epilepsy Flashcards
common presenting cause in venous sinus thrombosis?
seizure
approximate incidence of sz / epilepsy / yr?
sz: 80/100,000
epilepsy: 45/100,000
temporal lobe sz in adults, think what?
HSV 1
hsv in newborns vs adults
nb: Hsv 2
adult: Hsv 1
eclampsia seizure tx?
Magnesium…. then aed
make sure no venous thrombosis
seizures increased with what key meds
6
bupoprion, fentanyl, tramadol, lido, baclofen, cefepime
Na hyponatremia w/ concern for seizures?
<125
Neurodevelopmental issue in people with mesial temporal sclerosis / temporal lobe epilepsy
Treatment of this?
short term memory loss, improved with surgery if failed 3 meds in TLE
Drug that is contraindicated in absence seizures?
carbamazepine
Generalized spike and wave discharges evoked by photic?
Dx?
JME
Batten’s disease is what? what do you see diagnostically?
Neuronal ceroid lipofuscinosis NCLs (Progressive myoclonic epilepsies)
+PAS
curvilinear and fingerprint bodies on EM
epilepsy d/o w/ dramatic / agitated behavior at night during sleep, brief < 1min
frontal lobe epilepsy (quick, very agitated and moving, quickly out of it w/o significant postictal state)
epilepsia partialis continua - make sure you look for what?
possible brain tumor
fencer posturing in sleep, dx?
frontal lobe epilepsy affecting supplementary motor
what type of epilepsy/lesion is associated with people w/ hx of prolonged febrile sz as child?
mesial temporal sclerosis / TLE
in TLE, what sx usually ipsilat vs contralat?
ipsilat: automatism
contralat: dystonic posturing
type of epilepsy when seizures involved formed hallucinations?
parietal lobe, inferior posterior temporal parietal area
seizure length in atypical absence
longer than typical (>10sec)
3 spike waves in 1 sec block?
Absence 3Hz
Lafora Body disease characterized by what?
How to dx?
EEG?
What chromosome?
- severe myoclonus
- dementia
- death by 2nd decade
Dx w/ skin bx showing lafora body (polyglucosan neuronal inclusions)
Chr 6
EEG w/ prominent occipital spikes/sz, spike/wave d/c
early AM jerks, tonic clonic sz, provoked by alcohol, poor sleep, photic
Dx and Tx
JME, VPA
What is Unverricht-Lundborg disease?
Progressive myoclonic epilepsy (Baltic myoclonus)
Genetics of Unverricht-Lundborg (((Baltic Myoclonus)
EEG findings
Dx?
Time course distinction
AR, Chr 21q22, abnl cystatin gene w/ repeat
EEG w/ background slowing and 3-5Hz, polyspike and wave d/c
Skin bx w/ vacuoles in sweat glands, serum testing
Tends to be more slowly progressive with late cerebellar deterioration
progressive neurologic disorder, ataxia/dementia, vision issues, myoclonus
Batten’s Diseases: NCL
Santavuori disease
early infantile NCL/Batten’s also w/ microcephaly
Kufs disease?
adult Batten / NCL, slower progression, no visual
most common pediatric / juvenile neurodegenerative disorder
genetics/inheritance
Spielmeyer Vogt Sjogren (form of Batten)
AR, chr 16
progressive visual loss, myoclonus/ataxia/dementia
late infantile NCL?
Genetics / inheritance
Bielschowsky-Jansky dz
AR, Chr 15
Progressive visual loss, myoclonus, severe dementia / ataxia
genetics / inheritance of NCL early infantile form / Santavuori disease
AR / Chr 1
MERFF genetics?
mitochondrial point mutation for nucleotide pair 8344
MRI w/ leukencephalopathy and cerebellar atrophy
subacute necrotizing encephalomyelopathy?
Leighs
Sialidosis -enzyme issue -main characteristics onset? sx?
alpha neuraminidase def
diffuse cortical atrophy, vacuolar inclusions in liver
Onset adolescent
Cherry red spot / night blindness + loss of color vision, ataxia, severe myoclonus (inducible), photosensitive sz
Enzyme defects for TaySachs and Sandhoff?
Taysachs: Hexosaminidase A
Sandhoff: Hexosaminidase A and B
Aicardi synddrome
genetics, clinical sx
Xlinked dominant
- coloboma/chorioretinal lacunae
- agenesis of cc
- vertebral abnormalities
- sz w/ IS, hemiconvulsions
slow spike and wave < 3Hz discharges
Tx (3)
Lennox Gastaut
LTG, VPA, VNS
think of what if sensitivity marker is down to 200uV
hypssarhythmia