Epilepsy Flashcards
Appears between ages 1 and 10, sometimes de novo, sometimes after infantile spasms.
• Triad of
(1) multiple seizure types refractory to AEDs,
(2) mental retardation (most), and
(3) slow spike-and-wave (1.5-2.5 HZ) activity on EEG.
Lennox-Gastaut Syndrome
Lamotrigine and OCPs/HRT/Pregnancy
Oral contraceptive pills and hormone replacement therapy result in increased clearance of lamotrigine (up to 65%) and thus lower serum levels
• Specifically OCPs containing ethinylestradiol (not progesterone)
PREGNANCY also results in increased clearance thus may need to INCREASE amount of lamotrigine during pregnancy (just remember to go back down, post-partum)
SUDEP
-3 most significant risk factors
Sudden unexplained death in epilepsy (SUDEP)
Studies suggest that the most significant risk factors include uncontrolled generalized tonic-clonic convulsions, higher frequency of seizures, and the need for multiple AEDs.
Absence seizures, nonconvulsive seizures, and etiology of the epilepsy, have no known bearing on the risk of SUDEP.
4 year old with seizures that worsened with carbamazepine. What is the diagnosis?
Landau-Kleffner syndrome
consists of language regression, seizures, and sleep-activated spike discharges.
*can be worsened by carbamazepine!
Treat with high dose benzos, steroids, IVIG, depakote
Topiramate side effects
Increases risk of developing calcium phosphate stones (not other types)
-50-70% renally eliminated
hypsarrhythmia= interictal high amplitude, arrhythmic delta activities with independent multifocal spike discharges
Patient presents with the following…what antiepileptic medication are they on?
- Tremor
- Elevated lipase and amylase
- Eye pain
- Severe flank pain
- Hyperammonemia
- Depakote
- Depakote
- Topamax
- Topamax
- Depakote
Generalized epilepsy with febrile seizure PLUS
- AD with 50%-60% penetrance
- includes pts in whom febrile sz persist past the defined upper limit of age
- May be associated w afebrile GTC seizures
- sodium channel mutations (3 different Na channels or 1 GABA receptor subunit)
- SCN1A (most frequent), SCN1B, SCN2A mutations
What is first line treatment for myoclonic epilepsy?
In patients with what mutations must you be careful with the use of this AED?
Valproic acid
Be careful with its use in pts with Mitochondrial mutations such as POLG gene mutations bc fulminant hepatic failure may result
Rasmussen disease
Progressive focal seizures that increase in duration & severity
Changes in white matter with hyperintensity -> then atrophy
Focal & multifocal epileptiform discharges & slowing
Patient is encephalopathic. What is the most likely cause?
Triphasic Waves
Usually neg-pos-neg, with duration increasing in each. Frontal predominance; Bilateral; Most DO NOT have A-P lag, but some do.
Think metabolic encephalopathy; usually hepatic.
What stage of sleep is this found in?
Sleep Spindles
Define stage 2 sleep, ~13-14 Hz, max ~central
After 2 years of age, asynchronous sleep spindles are considered abnormal.
Normal awake EEG
Eyeblinks, posterior dominant rhythm
Normal EEG
The background activity is posteriorly dominant alpha rhythm at 9 to 9.5 Hz. This is normal waking occipital activity. The normal
occipital background rhythm disappears on eye opening which is shown in the EEG tracing.
Large L hemisphere subdural hematoma
POSTS: Positive Occipital Sharp Transients of Sleep
Occur in stage 1 and 2 sleep
Focal Polymorphic delta
Consider underlying structural abnormality (infarct/tumor).
What disease process is this associated with?
What are potential treatment options?
Lennox Gastaut:
Interictal slow spike and wave
- multiple seizure types
- MR
- interictal EEG showing spike & wave
•Possible Rx: felbamate, rufinamide
Hypsarrhythmia
seen in patients with infantile spasms
High voltage, irregular slow waves w/ multiple sharp & spike d/cs
Subacute Sclerosing Panencephalitis (SSPE)
•Generalized periodic long-interval diffuse discharges in the EEG that recur every 4 to 15 seconds.
If not vaccinated against measles. Progressive cognitive decline after infection, -> seizures, motor abnormalities -> coma -> death in 1-3 years.
“Fourteen and 6” spikes
Benign variant; Posterior head regions during light sleep;
Most common in adolescents;
Best seen on referential montage
Fourteen and 6 positive spikes
Sharply contoured and occur in the posterior head regions during light sleep.
Best demonstrated on referential EEG montages and are most common in adolescent patients.
EKG Artifact
PLEDs
Periodic lateralized epileptiform discharges (PLEDs) are seen after an acute or subacute focal cerebral insult such as a stroke and are often associated with seizures.
Patient presents encephalopathic and you suspect an infectious etiology. What is the most likely diagnosis?
Periodic lateralized epileptiform discharges (PLEDS) occur in the majority of cases of HSV encephalitis.
EEG from a 5 year old boy with focal twitching of the face and excessive salivation.
1) What is the diagnosis?
2) What is the prognosis?
3) What is treatment of choice?
1) Benign Epilepsy with Centrotemporal Spikes (BECTS; Rolandic epilepsy)
•Onset age 3-16
•Focal twitching of the face and/or hand, excessive salivation, gurgling/choking and inability to speak. May become generalized.
•About 20% of patients only have one seizure and in two thirds of the cases seizures are infrequent.
2) Children are neurologically normal, and the majority outgrow the disorder.
3) Carbamazepine is the treatment of choice, if treatment is necessary.
Child presents with following EEG.
What is the diagnosis?
What is the treatment of choice?
Absence Seizure
3 Hz spike and wave
- Typical childhood absence seizures do not have auras or post-ictal confusion.
- Automatisms can be seen.
- EEG shows 3 Hz to 4 Hz generalized spike-and-wave discharges
- Imaging studies of the brain are normal.
- Tx: Ethosuximide or valproate. Lamotrigne now approved for absence.
What drug likely caused this EEG finding?
Benzodiazepine
Excess Beta
Status Epilepticus
CJD
periodic, short-interval (0.5 to 1.0 second), generalized, bisynchromous discharges on EEG
(GPEDS)
2 month old male with tonic spasms in clusters. What is the most likely diagnosis?
Ohtahara Syndrome
EEG shows burst suppression
(if onset in first few weeks of life with prominent myoclonus, could also consider Early Myoclonic Encephalopathy which would also have burst suppression)
Patient presents with partial seizures with olfactory hallucinations and the following MRI. What is diagnosis?
Mesial temporal lobe epilepsy
Associated with mesial temporal sclerosis
Partial seizures with olfactory hallucinations, impaired awareness, and automatisms
