[34] Epilepsy Flashcards
1
Q
What is epilepsy?
A
A group of neurological disorders characterised by epileptic seizures
2
Q
What physically happens in epileptic seizures?
A
Episodes that vary from brief, nearly undetectable periods, to long periods of vigorous shaking
3
Q
What is a seizure?
A
A paroxysmal abnormality of motor, sensory, autonomic, and/or cognitive function due to transient brain dysfunction
4
Q
What does the term ‘seizure’ include?
A
- Epileptic
- Syncopal (anoxic)
- Brainstem
- Emotional
- Functional
- Undetermined
5
Q
What are the types of brainstem seizures?
A
- Hydrocephalic
- Coning
6
Q
What are functional seizures also known as?
A
Psychogenic pseudo-seizures
7
Q
What makes a seizure epileptic?
A
The nature of the underlying electrical activity in the brain, especially the cerebral cortex
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
9
Q
What are epileptic seizures due to (brain activity)?
A
Excessive and hypersynchronous electrical activity, typically in neural networks in all or part of the cerebral cortex
10
Q
When can an epilepsy generally be recognised?
A
After two or more unprovoked epileptic seizures
11
Q
What is the incidence of epilepsy in childhood?
A
0.05%
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
13
Q
What are seizures, in terms of pathophysiology?
A
A paroxysmal manifestation of the electrical properties or the cerebral cortex
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
15
Q
What % of epilepsies are genetic?
A
70-80%
16
Q
What is meant by ‘genetic’ epilepsies?
A
Idiopathic syndromes with complex inheritance
17
Q
What are the other causes of epilepsy?
A
- Cerebral dysgenesis/malformation
- Cerebral vascular occlusion
- Cerebral damage
- Cerebral tumour
- Neurodegenerative disorders
- Neurocutaneous syndromes
18
Q
Give two examples of causes of cerebral damage that can lead to epilepsy
A
- Congential infection
- Hypoxic-ischaemic encephalopathy
19
Q
Give an example of a neurocutaneous syndrome that can cause epilepsy
A
Tuberous sclerosis
20
Q
What is the main risk factor for epilepsy?
A
Family history
21
Q
What can epilepsies be broadly classified as?
A
- Generalised
- Focal
22
Q
When do focal seizures occur?
A
When the seizures arise from one or part of the hemisphere
23
Q
What does the manifestation of focal seizures depend on?
A
The part of the brain where the discharge originates and moves too
24
Q
What happen to the level of consciousness during focal seizures? `
A
May be retained or lost
25
How can a focal seizure progress?
May evolve to a generalised secondary tonic-clonic seizure
26
What region of the brain do frontal seizures affect?
The motor or pre-motor cortex
27
How might frontal seizures present?
- Clonic movements, which may travel proximally (Jacksonian march)
- Tonic seizure with both upper limbs raised high for several seconds
28
How might temporal seizures present?
- Aura
- Smell or taste abnormalities
- Distortion of sound and shape
- Automatisms, e.g. lip smacking, plucking at one’s clothing
- Deja-vu
29
How might occipital seizures present?
- Stereotyped visual hallucinations
30
How might parietal seizures present?
- Contralateral dysesthesias
| - Distorted body image
31
What is dysesthesias?
Altered sensation
32
What are generalised epileptic seizures?
Where discharges arise from both hemispheres
33
What are the types of generalised epileptic seizures?
- Absence
- Myoclonic
- Tonic-clonic
- Atonic
34
How do absence seizures present?
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
35
What can absence seizures often be precipitated by?
Hyperventilation
36
How do myoclonic seizures present?
Brief, often repetitive jerking movements of the limbs, neck, or trunk
37
How do tonic seizures present?
Generalised increase in tone
38
How does the tonic phase of tonic-clonic seizures present?
- May fall to ground
| - Do not breath, become cyanosed
39
What might happen when the patient falls to the ground in tonic-clonic seizures?
May injure themselves
40
What happens in the clonic phase of tonic-clonic seizures?
