures often be precipitated by?

A

Hyperventilation

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36
Q

How do myoclonic seizures present?

A

Brief, often repetitive jerking movements of the limbs, neck, or trunk

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37
Q

How do tonic seizures present?

A

Generalised increase in tone

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38
Q

How does the tonic phase of tonic-clonic seizures present?

A
  • May fall to ground

- Do not breath, become cyanosed

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39
Q

What might happen when the patient falls to the ground in tonic-clonic seizures?

A

May injure themselves

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40
Q

What happens in the clonic phase of tonic-clonic seizures?

A
  • Jerking of limbs
  • Breathing is irregular and cyanosis persists
  • Saliva may accumulate in mouth
  • Biting of tongue
  • Incontinence of urine
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41
Q

How long do tonic-clonic seizures last?

A

From a few seconds to minutes, followed by unconsciousness or deep sleep for up to several hours

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42
Q

How do atonic seizures present?

A

Transient loss of muscle tone, causing sudden fall to floor or drop of head

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43
Q

What are atonic seizures often preceded by?

A

Myoclonic jerk

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44
Q

What is a seizure pattern?

A

A pattern of epileptic seizures that has different clinical features and different ages of onset

45
Q

Give 7 seizure patterns seen in children

A
  • Infantile spasms (West syndrome)
  • Lennox-Gastaut syndrome
  • Childhood absence epilepsy
  • Benign rolandic epilepsy
  • Panaviotopolous syndrome
  • Juvenile absence epilepsy
  • Juvenile myoclonic epilepsy
46
Q

What is the age of onset of West syndrome?

A

3-12 months

47
Q

Describe the seizures in West syndrome

A

Violent flexor spasms of the head, trunk,and limbs, followed by extension of the arms
Lasts 1-2 seconds, often in multiple bursts of 20-30, often on walking or many times a day

48
Q

What is the age of onset of Lennox-Gastaut syndrome?

A

1-3 years

49
Q

Describe the seizures in Lennox-Gastaut syndrome?

A

There are multiple seizure types, but mostly atonic, atypical (subtle absences), and tonic seizures in sleep

50
Q

What other disorders are associated with Lennox-Gastaut syndrome?

A

Neurodevelopmental arrest or regression and behaviour disorder

51
Q

What is the age of onset of childhood absence epilepsy?

A

4-12 years

52
Q

Describe the seizures in childhood absence epilepsy

A

Momentary unresponsive state with motor arrest, may twitch their eyelids or hand/mouth minimally.
Only lasts a few seconds (<30s)

53
Q

Does the child recall the episodes in childhood absence epilepsy?

A

No, except realises they’ve missed something

54
Q

Does childhood absence epilepsy impact the child developmentally?

A

No, they are developmentally normal, but can interfere with schooling

55
Q

What is the age of onset of benign rolandic epilepsy?

A

4-10 years

56
Q

Describe the seizures in benign rolandic epilepsy

A

Tonic-clonic seizures in sleep, or simple focal seizures with awareness of abnormal feelings in tongue, and distoration of face

57
Q

What is the age of onset of panaviotopoulous syndrome?

A

1-5 years

58
Q

Describe the seizures in panaviotopoulous syndrome

A

Autonomic features with vomiting and unresponsive staring in sleep, with head-eye deviation, progressing sometimes to convulsive seizure

59
Q

What is the age of onset of juvenile absence epilepsy?

A

10-20 years

60
Q

Describe the seizures in juvenile absence epilepsy?

A

Absences and generalised tonic-clonic seizures, often with photosensitivity

61
Q

Is learning impaired in juvenile absence epilepsy?

A

No

62
Q

What is the age of onset of juvenile myoclonic epilepsy?

A

10-20 years

63
Q

Describe the seizures in juvenile myoclonic epilepsy

A

Myoclonic, generalised tonic-clonic, and absences may occur, mostly shortly after walking

64
Q

Is learning impaired in juvenile myoclonic epilepsy?

A

No

65
Q

What is the diagnosis of epilepsy based on?

A

Primarily on a detailed history from the child and eyewitnesses, sustained by video if available

66
Q

What should particular attention be paid to when taking a history for epilepsy?

A
  • Any specific triggers

- If the child has any impairment

67
Q

What investigations may help in epilepsy?

A
  • EEG
  • Neuroimaging
  • Blood and urine biochemistry
  • Neuropsychological assessment
  • ECG
68
Q

What is the purpose of EEG in epilepsy?

A

Should be performed only to support a diagnosis of epilepsy where the clinical history suggest a seizure is likely to be epileptic in origin

69
Q

What should the EEG in epilepsy involve?

A
  • Photic stimulation

- Hyperventilation

70
Q

What should be done when a standard EEG has not contributed to diagnosis or classification?

A

A sleep EEG should be performed

71
Q

How is a sleep EEG best performed in children and young people?

A

Sleep deprivation or the use of melatonin

72
Q

What does the EEG show in infantile spasms?

A

Hypsarrhythmia

73
Q

What does the EEG show in Lennox-Gastaut syndrome?

A

Slow generalised spike and wave (1-3Hz)

74
Q

What does EEG show in childhood absence epilepsy?

