Seizures/Epileptic encephalopathies

Question 1

Define: Seizure

Answer 1

Neurologic dysfunction in which abnormal neuronal firing is manifest clinically

Question 2

Define: Epilepsy

Answer 2

Tendency towards recurrent seizures

Question 3

What is depolarisation caused by?

Answer 3

Inward Na & Ca influx

Question 4

What is repolarisation caused by?

Answer 4

Potassium channels

Question 5

Most common excitatory neurotransmitter in CNS?

Answer 5

Glutamate

Question 6

Most common inhibitory neurotransmitter in CNS?

Answer 6

GABA (gamma-aminobutyric acid)

Question 7

Name the 5 main types of generalised seizures?

Answer 7

Tonic
Clonic
Tonic-Clonic
Myoclonic
Absence

Question 8

If rapid sequence induction is required for prolonged Seizure activity what induction agent would you use and why?

Answer 8

Thiopentone

Anti-seizure activity

Question 9

Benign familial neonatal seizures are caused by what?

Answer 9

K+ Chanelopathy

Question 10

What day do pyridoxine dependent seizures usually present?

Answer 10

Day 1

Question 11

Pyridoxine is required for synthesis of which neurotransmitter?

Answer 11

GABA

Question 12

Dravet syndrome is also called?

Answer 12

Severe myoclonic epilepsy of infancy

Question 13

Which genetic mutation has been found to cause Dravet syndrome?

Answer 13

Na Channel mutation

SCN1A, SCN1B, SCN2A

Question 14

Co-mornidities and natural history of Dravet syndrome?

Answer 14

Developmental delay (apparent by 2yrs)
Language delay
Gradual appearance of ataxia and hyper-reflexia
Often refractory seizures

Question 15

West syndrome is characterised by what 3 findings?

Answer 15

Infantile spasms + hypsarrhythmia on EEG + developmental delay/regression

Question 16

Age of onset of benign occipital epilepsy?

Answer 16

5 - 7yrs

Question 17

EEG findings with benign occipital epilepsy?

Answer 17

Spikes in occipital region on eye closure

Question 18

Rolandic epilepsy accounts for what percentage of childhood epilepsies?

Answer 18

~15%

Question 19

What percentage of school age children will have changes in occipito-temporal region (with or without seizures)?

Answer 19

2%

Question 20

What type of Sz is found in Benign childhood epilepsy with centrotemporal spikes (Rolandic epilepsy)?

Answer 20

Partial Seizures

Most commonly face with drooling, oropharyngeal stimulation, mouth twitching, paraesthesia of gums

Question 21

Natural history of Rolandic epilepsy?

Answer 21

Most seizures resolve by 15yrs

Question 22

Panayiotopoulos syndrome is characterised by;
Age?
Seizure type?

Answer 22

Age: 3-6yrs
Seizure type: Seizures during sleep where autonomic symptoms predominant (vomiting, pallor, salivation)
Seizures infrequent (25% will only have 1 Sz)

Question 23

Characteristics of an absence seizure?

Answer 23

No aura
No post-ictal period
Brief
Many
Complete interruption/unawareness of surrounding events

Question 24

Landau-Kleffner syndrome is also called?

Answer 24

Acquired Epileptiform Aphasia

Question 25

EEG findings associated with Landau-Kleffner syndrome?

Answer 25

Diffuse slow wave activity

Question 26

Long term outcomes for Landau-Kleffner syndrome?

Answer 26

Seizures may regress, but long term speech impediment may remain

Question 27

Percentage of patients with family history of epilepsy with Juvenile Myoclonic Epilepsy?

Answer 27

~30%

Question 28

Typical seizure types and age of onset with JME?

Answer 28

Absence Sz: 7-13yrs
Myoclonic Jerks: 12 - 18yrs
GTCS: 13 - 20yrs

Question 29

Natural history of JME?

Answer 29

50% persist into adulthood, usu well controlled

Question 30

Incidence of infantile spasms?

Answer 30

25/100 000

Question 31

Peak age of onset of infantile spasms?

Answer 31

4 - 7mths

Question 32

EEG findings diagnostic for infantile spasms?

Answer 32

hypsarrhythmia

Question 33

Rule of 3’s for Febrile convulsions?’

% occurrence in children
% reoccurrence post initial febrile convulsion
% chance of epilepsy

Answer 33

Febrile seizures:
Occur in 3% of children
30% chance of reoccurrence
3% chance of epilepsy

Question 34

Outcomes with temporal lobe epilepsy?

Answer 34

30% full recovery
30% ongoing problems
30% totally dependent

Question 35

Define: Encephalopathy

Answer 35

Any diffuse process which alters brain structure or function

Question 36

What is Otahara syndrome?

Answer 36

Early infantile epileptic encephalopathy

Question 37

What is Rasmussen’s syndrome?

Answer 37

Rasmussen’s encephalitis: hemi-convulsions, hemi-plegia syndrome
- chronic progressive encephalitis with frequent focal seizures and progressive hemiplegia

Question 38

Management with a ketogenic diet aims to switch cerebral metabolism from glucose to what?

Answer 38

beta-hydroxybutyrate