- Jerking of limbs
- Breathing is irregular and cyanosis persists
- Saliva may accumulate in mouth
- Biting of tongue
- Incontinence of urine
41
How long do tonic-clonic seizures last?
From a few seconds to minutes, followed by unconsciousness or deep sleep for up to several hours
42
How do atonic seizures present?
Transient loss of muscle tone, causing sudden fall to floor or drop of head
43
What are atonic seizures often preceded by?
Myoclonic jerk
44
What is a seizure pattern?
A pattern of epileptic seizures that has different clinical features and different ages of onset
45
Give 7 seizure patterns seen in children
- Infantile spasms (West syndrome)
- Lennox-Gastaut syndrome
- Childhood absence epilepsy
- Benign rolandic epilepsy
- Panaviotopolous syndrome
- Juvenile absence epilepsy
- Juvenile myoclonic epilepsy
46
What is the age of onset of West syndrome?
3-12 months
47
Describe the seizures in West syndrome
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
What is the age of onset of Lennox-Gastaut syndrome?
1-3 years
49
Describe the seizures in Lennox-Gastaut syndrome?
There are multiple seizure types, but mostly atonic, atypical (subtle absences), and tonic seizures in sleep
50
What other disorders are associated with Lennox-Gastaut syndrome?
Neurodevelopmental arrest or regression and behaviour disorder
51
What is the age of onset of childhood absence epilepsy?
4-12 years
52
Describe the seizures in childhood absence epilepsy
Momentary unresponsive state with motor arrest, may twitch their eyelids or hand/mouth minimally.
Only lasts a few seconds (<30s)
53
Does the child recall the episodes in childhood absence epilepsy?
No, except realises they’ve missed something
54
Does childhood absence epilepsy impact the child developmentally?
No, they are developmentally normal, but can interfere with schooling
55
What is the age of onset of benign rolandic epilepsy?
4-10 years
56
Describe the seizures in benign rolandic epilepsy
Tonic-clonic seizures in sleep, or simple focal seizures with awareness of abnormal feelings in tongue, and distoration of face
57
What is the age of onset of panaviotopoulous syndrome?
1-5 years
58
Describe the seizures in panaviotopoulous syndrome
Autonomic features with vomiting and unresponsive staring in sleep, with head-eye deviation, progressing sometimes to convulsive seizure
59
What is the age of onset of juvenile absence epilepsy?
10-20 years
60
Describe the seizures in juvenile absence epilepsy?
Absences and generalised tonic-clonic seizures, often with photosensitivity
61
Is learning impaired in juvenile absence epilepsy?
No
62
What is the age of onset of juvenile myoclonic epilepsy?
10-20 years
63
Describe the seizures in juvenile myoclonic epilepsy
Myoclonic, generalised tonic-clonic, and absences may occur, mostly shortly after walking
64
Is learning impaired in juvenile myoclonic epilepsy?
No
65
What is the diagnosis of epilepsy based on?
Primarily on a detailed history from the child and eyewitnesses, sustained by video if available
66
What should particular attention be paid to when taking a history for epilepsy?
- Any specific triggers
| - If the child has any impairment
67
What investigations may help in epilepsy?
- EEG
- Neuroimaging
- Blood and urine biochemistry
- Neuropsychological assessment
- ECG
68
What is the purpose of EEG in epilepsy?
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
What should the EEG in epilepsy involve?
- Photic stimulation
| - Hyperventilation
70
What should be done when a standard EEG has not contributed to diagnosis or classification?
A sleep EEG should be performed
71
How is a sleep EEG best performed in children and young people?
Sleep deprivation or the use of melatonin
72
What does the EEG show in infantile spasms?
Hypsarrhythmia
73
What does the EEG show in Lennox-Gastaut syndrome?
Slow generalised spike and wave (1-3Hz)
74
What does EEG show in childhood absence epilepsy?
