Epilepsy and other Seizure Disorders Flashcards
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
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
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
Juvenile Myoclonic Epilepsy
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
true
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
true
semiology corresponds to the regions of the brain affected
clonic contractions
motor cortex
semiology corresponds to the regions of the brain affected
contractions of the contralateral arm, face, neck, or all of one side of the body
premotor cortex BA 6
semiology corresponds to the regions of the brain affected
tonic elevation and extension of the contralateral arm (fencing posture) and choreoathetotic and dystonic postures
high medial frontal lesions BA 8 and supplementary motor cortex
semiology corresponds to the regions of the brain affected
olfactory hallucinations
inferior and medial parts of temporal lobe
region of the parahippocampal convolution or the uncus
unicinate seizures
semiology corresponds to the regions of the brain affected
gustatory hallucinations
temporal lobe lesions
insula and parietal operculum
semiology corresponds to the regions of the brain affected
visual seizures
lesions in or near the striate cortex of the occipital lobe
red>blue>green>yellow
also in anteromedial temporal and occipitotemporal regions
semiology corresponds to the regions of the brain affected
vertiginous sensations
superoposterior temporal region or junction between parietal and temporal lobes
intense perception of familiarity in an unfamiliar circumstance or place
deja vu
strangeness or unfamiliarity in a previously known place or circumstance
jamais vu
True or False
Volvular epilepsy, epilepsia procursiva, and promania are actually more common in temporal lobes than with frontal lobe foci of origin.
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.
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
Benign Epilepsy of Childhood with Centrotemporal Spikes
Rolandic Epilepsy
Sylvian Epilepsy
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
Infantile Spasms
or West Syndrome
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
Febrile seizure
one of the most common illness to precipitate febrile seizure
herpesvirus 6
chronic focal encephalitis
intractable epilepsy in association with progressive hemiparesis
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
finding of antibodies _____ in cases of Rasmussen encephalitis has raised interest in an immune causation
Glutamate receptors GluR3
repeated nonconvulsive electrical stimulation of normal cortex induces a permanent epileptic focus
kindling phenomenon
True or False
A small number of healthy persons, about 2-3%, show paroxysmal EEG abnormalities.
True
p 335
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
30-50%
60-70%
True or False
The region of earliest spike activity corresponds best to the epileptogenic focus.
True
p335
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.
prolactin
True or False
Serum prolactin may also be elevated after a syncopal episode.
True
T/F
Seizures on awakening usually signify an generalized type, whereas those occurring during the period of sleep are more often focal in nature.
True
p322
EEG in absence seizures
3 hz spike-and-wave pattern
T/F
It is common to see absence seizure in patients less than 4 years old and after puberty.
False
rare
p323
EEG in JME
bursts of 4-6 Hz irregular polyspike activity
For Monogenic Epileptic Disorders
What protein/channels are affected for the ffg
Dravet syndrome
SCN1A
sodium channel a-subunit
For Monogenic Epileptic Disorders
What protein/channels are affected for the ffg
Benign Familial Neonatal Convulsions
SCN2A
sodium channel subunits
For Monogenic Epileptic Disorders
What protein/channels are affected for the ffg
Benign Infantile epilepsy
KCNQ2,3
potassium channels
For Monogenic Epileptic Disorders
What protein/channels are affected for the ffg
JME
GABRA1
CACNB4
GABA a receptor subunit
less often calcium channel
For Monogenic Epileptic Disorders
What protein/channels are affected for the ffg
Tuberous sclerosis
TSC1, 2
hamartin
tuberin
laughter as the most striking feature of a seizure
Gelastic epilepsy
combination of gelastic seizures and precocious puberty
hamartoma of the hypothalamus
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.
True
p329
increased mortality rate in healthy people with epilepsy is
unexpected death outside of circumstances such as drowning, trauma from a fall, myocardial infarction and automobile accidents during the seizure
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
ANS: period of childhood
should be early adulthood
p. 330
T/F
Most instances of SUDEP occur when the patient is awake.
FALSE
when unattended or during sleep
Risk of SUDEP in untreated epilepsy patients
20x greater
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
Benign Epilepsy of Childhood with Centrotemporal Spikes
Rolandic Epilepsy, Sylvian Epilepsy
prognosis in Rolandic Epilepsy
seizures are readily controlled by a single anticonvulsant drug
gradually disappear during adolescence
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
Epilepsy with Occipital Spikes
cause: mainly cortical heteroptropias
spikes are greatly accentuated by sleep
T/F
Febrile seizures
Prophylatic antiepileptic drugs have been found to be helpful in preventing febrile seizures.
