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ADAMS > Epilepsy and other Seizure Disorders > Flashcards

Epilepsy and other Seizure Disorders Flashcards

1
Q

onset between 2-6 years of age
characterized by atonic/astatic seizures, succeeded by minor motor, tonic-clonic, partial seizures
progressive intellectual impairment in association with distinctive slow 1-2 Hz spike and wave EEG pattern

A

Lennox-Gastaut syndrome

often preced in early life by infantile spasms
characteristic high amplitude chaotic EEG picture (hypsarrhythmia)

persists in adult life and one of the most difficult forms of epilepsy to treat

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2
Q

Most common form of idiopathic generalized epilepsy in older children and young adults
begins in adolescence, approx 15 yrs

generalized tonic-clonic upon awakening, myoclonic jerks in the morning that involved the entire body
sometimes absence seziures are prominent
precipitating factors: fatigue, early stages of sleep, alcohol ingestion
EEG shows: characteristic bursts of 4-6 Hz irregular polyspike activity
does not impair intelligence and tends not to be progressive
Tc: Valproate, alternatives for pregnant women: levetiracetam, lamotrigine

A

Juvenile Myoclonic Epilepsy

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3
Q

true or false

Neonatal seizures occurring within 24 to 48 h of a difficult birth are usually indicative of severe cerebral damage, usually anoxic, either antenatal or parturitional. Such infants often succumb, and about half of the survivors are seriously handicapped

A

true

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4
Q

true or false
Seizures having their onset several days or weeks after birth are more often an expression of acquired or hereditary metabolic disease. In the latter group, hypoglycemia is the most frequent cause; another, hypocalcemia with tetany, has become infrequent

A

true

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5
Q

semiology corresponds to the regions of the brain affected

clonic contractions

A

motor cortex

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6
Q

semiology corresponds to the regions of the brain affected

contractions of the contralateral arm, face, neck, or all of one side of the body

A

premotor cortex BA 6

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7
Q

semiology corresponds to the regions of the brain affected

tonic elevation and extension of the contralateral arm (fencing posture) and choreoathetotic and dystonic postures

A

high medial frontal lesions BA 8 and supplementary motor cortex

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8
Q

semiology corresponds to the regions of the brain affected

olfactory hallucinations

A

inferior and medial parts of temporal lobe
region of the parahippocampal convolution or the uncus
unicinate seizures

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9
Q

semiology corresponds to the regions of the brain affected

gustatory hallucinations

A

temporal lobe lesions

insula and parietal operculum

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10
Q

semiology corresponds to the regions of the brain affected

visual seizures

A

lesions in or near the striate cortex of the occipital lobe

red>blue>green>yellow

also in anteromedial temporal and occipitotemporal regions

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11
Q

semiology corresponds to the regions of the brain affected

vertiginous sensations

A

superoposterior temporal region or junction between parietal and temporal lobes

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12
Q

intense perception of familiarity in an unfamiliar circumstance or place

A

deja vu

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13
Q

strangeness or unfamiliarity in a previously known place or circumstance

A

jamais vu

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14
Q

True or False

Volvular epilepsy, epilepsia procursiva, and promania are actually more common in temporal lobes than with frontal lobe foci of origin.

A

False

more common in frontal lobe
The patient may walk repetitively in small circles (valvular epilepsy), run (epilepsia procursiva), or simply wander aimlessly, either as an ictal or postictal phenomenon (poriomania). Theseforms of seizure, according to some epileptologists, are actually more common with frontal lobe than with temporal lobe foci of origin.

