epilepsy Flashcards
WHat characterizes atypical absence seizures?
slower (1.5-2.5HZ vs 3hz), last longer, and occur in children with NDD issues
where do olfactory auras come from?
medial temporal lobe
Triad in Lennox Gastaut
- 2 seizure types: tonic, atonic, atypical absence
- EEG w/ 1.5-2.5 HZ spike and wave: slow spike and wave discharges
- developmental delay
Best treatment for Absence epilepsy
ethosuximide
best tx for GTCs with absence?
VPA, Lamictal
JME patients jerk more when?
alcohol, sleep deprivation
JME patients have what on EEG (2 things)
- photic response
2. 3.5-4.5 spike/polyspike wave discharges
prominent drooling, speech arrest, some GTC at night in otherwise healthy older kid?
BRE
EEG left side?
odd
Panayiotopoulus syndrome?
early onset, benign epilepsy of childhood with occipital paroxysms: nighttime seizures may be prolonged, vomiting, eye deviation with hemiclonic activity or secondary generalization
late onset benign occipital epilepsy name?
Symptoms?
Gastaut type in adolescence. visual symptoms with secondary generalization, then HA
Most common type of progressive myoclonic epilepsy? Genetic cause?
Dx?
Unverricht Lundborg disease, mutation in cystatin B on Chr 21. Dx with skin biopsy in axilla showing membrane bound vacuoles in eccrine sweat glands.
what is lafora disease and how to distinguish from Unverricht-Lundborg? Pathology, chromosome?
occipital seizures, visual hallucinations. Lafora bodies with polyglucosan are in tissues and eccrine sweat glands, it progresses faster. Chr 6. Muscle, skin, liver, brain inclusions
What is NCL? What is the genetics and the hallmark? Infantile form?
Neuronal Ceroid Lipofuscinoses, AR lysosomal storage disease, abnl accum of lipopigment. Get vision loss. Infantile form is Chr 1p32 for palmitoyl protein thioesterase PPT1. Microcephaly, epilepsy, regression.
PAth seen in NCL?
granular deposits, curvilinear bodies, fingerprint profiles, rectilinear complexes.
MERRF is associated with gene in what
lyseine
What causes sialidosis type 1 and what do you get on exam? Dx?
defect in neuraminidase, so no cleavage of sialic acid from galactose. Cherry red spot and cognitive deterioration. Dx with high sialyloligosaccharides in urine
What causes benign familial neonatal convulsions?
voltage gated K channel mutation
What causes GEFs syndromes generally?
Voltage gated Na channel
SCN1A mutation on what chromosome causing severe myoclonic epilepsy of infancy and GEFS+ type 2
Chr 2q24
s/e of Carbamazepine (tegretol/carbatrol)
hypoNA, agranulocytosis, liver inducer, autoinduction
s/e of Ethosux / Zarontin?
GI
s/e of felbatol/felbamate
liver failure, aplastic anemia, insomnia
s/e of neurontin?
periph edema can worsen myoclonus
s/e of trileptal / oxcarb?
Hypo Na
Fast IV Dilantin can cause what?
purple glove syndrome and arrhythmia
Topamax: s/e
kidney stones, oligohydrosis, wt loss, glaucoma, metabolic acidosis, word finding probs at high dose
AED w/ sulfa?
Zonisamide
Tiagabine can cause what?
absence status
AEDs which mood stabilize?
CLOV: carbamazepine, lamictal, oxcarb, valproate
Wt loss AEDs
felbamate, topiramate, zonisamide
AED w/ migraine help
gabapentin, topamax, valproate
Tremor prevention with AED?
primidone, topiramate
Kidney eliminators AED
keppra, neurontin, vigabatrin
THE seizure GABA
GABA A
Na channel AEDS (6)
carbamazepine, lamotrigine, phenytoin, topiramate, valproate, zonisamide
barbiturate vs benzo mechanism?
GABA A channel:
Benzo: frequency of opening
Barb: open longer duration
(Ben photographed frequently, bars open late)
AED T- voltage gated Ca channel
Zonisamide, VPA, ethosux