False
p331
Most common reflex epilepsy
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
highly restricted and very persistent focal motor status epilepticus
Epilepsia Partialis Continua
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.
True
p331
T/F
In pseudoseizures, the lids are open and may show clonic movement.
False
p333
In pseudoseizures, the eyes are kept quietly or forcefully closed, whereas the lids are open and show clonic movement in epilepsy.
basis for Todd’s postepileptic paralysis
exhaustion (paralysis) of the neurons of the epileptogenic focus
regional increase in permeability of blood brain barrier and regional edema in MRI
findings in Medial Temporal Sclerosis
loss of neurons in the CA1 segment of the pyramidal cell layer of the hippocampus
often unilateral
Role of genetics in epilepsy
concordance rate in monozygotic twins, dizygotic twins
monozygotic: 60%
dizygotic: 13%
Monogenic Epileptic Disorder
protein involved
Autosomal Dominant Nocturnal Frontal seizures
Nicotinic acetylcholine receptor subunits
progressive myoclonic epilepsy
involved protein is cystatin B
protein tyrosine phosphatase, laforin
protein is cystatin B - Unverricht-Lundborg disease with PME
protein tyrosine phosphatase, laforin - Lafora body disease with PME
T/F
Seizures having their onset several days or weeks after birth are more often an expression of an acquired or hereditary metabolic disease.
True
p339
hypoglycemia is the most frequent cause
infantile epilepsy myoclonic and focal seizures resistant epilepsy learning disability, developmental delay abnormality of Sodium channels (SCN1A)
Dravet syndrome
T/F
Seizures during Pregnancy
According to EURAP, about 2/3 of epileptic women who became pregnant have increased severity and frequency of seizures.
FALSE
no change in frequency and severity
p343
pregnant women on AED
the risks of teratogenic effects is highest in those taking
valproate
pregnant women on AED
most common teratogenic effects
cleft lip and cleft palate
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.
increase risk in pregnant on AED 4-5%
overall population of 2-3%
polytherapy with lamotrigine and valproate during pregnancy has an estimate risk of _____ for developing teratogenic effects
12%
most frequent idiosyncratic reactions to AEDs
rashes
Idiosyncratic phenytoin hypersensitivity
rashes fever lymphadenopathy eosinophilia blood dyscrasias polyarteritis
Prolonged used of phenytoin
hirsutism
hypertrophy of gums
coarsening of facial features
overdose phenytoin
ataxia
diplopia
stupor
rare idiosyncratic side effect of phenytoin
choreoathetosis
Carbamazepine idiosyncratic reactions
mild leukopenia
pacytopenia
hyponatremia
diabetes insipidus
Valproate side effects
weight gain in first months of tx
mesntrual irregularities
PCOS
rare: pancreatitis
tremor and slight bradykinesia
interaction of valproate and lamotrigine
Increased serum level of LAMOTRIGINE
MOA Lamotrigine
selectively blocks slow sodium channel
prevents release of excitatory transmitters: glutamate and aspartate
MOA levetiracetam
affects SV2A synaptic vesicle protein
Side effect Levetiracetam
dizziness and sleepiness
irritability
depression
MOA Gabapentin
affects calcium channels
MOA Vigabatrin
inhibits GABA transaminase
no longer use because of side effect: retinal damage
MOA Tiagabine
inhibits GABA reuptake
Topiramate Side effects
renal stones
angle closure glaucoma
hyperchloremic metabolic acidosis
MOA Lacosamide
modulate voltage-gated sodium channel activity
Lacosamide side effects
headache
diplopia
may prolong PR interval
worsen heart failure
concurrent use of valproate and clonazepam produces
absence status
overall mortality in Status Epilepticus
20-30%
Maximum rate of infusion of valproate
3mg/kg per min
rate of phenytoin IV infusion
<50mg/min
Propofol syndrome
hypertriglyceridemia-associated pancreatitis
fatal shock and acidosis
T/F
In general, higher serum concentrations of AEDs are necessary for the control of focal seizures than for generalized ones.
True
p346
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.
FALSE
p346
not a precise reflection
most AEDs have a large proportion that are bound to albumin and does not penetrate nervous tissue
effect of VPA on metabolite of CBZ
VPA inhibits epoxide hydroxylase leads to buildup of CBZ epoxide
Drug interaction
VPA on phenytoin and phenobarbital
accumulation of active PHT and of phenobarbitak by displacing them from serum proteins
Effect of Pb and CBZ on warfarin
reduced levels of warfarin
Effect of Phenytoin on Warfarin
Increased levels of warfarin
