CHAPTER 16 EPILEPSY Flashcards

1
Q

are a group of somewhat diverse,
age-dependent phenotypes that are characterized by generalized 2.5- to 4-Hz bifrontally predominant spikes or polyspike-and-slow-wave discharges that arise without underlying structural abnormalities.

genetic component underlies many
of these disorders

A

primary generalized epilepsies

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2
Q

This is the tonic phase of the seizure and lasts for

_______

A

10 to 20 s .

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3
Q

clonic jerking of the extended limbs (usually less

severe than those of a grand mal seizure) that occurs with _________

A

vasodepressor syncope or a Stokes-Adams hypotensive

attack.

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4
Q

Absence seizures are said to be________ if they have a

rapid onset and offset, typical three per second spike andwave, and complete loss of awareness

A

“typical”

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5
Q

In many such patients, ________ for 2 to 3 min is an effective way of inducing absence attacks.

A

voluntary

hyperventilation

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6
Q

Cases of absence status have also been

described in adults with ________

A

frontal lobe epilepsy

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7
Q

and about _______of absence pts will at some time

have major generalized (tonic-clonic) convulsions

A

half

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8
Q

_________ is a
term that was introduced to describe long runs of slow
spike-and-wave activity, usually with no apparent loss
of consciousness.

A

Atypical absence

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9
Q

onset between 2 and 6 years of age and
is characterized by atonic, or astatic, seizures (i.e., falling attacks), often succeeded by various combinations of minor motor, tonic-clonic, and partial seizures and by progressive intellectual impairment in association with a distinctive, slow (1- to 2-Hz) spike-and-wave EEG pattern.

A

Lennox-Gastaut syndrome

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10
Q

infantile spasms, a characteristic
high-amplitude chaotic EEG picture (“hypsarrhythmia”),
and an arrest in mental development

A

West syndrome

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11
Q

Conditions associted with West

A

Prematurity, perinatal injury and metabolic
diseases of infancy are the most common underlying
conditions.

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12
Q

_____________,
when occurring in isolation, is relatively benign
and usually responds well to medication

A

seizure-associated myoclonus

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13
Q

___________characterizes the group of juvenile
lipidosis, Lafora-type familial myoclonic epilepsy,
certain mitochondrial disorders, and other chronic familial degenerative diseases of undefined type

A

Chronic progressive polymyoclonus

with dementia

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14
Q

the most common form of idiopathic generalized
epilepsy in older children and young adults. It begins in
adolescence, typically around age 15 years, with a range that essentially spans all of the teenage years.

A

JME

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15
Q

myoclonic jerks of the arm and upper trunk that is brought out with __________

A

fatigue, early stages of sleep, or alcohol ingestion.

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16
Q

EEG of JME

A linkage has been established to ______

A

4- to 6-Hz irregular polyspike activity.

chromosome 6

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17
Q

AEDS for JME

A

VA, Lev, LTG

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18
Q

MC origin of Frontal Lobe Seizures

A

SMA

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19
Q

Tonic elevation and extension of the
contralateral arm (“fencing posture”) and choreoathetotic
and dystonic postures have been associated with _____________

A

high medial frontal lesions

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20
Q

___________been recorded in proven

cases of temporal lobe disease and less often with lesions of the insula and parietal operculum;

A

Gustatory hallucinations

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21
Q

It has been noted that lesions on the
___________ (Brodmann areas 18
and 19) are likely to cause a sensation of twinkling or
pulsating light

A

lateral surface of the occipital lobe

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22
Q

_________usually located in the superoposterior
temporal region or the junction between parietal
and temporal lobes

A

Vertiginous sx

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23
Q

_____ sensations come from temporal lobe, upper bank of the sylvian fissure, in the upper or
middle frontal gyrus, or in the medial frontal area near
the cingulate gyrus

A

visceral sensations

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24
Q

gelastic seizures and precocious puberty has been traced __________

A

to a hamartoma of the hypothalamus.

