Epilepsy & Seizures

Question 1

What is deja vu?

Answer 1

Perception of familiarity with previously unfamiliar people or events

Question 2

Perception of unfamiliarity with previously familiar people or events

Answer 2

Deja entendu

Question 3

What is jamais entendu?

Answer 3

Perception of unfamiliarity with previously familiar auditory stimulus

Question 4

Perception of familiarity with previously unfamiliar auditory stimulus

Answer 4

jamai vu

Question 5

Neonatal Epilepsy Syndromes 5

Answer 5

Benign familial neonatal seizures
Early myoclonic encephalopathy
Ohtahara syndrome
Migrating partial seizures of infancy

Question 6

Infantile Epilepsy Syndromes
Mnemonic:
DraW-A-BEBE

Answer 6

West’s syndrome
Aicardi’s syndrome
Benign myoclonic epilepsy of infancy
Benign infantile seizures
Dravet syndrome

Question 7

Childhood Epilepsy Syndromes

Answer 7

Benign childhood epilepsy with centrotemporal spikes
Early-onset benign childhood occipital epilepsy
Late-onset childhood occipital epilepsy
Epilepsy with myoclonic absences
Myoclonic-astatic epilepsy of childhood
Lennox-Gastaut syndrome
Landau-Kleffner syndrome
Epilepsy with continuous spike and waves during slow wave
sleep
Childhood absence epilepsy
Progressive myoclonic epilepsies

Question 8

Also called “fifth-day fits”

Answer 8

Benign neonatal seizures

Question 9

Benign familial neonatal convulsions is an autosomal

dominant disorder caused by mutation of

Answer 9

KCNQ2 or KCNQ3

mutations impair potassium-dependent repolarization resulting in hyperexcitability

Question 10

West’s syndrome is a nonspecific diagnosis referring to

the triad of

Answer 10

1 infantile spasms
2 hypsarrhythmia
3 developmental arrest

Question 11

Aicardi’s syndrome is the X-linked triad of

Answer 11

1 infantile spasms
2 agenesis of corpus callosum
3 retinal malformations

Question 12

Early myoclonic encephalopathy is characterized by

Answer 12

newborn infants with migrant focal myoclonic epilepsy

and progressive psychomotor abnormalities

Question 13

Early myoclonic encephalopathy EEG findings

Answer 13

• Generalized or focal epileptiform discharges
• burst suppression which may evolve to hypsarrythmia later
• Myoclonus has no EEG counterpart

Question 14

Epilepsy Syndromes which show burst suppression on EEG

Answer 14

Early myoclonic encephalopathy

Ohthara Syndrome

Question 15

Epilepsy Syndromes which show hypsarrythmia

Answer 15

Infantile Spasms

West Syndrome

Question 16

How to differentiate Early myoclonic encephalopathy from Ohthara Syndrome?

Answer 16

no myoclonic seizures in Ohtahara syndrome

Question 17

Migrating partial seizures of infancy

Answer 17

Multifocal seizures, shift from hemisphere to hemisphere

Question 18

Pyridoxine (vitamin B6)-dependent seizures (congenital

dependency on pyridoxine) is said to be because of

Answer 18

diminished activity of glutamic acid decarboxylase (GAD); leading to increased glutamic acid

Question 19

Severe myoclonic epilepsy in infancy also known as

Answer 19

Dravet Syndrome

Question 20

Dravet syndrome

Answer 20

progressive myoclonic seizures (begin mild, worsen over time, partial seizures develop later

progressive neurologic deterioration that may be secondary to recurrent seizures

refractory to treatment

Question 21

benign rolandic epilepsy of childhood

Answer 21

Centrotemporal spikes on EEG

Question 22

Early-onset benign childhood occipital epilepsy also known as?

Answer 22

Panayiotopoulos syndrome

Question 23

Panayiotopoulos syndrome

Answer 23

Autonomic seizures and status epilepticus: commonly,
ictal vomiting, eye deviation; often progress to partial
clonic or generalized tonic-clonic seizures (often
nocturnal)
Visual seizures

Question 24

Panayiotopoulos syndrome EEG

Answer 24

bursts or trains of high-voltage rhythmic occipital 1 to 3 Hz spikes & spike-wave complexes, localized to uni or bilateral occipital, w normal background, increases during NREM sleep, disappears with eye opening

Question 25

Panayiotopoulos syndrome first line of treatment

Answer 25

carbamazepine

Question 26

AEDs that can worsen abscence seizure and why

Answer 26

Carbamazepine

Phenytoin

Question 27

AEDs that can worsen abscence seizure and why

Answer 27

Carbamazepine
Phenytoin

Absence seizures are driven by T-type calcium channels of the thalamus, which are promoted by GABAergic drugs
Therefore, GABAergic anticonvulsants may promote
absence seizures

Question 28

Rasmussen’s encephalitis pathogenesis

Answer 28

antibodies to GLUR3 (glutamate receptor-3)

Question 29

Progressive Myoclonic Epilepsies

Answer 29

Lafora body disease
Unverricht-Lundborg syndrome
Neuronal ceroid lipofuscinosis
Myoclonic epilepsy with ragged-red fibers
Sialidoses

Question 30

Autosomal dominant partial epilepsy with auditory
features is an example of a monogenic temporal lobe
epilepsy, caused by a mutation of the

Answer 30

LGI1 (leucine-rich,

glioma-inactivated 1) gene

Question 31

Features of a TYPICAL Absence Seizure

Adams

Answer 31

Rapid onset and offset
Typical 3 per second spike and wave
Complete loss of awareness

Question 32

What is ATYPICAL Absence Seizure

Adams

Answer 32

Long runs of SLOW spike and wave activity, usually with NO apparent loss of consciousness

Question 33

Most common underlying conditions of Lennox- Gestaut Syndrome (Adams)

Answer 33

Prematurity, perinatal injury and metabolic diseases of infancy

Question 34

Most common form of idiopathic generalized epilepsy in older children and young adults (Adams)

Answer 34

Juvenile Myoclonic Epilepsy

Question 35

EEG findings in JME

Answer 35

4- to 6- Hz irregular polyspike activity

Question 36

Focal seizures with sensory or motor features at the onset most often arise from the SENSORIMOTOR CORTEX.
Where does seizures with impairment of consciousness mostly arise from?

Answer 36

Limbic and Autonomic areas or in the Temporal Lobe

Question 37

The FENCING POSTURE in frontal lobe seizures have been associated with what specific area of the frontal lobe?

Answer 37

High medial frontal region corresponding to Area 8 and Supplementary Motor Cortex

Question 38

Olfactory hallucinations are associated with which area?

Answer 38

Inferior and medial parts of the temporal lobe (parahippocampal convolution) or Uncus (Uncinate Seizures)

Question 39

Gustatory hallucinations are associated with which area?

Answer 39

Temporal Lobe> Insula and Parietal operculum

Question 40

Vertiginous sensations are associated with which area?

Answer 40

Superoposterior Temporal region or junction between parietal and temporal lobes

Question 41

Particular combination seen in Hypothalamic Hamatoma?

Answer 41

Gelastic Seizures

Precocious Puberty

Question 42

What is the risk of sudden death among untreated patients with epilepsy?

Answer 42

as high as 20 times greater

Question 43

True or False: Benign Epilepsy of Childhood with Centrotemporal spikes is self- limited.

Answer 43

True.

Question 44

Common viral precipitant in Febrile Seizures probably due to its tendency to cause high fever

Answer 44

Herpesvirus 6

Question 45

True or False: Prophylactic AED has been found to be helpful in preventing Febrile Seizures

Answer 45

False