A

Fast generalised spike and wave (3-4Hz) discharges, bilaterally synchronous during and sometimes between absences

75
Q

What does the EEG show in benign rolandic epilepsy?

A

Focal sharp waves from rolandic area

76
Q

What does the EEG show in Panaviotopoulous syndrome?

A

Posterior focal sharp waves and occipital discharges when eyes are shut

77
Q

What is the purpose of neuroimaging in epilepsy?

A

Should be used to identify structural abnormalities that cause certain epilepsies

78
Q

What is the neuroimaging investigation of choice in epilepsy?

A

MRI

79
Q

What in the purpose of functional neuroimaging, e.g. PET and SPECT, in epilepsy?

A

Can detect areas of abnormal metabolism by using isotopes and ligands injected and taken up by metabolically active cells

80
Q

When is functional neuroimaging done in epilepsy?

A

Used in the workup for patients for possible epilepsy surgery

81
Q

When should investigations such as blood and urine biochemistry?

A

Should be done at discretion of specialist, to exclude other diagnoses and determine an underlying cause of epilepsy

82
Q

When should neuropsychological assessment be performed in epilepsy?

A

When it is important to evaluate learning disabilities and cognitive dysfunction, particularly in regard to language and memory

83
Q

Who is ECG recommended for in epilepsy?

A

All children with seizures, even when epilepsy is most likely

84
Q

What is the purpose of ECG is epilepsy?

A

To ensure there is no convulsive syncope to arrhythmia, e.g. long-QT syndrome

85
Q

What are the differentials for epilepsy?

A
  • Syncope
  • Night terrors
  • Reflex anoxic seizures
  • Febrile convulsions
  • Fabricated or induced illness by carers
  • Migraine
86
Q

What things should be considered when deciding on a drug management for epilepsy?

A
  • Not all children with epileptic seizures require anti-epileptic drug (AED) therapy
  • Choose an appropriate AED for the seizure and epilepsy
  • All AEDs have potential unwanted effects
87
Q

What should the decision as to whether a child needs AED therapy be made based on?

A
  • Seizure type
  • Epilepsy type
  • Frequency
  • Social and educational consequences of the seizures set against the possibility of unwanted effects of AED
88
Q

What is the desired goal of AED therapy?

A

Monotherapy at the minimum dosage, to prevent seizures without adverse effects

89
Q

Who should the potential unwanted effects of AED therapy be discussed with?

A

Child and parent

90
Q

When can AED therapy be discontinued?

A

After 2 years free of seizures, depending on the seizure syndrome

91
Q

What are the first line drug options for tonic-clonic, absence, and myoclonic seizures?

A
  • Valproate
  • Carbamazepine
  • Lamotrigine
92
Q

What are the first line drug options for focal seizure?

A
  • Carbamazepine
  • Valproate
  • Levetiracetam
  • Lamotrigine
93
Q

What are the second-line drug options for tonic-clonic and absence seizures?

A
  • Clobazam
  • Levetiracetam
  • Topiramate
94
Q

What are the second-line drug options in myoclonic seizures?

A
  • Clobazam

- Piracitam

95
Q

What are the second line drug options in focal seizures?

A
  • Clobazam
  • Topiramate
  • Gabapentin
  • Tigabine
96
Q

What other treatment options are there for children with intractable epilepsies?

A
  • Ketogenic diet
  • Vagal nerve stimulation
  • Surgery
97
Q

How is vagal nerve stimulation delivered?

A

Using an externally programmable stimulation of a wire implanted around the afferent (left) vagal nerve

98
Q

What is the purpose of surgery in epilepsy?

A

Cessation of seizures and AED therapy

99
Q

When can surgery be performed in epilepsy?

A

When epilepsy has a well-localised structural cause or epileptogenic zone

100
Q

How can a well-localised structural cause or epileptogenic zone be demonstrated?

A

Good concordance between ictal EEG, MRI, and functional imaging findings

101
Q

What is the main surgical procedure used in epilepsy?

A

Temporal lobectomy

102
Q

What is temporal lobectomy used for in epilepsy?

A

Mesial temporal sclerosis

103
Q

What other surgical procedures may be used in epilepsy?

A
  • Hemispherotomy

- Focal restrictions

104
Q

What is a hemispherotomy?

A

Disconnection of the hemisphere

105
Q

What biopsychosocial considerations are there when managing epilepsy?

A
  • Some children with epilepsy and their families need psychological help to adjust to the condition
  • School needs to be aware of the child’s problem, and teachers advised on the management of seizures
  • Situations where having a seizure could lead to injury or death should be avoided, e.g. swimming
106
Q

What biopsychosocial considerations are there specifically regarding adolescents when managing epilepsy?

A

Issues to discuss around driving, contraception, and pregnancy. May also be issues with concordance and the precipitation of seizures by alcohol and poor sleep routines

107
Q

How do the educational and social outcomes of epilepsy compare to those of other chronic illnesses such as diabetes?

A

They are worse

108
Q

What are the potential complications of epilepsy?

A
  • Status epilepticus

- SUDEP

A - MSRA Paediatrics [15] (115 decks)

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