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15
Q

common focal motor epilepsy
self-limiting despite very abnormal EEG
Autosomal dominant begins 5-9 yrs of age
nocturnal tonic-clonic seizure, focal
then clonic contractions of the face, less often of one arm or leg
interictal EEG - high-voltage spikes in the contralateral lower rolandic or centrotemporal area
controlled by singe AED, disappear during adolescences

A

Benign Epilepsy of Childhood with Centrotemporal Spikes
Rolandic Epilepsy
Sylvian Epilepsy

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16
Q

dramatic form of epilepsy of infancy and early childhood
appears during the first year of life
recurrent, single or brief episodes of gross flexion movements of the trunk and limbs and less frequently by extension movements
(infantile spasms, salaam, jackknife seizures)
EEG: multifocal spikes and slow waves of large amplitude - hypsarrhythmia
child matures, the seizures diminish and disappears by the 4th-5th year
MRI: cortical dygeneses
may respond well to ACTH, corticosteroids, benzodiazepine drugs, clonazepam is most widely used

May later progress to Lennox-Gastaut syndrome

A

Infantile Spasms

or West Syndrome

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17
Q

specific to infants and children between 6 months and 5 years of age (peak incidence 9-20 months)
and with a strong inherited tendency
regarded as a benign condition
single, generalized motor seizure occurring at the patient’s core temperature rises or reaches its peak
temp usually above 38C

A

Febrile seizure

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18
Q

one of the most common illness to precipitate febrile seizure

A

herpesvirus 6

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19
Q

chronic focal encephalitis

intractable epilepsy in association with progressive hemiparesis

A

Rasmussen syndrome

cortex: mild meningeal infiltration of inflammatory cells and an encephalitic process marked by neuronal destruction, gliosis, neuronophagia, some degree of tissue necrosis, perivascular cuffing
neuropatho: extensive destruction of the cortex and white matter with intensive gliosis and lingering inflammatory reactions

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20
Q

finding of antibodies _____ in cases of Rasmussen encephalitis has raised interest in an immune causation

A

Glutamate receptors GluR3

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21
Q

repeated nonconvulsive electrical stimulation of normal cortex induces a permanent epileptic focus

A

kindling phenomenon

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22
Q

True or False

A small number of healthy persons, about 2-3%, show paroxysmal EEG abnormalities.

A

True

p 335

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23
Q

A singe EEG tracing obtained during the interictal state is abnormal to some degree in _____% of epileptic patients
this figure rises to ____% if patients are subjected to several recordings

A

30-50%

60-70%

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24
Q

True or False

The region of earliest spike activity corresponds best to the epileptogenic focus.

A

True

p335

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25
Q

Concentrations of serum ______ like those of hypothalamic hormones, rise 10-20 mins after all types of generalized seizures, including complex partial types, but not in absence and myoclonic types.

A

prolactin

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26
Q

True or False

Serum prolactin may also be elevated after a syncopal episode.

A

True

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27
Q

T/F

Seizures on awakening usually signify an generalized type, whereas those occurring during the period of sleep are more often focal in nature.

A

True

p322

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28
Q

EEG in absence seizures

A

3 hz spike-and-wave pattern

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29
Q

T/F

It is common to see absence seizure in patients less than 4 years old and after puberty.

A

False

rare
p323

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30
Q

EEG in JME

A

bursts of 4-6 Hz irregular polyspike activity

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31
Q

For Monogenic Epileptic Disorders
What protein/channels are affected for the ffg

Dravet syndrome

A

SCN1A

sodium channel a-subunit

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32
Q

For Monogenic Epileptic Disorders
What protein/channels are affected for the ffg

Benign Familial Neonatal Convulsions

A

SCN2A

sodium channel subunits

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33
Q

For Monogenic Epileptic Disorders
What protein/channels are affected for the ffg

Benign Infantile epilepsy

A

KCNQ2,3

potassium channels

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34
Q

For Monogenic Epileptic Disorders
What protein/channels are affected for the ffg

JME

A

GABRA1
CACNB4

GABA a receptor subunit
less often calcium channel

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35
Q

For Monogenic Epileptic Disorders
What protein/channels are affected for the ffg

Tuberous sclerosis

A

TSC1, 2

hamartin
tuberin

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36
Q

laughter as the most striking feature of a seizure

A

Gelastic epilepsy

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37
Q

combination of gelastic seizures and precocious puberty

A

hamartoma of the hypothalamus

38
Q

T/F

Diminished sexual interest and potency in men and menstrual problems in women, not readily attributable to antiepileptic drugs, are common among patients with complex partial seizures of temporal lobe origin.

