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
Rasmussen Disease
1) Describe EEG findings
2) What is typical age of onset?
3) What do you see on histology?
4) What is the best established treatment?
1) EEG Findings: Continuous unilateral epileptiform discharges. Progressive and usually involves only one hemisphere.
2) Presents in childhood (6-10y) with uncontrollable focal seizures that rarely respond to AEDs.
3) Histologically, perivascular lymphocytic infiltrates with vascular injury, astrogliosis, neuronal loss, and cortical atrophy are seen
4) Best extablished tx: hemispherectomy
Hypsarrhythmia
seen in patients with infantile spasms
High voltage, irregular slow waves w/ multiple sharp & spike d/cs
Infantile Spasms
1) Describe EEG Findings
2) What syndrome presents with infantile spasms + MR?
3) Name 3 potential underlying etiologies
4) What is most common treatment?
1) EEG: Hypsarrythmia, high voltage diffuse slowing with multifocal spikes and sharps in a chaotic fashion.
2) Often with mental retardation (= West Syndrome)
3) Etiology: Inborn errors of metabolism; structural brain abnormalities; TS
4) Tx: ACTH; (Vigabatrin with TS)
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.
Subacute sclerosing panencephalitis
1) What infectious disease is this associated with?
2) Describe clinical presentation
3) Describe EEG findings
4) Describe MR Brain findings
1) late neuro presentation of previous measles, caused by host response to persistent measles virus in brain
2) presents age 5 to 15 yrs (primary infection < 4 yrs)
•regression w insidious onset over wks to mos
–behavioral changes & decline in school performance
–later tremors, myoclonus, seizures (often drop attacks), visual loss 2/2 chorioretinitis or cortical blindness
–chorea, pyramidal signs, cerebellar ataxia
–rapid motor deterioration, abn posturing, autonomic dysfunc, coma
3) Generalized periodic long-interval diffuse discharges in the EEG that recur every 4 to 15 seconds.
(Spongiform encephalopathies due to prion disease presents with GPEDs, but intervals are typically shorter than those of SSPE)
4) MRI: T2 Hyperintensities in the periventricular frontal, temporal, & occipital white matter
•Gen cerebral atrophy & ventric dilatation w progression of disease
“Fourteen and 6” spikes
Benign variant; Posterior head regions during light sleep;
Most common in adolescents;
Best seen on referential montage