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25
43 percent displayed some of the motor changes; 32 percent, automatic behavior; and 25 percent, alterations in psychic function what is this triad
psycho-motor triad
26
______which is reported on video recording to occur in half of patients with temporal lobe seizures, is carried out by the hand ipsilateral to the seizure focus
Postictal | nose wiping,
27
Psych comorbids of MTS
Depression Anxiety Psychotic: 10%
28
The EEG during this period may show no seizure discharge, although this does not exclude repeated seizures in temporal lobe structures that are remote from the recording electrodes. This disorder, virtually indistinguishable from psychosis, may also present in the interictal period.
paranoid-delusional or amnesic psychosis
29
anger, paranoia, and cosmologic or religious conceptualizing are more characteristic of_______ temporal lesions.
left
30
SUDEP has _______lifetime incidence
9%
31
pathophysiology of SUDEP
A postictal "shutdown" of brainstem activity resulting in hypercapnia or hypoxemia has been suggested.
32
The risk of sudden death is as high as ______ times greater for untreated patients
20
33
common focal motor epilepsy is unique among the focal epilepsies of childhood in that it is self-limiting despite a very abnormal EEG pattern autosomal dominant trait and begins between 5 and 9 years of age.
BECTS
34
benign in the sense that there is no intellectual deterioration and the seizures often cease in adolescence, has been associated with spike activity over the occipital lobes
Epilepsy with Occipital Spikes
35
spikes are greatly accentuated by sleep
Epilepsy with Occipital Spikes Benign Epilepsy of Childhood with Centrotemporal Spikes
36
Both the seizures and the EEG abnormalities may respond dramatically to treatment with adrenocorticotropic hormone (ACTH), corticosteroids, or the benzodiazepine drugs, of which clonazepam is probably the most widely used.
Infantile spasms
37
Infantile spasms may later progress to the _______a seizure disorder of early childhood of graver prognosis
Lennox-Gastaut st;ndrome,
38
Sz evoked in certain individuals by a discrete physiologic or psychologic stimulus
Reflex epilepsy
39
MC type of reflex epilepsy
visual
40
____________________ (as well as many of the new antiepileptic drugs) are all effective in controlling individual instances of reflex epilepsy
Clonazepam, valproate, carbamazepine, and phenytoin
41
focal motor status epilepticus The distal muscles of the leg and arm, especially the flexors of the hand and fingers, are affected more frequently than the proximal ones
EPC
42
Epilepsia partialis continua is particularly common in patients with the rare condition, __________. The use of high doses of _________, when started within the first year of the disease, proved beneficia
Rasmussen encephalitis corticosteroids
43
mild meningeal infiltration of inflammatory cells and an encephalitic process marked by neuronal destruction, gliosis,__________ some degree of , and __________
neuronophagia, tissue necrosis perivascular cuffing
44
The finding of antibodies to ____________in a proportion of patients with Rasmussen encephalitis has raised interest in an immune causation
glutamate receptors (GluR3)
45
conversion-hysterical disorder
Briquet disease
46
psychogenic states that generate pseudoseizures
panic disorder dissociative disorders, malingering
47
Psychogenic spells are likely if attacks are ___________ (many minutes, even hours), if there is __________ (whereas apnea is typical during and after a seizure), or if there is tearfulness after an episode.
prolonged rapid breathing
48
Levels of extracellular______ are elevated in glial scars near epileptic foci, and a defect in _______ channels has also been postulated.
potassium voltage-sensitive calcium
49
A deficiency of the inhibitory neurotransmitter ____, increased glycine, decreased taurine, and either decreased or increased _____
GABA glutamic acid
50
seizures could be triggered either by a change in | _________ or a subtle alteration in the electrical activity in the region of a focal lesion
central thalamic rhythm generators
51
subcortical, thalamic, and brainstem centers corresponds to the _________of the seizure and to loss of consciousness as well as to the signs of autonomic nervous system overactivity
tonic phase
52
a ______ begins and intermittently interrupts the seizure discharge, changing it from the persistent tonic phase to the intermittent bursts of the clonic phase
diencephalic inhibition
53
_______ increase in glucose utilization during seizure discharges and suggested that the paralysis that follows might be a result of neuronal depletion of glucose and an increase in ______
two- to threefold lactic acid.
54
that a small number of healthy persons________ show paroxysmal EEG abnormalities
(approximately 2 to 3 percent)
55
A single EEG tracing obtained during the interictal state is abnormal to some degree in ______ percent of epileptic patients; this figure rises to ______ percent in several tracings
30 to 50 60 to 70
56
_________have been used to detect inferomedial temporal seizure activity,
Sphenoidal leads
57
increased _______ or restricted diffusion in the hippocampi after a prolonged seizure or status epilepticus
T2 signal
58
Concentrations of serum_________, like those of other hypothalamic hormones, rise for 10 to 20 min after all types of generalized seizures, including complex partial types, but not in absence or myoclonic types.
prolactin
59
There is also a postictal | rise in ______and serum cortisol,
ACTH
60