A

True

p329

39
Q

increased mortality rate in healthy people with epilepsy is

A

unexpected death outside of circumstances such as drowning, trauma from a fall, myocardial infarction and automobile accidents during the seizure

40
Q 
SUDEP
Which of the following is NOT an emerging risks from population-based and cohort case controlled studies?
a. postictal period immediately after tonic clonic seziure
b. increasing seizure frequency
c. lack of successful treatment
d. subtherapeutic levels of AED
e. period of childhood
f. mental retardation
A

ANS: period of childhood

should be early adulthood
p. 330

41
Q

T/F

Most instances of SUDEP occur when the patient is awake.

A

FALSE

when unattended or during sleep

42
Q

Risk of SUDEP in untreated epilepsy patients

A

20x greater

43
Q

common focal motor epilepsy unique among the focal epilepsies of childhood that it is self-limiting despite a very abnormal EEG pattern

autosomal dominant trait
5-9 yrs old
nocturnal tonic-clonic seizure with focal onset

EEG: high voltage spikes in the contralateral lower rolandic or centrotemporal area

A

Benign Epilepsy of Childhood with Centrotemporal Spikes

Rolandic Epilepsy, Sylvian Epilepsy

44
Q

prognosis in Rolandic Epilepsy

A

seizures are readily controlled by a single anticonvulsant drug
gradually disappear during adolescence

45
Q

Epileptic syndrome

no intellectual deterioration
seizures cease in adolescence
associated with spike activity over the occipital lobes as identified by Panayiotopolous

VISUAL hallucinations are the most common clinical feature
sensations of movements of the eyes, tinnitus, vertigo are also reported

A

Epilepsy with Occipital Spikes

cause: mainly cortical heteroptropias
spikes are greatly accentuated by sleep

46
Q

T/F
Febrile seizures

Prophylatic antiepileptic drugs have been found to be helpful in preventing febrile seizures.

A

False

p331

47
Q

Most common reflex epilepsy

A

visually induced seizures

seizures are usually myoclonic but may be generalized and triggered by photic stimulation of television or an EEG examination or by the photic or pattern stimulation of video games

48
Q

highly restricted and very persistent focal motor status epilepticus

A

Epilepsia Partialis Continua

49
Q

T/F
Epilepsia Partialis Continua

The distal muscles of leg and arm especially the flexors of the hand and fingers are affected more frequently than proximal ones.

A

True

p331

50
Q

T/F

In pseudoseizures, the lids are open and may show clonic movement.

A

False
p333
In pseudoseizures, the eyes are kept quietly or forcefully closed, whereas the lids are open and show clonic movement in epilepsy.

51
Q

basis for Todd’s postepileptic paralysis

A

exhaustion (paralysis) of the neurons of the epileptogenic focus
regional increase in permeability of blood brain barrier and regional edema in MRI

52
Q

findings in Medial Temporal Sclerosis

A

loss of neurons in the CA1 segment of the pyramidal cell layer of the hippocampus
often unilateral

53
Q

Role of genetics in epilepsy

concordance rate in monozygotic twins, dizygotic twins

A

monozygotic: 60%
dizygotic: 13%

54
Q

Monogenic Epileptic Disorder
protein involved

Autosomal Dominant Nocturnal Frontal seizures

A

Nicotinic acetylcholine receptor subunits

55
Q

progressive myoclonic epilepsy

involved protein is cystatin B
protein tyrosine phosphatase, laforin

A

protein is cystatin B - Unverricht-Lundborg disease with PME

protein tyrosine phosphatase, laforin - Lafora body disease with PME

56
Q

T/F

Seizures having their onset several days or weeks after birth are more often an expression of an acquired or hereditary metabolic disease.

