Epilepsy and other Seizure Disorders Flashcards by kat remigio

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

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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

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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

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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

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semiology corresponds to the regions of the brain affected

clonic contractions

motor cortex

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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

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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

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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

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semiology corresponds to the regions of the brain affected

gustatory hallucinations

temporal lobe lesions

insula and parietal operculum

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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

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semiology corresponds to the regions of the brain affected

vertiginous sensations

superoposterior temporal region or junction between parietal and temporal lobes

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intense perception of familiarity in an unfamiliar circumstance or place

deja vu

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strangeness or unfamiliarity in a previously known place or circumstance

jamais vu

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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.

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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

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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

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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

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one of the most common illness to precipitate febrile seizure

herpesvirus 6

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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

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

Glutamate receptors GluR3

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

kindling phenomenon

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True or False

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

True

p 335

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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%

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True or False

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

True

p335

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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