a specific pattern of neuronal loss with | gliosis (sclerosis) in the ________ in mTS
hippocampal and amygdaloid
61
The associated histologic finding is loss of neurons in the ______of the pyramidal cell layer of the hippocampus,
CAl segment | Sommer sector
62
familial incidence of patients with epilepsy
5-10%
63
Two forms of progressive myoclonic epilepsy, Unverricht-Lundborg disease and Lafora body disease, are the result, respectively, of mutations in genes encoding _________
cystatin B and tyrosine phosphatase
64
_________beginning on days 2 and 3, up to day 7, ("fifth day seizures") in which there were no specific EEG changes
benign neonatal clonic convulsions
65
massive sudden myoclonic | jerk of head and arms leading to flexion or, less often, to extension of the body
infantile spasms, salaam spasms
66
______ is characterized by | an EEG picture of large bilateral slow waves and multifocal spikes (hypsarrhythmia)
West syndrome
67
resultant abnormality of a sodium channel protein (SCNlA) and the manifestations have simply been bought forward by the initial neonatal event.
Dravet
68
it reveals a paroxysmal 2- to 2.5-per-second spike-and-wave pattern on a background of predominant 4- to 7-Hz slow waves.
LGS
69
In Idiopathic Generalized Epilepsy, the risk of another seizure over 10 years was________percent unless the first episode was status epilepticus, in which case the risk was ______ percent.
13 41
70
twitch-convulsive syndrome accompanies lupus erythematosus, seizures of undetermined cause, or generalized neoplasia, one should suspect its basis in________
renal failure.
71
most frequent of the metallic poisons that cause convulsions.
Lead and mercury
72
According to the extensive EURAP study; about ________ of epileptic women who become pregnant have no change in seizure frequency or severity
twothirds
73
Tx of coagulopathy from Pb
oral administration of vitamin K, 20 mg/ d during the eighth month or 10 mg IV 4 h before birth and 1 mg 1M to the neonate.
74
_______in human milk is found to be 40 percent of the mother 's serum concentration which results in a neonatal blood level that is below the conventionally detectable amount
carbamazepine
75
Phenytoin is excreted at | _______ percent of maternal serum concentration
15
76
In approximately _______ percent of all patients with epilepsy, the seizures are controlled completely or almost completely by medications; in an additional______percent, the attacks are significantly reduced in number and severity
70 20 to 25
77
lives. Because of the long half-lives of phenytoin, phenobarbital, and ethosuximide, these drugs need be taken only ______daily, preferably at bedtime
once
78
avoid drugs combinations with similar putative mechanisms because their side effects may be additive, for example, the addition of _____ to carbamazepine or of _______ to carbamazepine
lamotrigine phenytoin
79
the clinician should be aware of known interactions through metabolic pathways such as ______combined with either lamotrigine or phenobarbital as they share the cytochrome P450 degradation pathway
valproate
80
interactions between one anticonvulsant and the metabolites of another, as, for example, the inhibition of epoxide hydrolase by _________, leading to toxicity through the buildup of carbamazepine epoxide.
valproic acid
81
Agents that alter the concentrations of antiepileptic medications are ______ which causes the accumulation of phenytoin and phenobarbital, and _______, which causes the accumulation of carbamazepine.
chloramphenicol, erythromycin
82
warfarin levels are__________ by the addition of phenobarbital or carbamazepine and may be ________by phenytoin
decreased inc
83
Enzyme-inducing drugs such as phenytoin, carbamazepine, and barbiturates can greatly increase the chance of breakthrough menstrual bleeding in women taking _______
oral contraceptives
84
The most common teratogenic effects have been_________but infrequently also a subtle facial dysmorphism ("fetal anticonvulsant syndrome"),
cleft lip and cleft palate,
85
In general, the risk of major congenital defects is low; it increases to_________in women taking anticonvulsant drugs during pregnancy, in comparison to________percent in the overall population of pregnant women.
4 to 5 percent 2 to 3
86
When all types of malformations were included, both major and minor, _______ of infants born to mothers who took anticonvulsants during pregnancy showed abnormalities, compared to 9 percent of mothers who had not taken medications
20 percent
87
in utero exposure to _________was associated with lower IQs (by 9 points) compared to lamotrigine in children at the age of 4
valproate
88
major birth defects in the fetuses exposed to lamotrigine during the first trimester is just under_______
3 percent,
89
Polytherapy with lamotrigine and valproate raised | the estimate of risk to _____
12 percent.
90
If a woman with seizure disorder has been off epilepsy medications for a time before getting pregnant and seizes during the pregnancy, the best choice of medication currently may be ________for its advantage in rapid seizure control, or levetiracetam
phenytoin
91
The aromatic compounds | _________ are the ones most often responsible for skin eruptions
(phenytoin, carbamazepine, phenobarbital, prirnidone, | and lamotrigine)
92
_______ have been associated with an increased risk of these types of severe skin reactions, particularly those of Asian ancestry
(HLA-B*1502)
93
in patients who had been seizure-free during 2 years of treatment with a single drug, ______ relapsed after discontinuation of the drug,
one-third
94
JME pts should continue taking meds for _______
a lifetime