CHAPTER 16 EPILEPSY Flashcards
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
primary generalized epilepsies
This is the tonic phase of the seizure and lasts for
_______
10 to 20 s .
clonic jerking of the extended limbs (usually less
severe than those of a grand mal seizure) that occurs with _________
vasodepressor syncope or a Stokes-Adams hypotensive
attack.
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
“typical”
In many such patients, ________ for 2 to 3 min is an effective way of inducing absence attacks.
voluntary
hyperventilation
Cases of absence status have also been
described in adults with ________
frontal lobe epilepsy
and about _______of absence pts will at some time
have major generalized (tonic-clonic) convulsions
half
_________ is a
term that was introduced to describe long runs of slow
spike-and-wave activity, usually with no apparent loss
of consciousness.
Atypical absence
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.
Lennox-Gastaut syndrome
infantile spasms, a characteristic
high-amplitude chaotic EEG picture (“hypsarrhythmia”),
and an arrest in mental development
West syndrome
Conditions associted with West
Prematurity, perinatal injury and metabolic
diseases of infancy are the most common underlying
conditions.
_____________,
when occurring in isolation, is relatively benign
and usually responds well to medication
seizure-associated myoclonus
___________characterizes the group of juvenile
lipidosis, Lafora-type familial myoclonic epilepsy,
certain mitochondrial disorders, and other chronic familial degenerative diseases of undefined type
Chronic progressive polymyoclonus
with dementia
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.
JME
myoclonic jerks of the arm and upper trunk that is brought out with __________
fatigue, early stages of sleep, or alcohol ingestion.
EEG of JME
A linkage has been established to ______
4- to 6-Hz irregular polyspike activity.
chromosome 6
AEDS for JME
VA, Lev, LTG
MC origin of Frontal Lobe Seizures
SMA
Tonic elevation and extension of the
contralateral arm (“fencing posture”) and choreoathetotic
and dystonic postures have been associated with _____________
high medial frontal lesions
___________been recorded in proven
cases of temporal lobe disease and less often with lesions of the insula and parietal operculum;
Gustatory hallucinations
It has been noted that lesions on the
___________ (Brodmann areas 18
and 19) are likely to cause a sensation of twinkling or
pulsating light
lateral surface of the occipital lobe
_________usually located in the superoposterior
temporal region or the junction between parietal
and temporal lobes
Vertiginous sx
_____ 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
visceral sensations
gelastic seizures and precocious puberty has been traced __________
to a hamartoma of the hypothalamus.
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
______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,
Psych comorbids of MTS
Depression
Anxiety
Psychotic: 10%
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
anger, paranoia, and cosmologic or religious
conceptualizing are more characteristic of_______ temporal
lesions.
left
SUDEP has _______lifetime incidence
9%
pathophysiology of SUDEP
A postictal “shutdown”
of brainstem activity resulting in hypercapnia or hypoxemia
has been suggested.
The risk of sudden death is as high as ______ times greater for untreated patients
20
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
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
spikes are greatly accentuated by sleep
Epilepsy with Occipital Spikes
Benign Epilepsy of Childhood with
Centrotemporal Spikes
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
Infantile spasms may later progress to the _______a seizure disorder of early childhood of graver
prognosis
Lennox-Gastaut st;ndrome,
Sz evoked in certain individuals by a discrete physiologic or psychologic stimulus
Reflex epilepsy
MC type of reflex epilepsy
visual
____________________
(as well as many of the new antiepileptic drugs)
are all effective in controlling individual instances of
reflex epilepsy
Clonazepam, valproate, carbamazepine, and phenytoin
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
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
mild meningeal infiltration of inflammatory
cells and an encephalitic process marked by neuronal
destruction, gliosis,__________ some degree of
, and __________
neuronophagia,
tissue necrosis
perivascular cuffing
The finding of
antibodies to ____________in a proportion
of patients with Rasmussen encephalitis has raised
interest in an immune causation
glutamate receptors (GluR3)
conversion-hysterical disorder
Briquet disease
psychogenic states that generate pseudoseizures
panic disorder
dissociative disorders,
malingering
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
Levels of extracellular______ are elevated in glial scars near epileptic
foci, and a defect in _______ channels
has also been postulated.
potassium
voltage-sensitive calcium
A deficiency of the inhibitory neurotransmitter ____, increased glycine, decreased taurine, and either decreased or increased _____
GABA
glutamic acid
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
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
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
_______ 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.
that a small number of healthy persons________ show paroxysmal EEG abnormalities
(approximately 2 to 3 percent)
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
_________have been used to detect inferomedial temporal seizure activity,
Sphenoidal leads
increased _______ or restricted diffusion
in the hippocampi after a prolonged seizure or
status epilepticus
T2 signal
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
There is also a postictal
rise in ______and serum cortisol,
ACTH
a specific pattern of neuronal loss with
gliosis (sclerosis) in the ________ in mTS
hippocampal and amygdaloid
The associated histologic finding is loss of neurons in the ______of the pyramidal cell layer of the hippocampus,
CAl segment
Sommer sector
familial incidence of patients with epilepsy
5-10%
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
_________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
massive sudden myoclonic
jerk of head and arms leading to flexion or, less often, to extension of the body
infantile spasms, salaam spasms
______ is characterized by
an EEG picture of large bilateral slow waves and multifocal spikes (hypsarrhythmia)
West syndrome
resultant abnormality of a sodium channel protein
(SCNlA) and the manifestations have simply been
bought forward by the initial neonatal event.
Dravet
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
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
twitch-convulsive syndrome accompanies lupus erythematosus, seizures of undetermined
cause, or generalized neoplasia, one should
suspect its basis in________
renal failure.
most frequent of the metallic poisons that cause convulsions.
Lead and mercury
According to the extensive EURAP study; about ________ of epileptic women who become pregnant have no change in seizure frequency or severity
twothirds
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.
_______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
Phenytoin is excreted at
_______ percent of maternal serum concentration
15
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
lives. Because of the long half-lives of phenytoin, phenobarbital, and ethosuximide, these drugs need be taken only ______daily, preferably at bedtime
once
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
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
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
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
warfarin levels are__________
by the addition of phenobarbital or carbamazepine and
may be ________by phenytoin
decreased
inc
Enzyme-inducing drugs such as phenytoin, carbamazepine, and barbiturates can greatly increase the chance of breakthrough menstrual bleeding in women taking _______
oral contraceptives
The most common teratogenic effects have been_________but infrequently also a subtle facial
dysmorphism (“fetal anticonvulsant syndrome”),
cleft lip and cleft palate,
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
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
in utero exposure to
_________was associated with lower IQs (by 9 points)
compared to lamotrigine in children at the age of 4
valproate
major birth defects in the
fetuses exposed to lamotrigine during the first trimester
is just under_______
3 percent,
Polytherapy with lamotrigine and valproate raised
the estimate of risk to _____
12 percent.
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
The aromatic compounds
_________ are the ones most often responsible for skin eruptions
(phenytoin, carbamazepine, phenobarbital, prirnidone,
and lamotrigine)
_______ have been associated with an increased risk of these types of severe skin reactions, particularly those of Asian ancestry
(HLA-B*1502)
in patients who had been seizure-free during 2 years of treatment with a single drug, ______ relapsed after discontinuation of the drug,
one-third
JME pts should continue taking meds for _______
a lifetime