A

True
p339

hypoglycemia is the most frequent cause

57
Q 
infantile epilepsy
myoclonic and focal seizures
resistant epilepsy
learning disability, developmental delay
abnormality of Sodium channels (SCN1A)
A

Dravet syndrome

58
Q

T/F
Seizures during Pregnancy

According to EURAP, about 2/3 of epileptic women who became pregnant have increased severity and frequency of seizures.

A

FALSE

no change in frequency and severity
p343

59
Q

pregnant women on AED

the risks of teratogenic effects is highest in those taking

A

valproate

60
Q

pregnant women on AED

most common teratogenic effects

A

cleft lip and cleft palate

61
Q

In general the risk of major congenital defects is low; it increases to _____% in women taking AED during pregnancy in comparison to _____% in the overall population of pregnant women.

A

increase risk in pregnant on AED 4-5%

overall population of 2-3%

62
Q

polytherapy with lamotrigine and valproate during pregnancy has an estimate risk of _____ for developing teratogenic effects

A

12%

63
Q

most frequent idiosyncratic reactions to AEDs

A

rashes

64
Q

Idiosyncratic phenytoin hypersensitivity

A 
rashes
fever
lymphadenopathy
eosinophilia
blood dyscrasias
polyarteritis
65
Q

Prolonged used of phenytoin

A

hirsutism
hypertrophy of gums
coarsening of facial features

66
Q

overdose phenytoin

A

ataxia
diplopia
stupor

67
Q

rare idiosyncratic side effect of phenytoin

A

choreoathetosis

68
Q

Carbamazepine idiosyncratic reactions

A

mild leukopenia
pacytopenia
hyponatremia
diabetes insipidus

69
Q

Valproate side effects

A

weight gain in first months of tx
mesntrual irregularities
PCOS
rare: pancreatitis

tremor and slight bradykinesia

70
Q

interaction of valproate and lamotrigine

A

Increased serum level of LAMOTRIGINE

71
Q

MOA Lamotrigine

A

selectively blocks slow sodium channel

prevents release of excitatory transmitters: glutamate and aspartate

72
Q

MOA levetiracetam

A

affects SV2A synaptic vesicle protein

73
Q

Side effect Levetiracetam

A

dizziness and sleepiness
irritability
depression

74
Q

MOA Gabapentin

A

affects calcium channels

75
Q

MOA Vigabatrin

A

inhibits GABA transaminase

no longer use because of side effect: retinal damage

76
Q

MOA Tiagabine

A

inhibits GABA reuptake

77
Q

Topiramate Side effects

A

renal stones
angle closure glaucoma
hyperchloremic metabolic acidosis

78
Q

MOA Lacosamide

A

modulate voltage-gated sodium channel activity

79
Q

Lacosamide side effects

A

headache
diplopia
may prolong PR interval
worsen heart failure

80
Q

concurrent use of valproate and clonazepam produces

A

absence status

81
Q

overall mortality in Status Epilepticus

A

20-30%

82
Q

Maximum rate of infusion of valproate

A

3mg/kg per min

83
Q

rate of phenytoin IV infusion

A

<50mg/min

84
Q

Propofol syndrome

A

hypertriglyceridemia-associated pancreatitis

fatal shock and acidosis

85
Q

T/F

In general, higher serum concentrations of AEDs are necessary for the control of focal seizures than for generalized ones.

A

True

p346

86
Q

T/F
for AEDs
The usual blood level assay is of the total concentration of the drug, it is a precise reflection of the amount of drug entering the brain.

A

FALSE
p346

not a precise reflection
most AEDs have a large proportion that are bound to albumin and does not penetrate nervous tissue

87
Q

effect of VPA on metabolite of CBZ

A

VPA inhibits epoxide hydroxylase leads to buildup of CBZ epoxide

88
Q

Drug interaction

VPA on phenytoin and phenobarbital

A

accumulation of active PHT and of phenobarbitak by displacing them from serum proteins

89
Q

Effect of Pb and CBZ on warfarin

A

reduced levels of warfarin

90
Q

Effect of Phenytoin on Warfarin

A

Increased levels of warfarin

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