Fast generalised spike and wave (3-4Hz) discharges, bilaterally synchronous during and sometimes between absences
75
What does the EEG show in benign rolandic epilepsy?
Focal sharp waves from rolandic area
76
What does the EEG show in Panaviotopoulous syndrome?
Posterior focal sharp waves and occipital discharges when eyes are shut
77
What is the purpose of neuroimaging in epilepsy?
Should be used to identify structural abnormalities that cause certain epilepsies
78
What is the neuroimaging investigation of choice in epilepsy?
MRI
79
What in the purpose of functional neuroimaging, e.g. PET and SPECT, in epilepsy?
Can detect areas of abnormal metabolism by using isotopes and ligands injected and taken up by metabolically active cells
80
When is functional neuroimaging done in epilepsy?
Used in the workup for patients for possible epilepsy surgery
81
When should investigations such as blood and urine biochemistry?
Should be done at discretion of specialist, to exclude other diagnoses and determine an underlying cause of epilepsy
82
When should neuropsychological assessment be performed in epilepsy?
When it is important to evaluate learning disabilities and cognitive dysfunction, particularly in regard to language and memory
83
Who is ECG recommended for in epilepsy?
All children with seizures, even when epilepsy is most likely
84
What is the purpose of ECG is epilepsy?
To ensure there is no convulsive syncope to arrhythmia, e.g. long-QT syndrome
85
What are the differentials for epilepsy?
- Syncope
- Night terrors
- Reflex anoxic seizures
- Febrile convulsions
- Fabricated or induced illness by carers
- Migraine
86
What things should be considered when deciding on a drug management for epilepsy?
- 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
What should the decision as to whether a child needs AED therapy be made based on?
- Seizure type
- Epilepsy type
- Frequency
- Social and educational consequences of the seizures set against the possibility of unwanted effects of AED
88
What is the desired goal of AED therapy?
Monotherapy at the minimum dosage, to prevent seizures without adverse effects
89
Who should the potential unwanted effects of AED therapy be discussed with?
Child and parent
90
When can AED therapy be discontinued?
After 2 years free of seizures, depending on the seizure syndrome
91
What are the first line drug options for tonic-clonic, absence, and myoclonic seizures?
- Valproate
- Carbamazepine
- Lamotrigine
92
What are the first line drug options for focal seizure?
- Carbamazepine
- Valproate
- Levetiracetam
- Lamotrigine
93
What are the second-line drug options for tonic-clonic and absence seizures?
- Clobazam
- Levetiracetam
- Topiramate
94
What are the second-line drug options in myoclonic seizures?
- Clobazam
| - Piracitam
95
What are the second line drug options in focal seizures?
- Clobazam
- Topiramate
- Gabapentin
- Tigabine
96
What other treatment options are there for children with intractable epilepsies?
- Ketogenic diet
- Vagal nerve stimulation
- Surgery
97
How is vagal nerve stimulation delivered?
Using an externally programmable stimulation of a wire implanted around the afferent (left) vagal nerve
98
What is the purpose of surgery in epilepsy?
Cessation of seizures and AED therapy
99
When can surgery be performed in epilepsy?
When epilepsy has a well-localised structural cause or epileptogenic zone
100
How can a well-localised structural cause or epileptogenic zone be demonstrated?
Good concordance between ictal EEG, MRI, and functional imaging findings
101
What is the main surgical procedure used in epilepsy?
Temporal lobectomy
102
What is temporal lobectomy used for in epilepsy?
Mesial temporal sclerosis
103
What other surgical procedures may be used in epilepsy?
- Hemispherotomy
| - Focal restrictions
104
What is a hemispherotomy?
Disconnection of the hemisphere
105
What biopsychosocial considerations are there when managing epilepsy?
- 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
What biopsychosocial considerations are there specifically regarding adolescents when managing epilepsy?
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
How do the educational and social outcomes of epilepsy compare to those of other chronic illnesses such as diabetes?
They are worse
108
What are the potential complications of epilepsy?
- Status epilepticus
| - SUDEP
