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Cheng-Ching > Epilepsy > Flashcards

Epilepsy Flashcards

1
Q

simple vs complex febrile seizures (gene)

A

simple >6 months and <5 yrs: generalized seizure <15 min, no more than 1 in 24 hrs
-peak: 18 months

complex “febrile plus”-SCN1A gene (sodium channel subunit) mutation
-can have afebrile GTCs

-FHx febrile seizures does not increase risk epilepsy
-Hx complex FS does not increase risk recurrent FS

-relationship with MTS?

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2
Q

Rasmussens

A
  1. progressive focal cortical atrophy (unilateral hemispheric atrophy)-inflammatory?
  2. progressive neurologic dysfunction: hemiparesis + cog delay
  3. epilepsia partialis continua

-Tx: IVIG; hemispherectomy; otherwise focal cortical atrophy spreads
-GLUR3? glutamate Ab
histo: perivascular cells; glial nodules; spongy degeneration

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3
Q

myoclonic epilpesy

A

progressive myoclonic epilepsy - PME - Lafora body, Unvcerricht Lundborg, MERRF, sialidosis, lipofuscinosis

Tx: Valproate acid

NO VPA if suspect mitochondiral mutation (POLG) - liver failure -POLG no myoclonus
-VPA liver enzyme inhibitor, ^^drugs/warfarin

*exacerbate myoclonus: phenytoin, gabapentin, carbamazepine, pregabalin, vigabatrin *

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4
Q

Unvvericht-Lundborg

A

Baltic myoclonic epilepsy
AR - EPM1 gene - cystatin B -

(protease inhibitor initiates apoptosis)
-action related myoclonus
-various seziure types
-ataxia, tremor, ID
Tx: VPA, onfi, KEppra

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5
Q

Lafora body

A

EPM2A encoding laforin protein
12-17 yrs
-Lafora bodies-skin biopsy - PAS+ intracell polyclucosan inclusion bodies - sweat gland duct cells

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6
Q

sialidosis

A

AR - NEU1
type 1 - no alpha-neuraminidase; presnt in adolescence/adults - ataxia, GTC, vision loss; normal cognition
-cherry red spot

type 2 - deficiency N acetyle neuraminidase, B galactosialidase; neonates to 20s; myoclonus, coarse faces, corneal clouding

Dx: urinary sialyl oligosaccharide; leukocyte lysosomal enzyme deficiency

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7
Q

PDR

A

alpha adult rhythm at 8-10 yrs (A for Adult for Alpha for Aight)

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8
Q

Absence

A

Peak 6 yrs, 70% girls
-provoked by hypoglycemia, hyperventilation
-staring few sec + eyelid/eyes/eyebrow movements
-CACNA1H calcium channel mutation (P/Q)
Or GABRG2
-Thalamus, T type calcium channels

Tx: ethosuxamide (inhibit T Ca); fewer attentional defects vs VPA but VPA can use

Exacerbate: lamotrigine, phenytoin, cabamazepine, gabapentin
-GABAb R activate T type Ca channels

GTCs + absence - Tx VPA, lamotrigine

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9
Q

enzyme inducing ASMs

A

primidine, PB, phenytoin, carbamazepine, oxcarbazepine
topiramate at doses >200 mg/d
-topiramate causes uric acid stones

-increase metabolic OCPs
-OCs containing estrogen could decrease the concentrations of some ASMs such as lamotrigine (LTG), through enhancing their metabolism by UGT1A4 (an enzyme responsible for the glucoronidation of some ASMs by ethinylestradiol)

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10
Q

SELECTS

A

Tx: Keppra; carbamazepine

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11
Q

childhood epilepsies age

A

6 months: focal onset seizure->West syndrome
3 yrs: focal onset->LGS
14 yrs: CAE (childhood absance)->JME

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12
Q

Phenytoin

A

-inhibits Na channels
zero order nonlinear kinetics at 10-15 ug/mL - small dose increases can cause toxicity
; mostly liver metabolism
enzyme inducer-lowers drug
-highly protein bound - check free phosphy levels

-TMP/SMX; fluconazoledisplaces PHT from albumin and increase free levels-> PHT toxicity

SE: gingival hyperplasia.
-DRESS, SJS
aplastic anemia, hepatic failure
-hirsutism, osteoporosis, coarse facial features
-peripheral neuropathy
-cerebellar atrophy (not cortical atrophy)

dosing IV bolus:
[target level 15 - current level XX] x [body weight x 0.8 L/kg volume distribution]

-worsen myoclonic + absence epilepsy

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13
Q

VPA

A

-inhibits Na + T type Ca; agonist at GABAa
enzyme inhibitor-raises drug levels; liver metabolized (increases half life LTG)
-not if mitochondrial disorder
-hepatotoxicity-in kids <6 on polytherapy
-secondary parkinsonism, weight gain, hair loss, PCOS

-loading dose = 20-25 mg/kg bolus

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14
Q

Lamotrigine

A

blocks Na channels, inhibits glutamate

-least cognitive side effects
-estrogen OCPs + pregnancy increase LTG clearance
therapeutic at >14; can get anxiety

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15
Q

vigabatran

A

inhibit GABA transaminise which breaks down GABA causing increased GABA
-exacerbates myoclonus

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16
Q

carbamazepine

A

blocks Na channels which prevents
AP firing in depolarized neurons

-oxidized to carbamazepine epoxide -active metabolite; causes side effects

-enzyme inducer; autoinduces which reaches max in 3-5 weeks->need gradual increase when levels decrease in 1-2 months
-VPA inhibits metabolism of 10,11 epoxide->levels increased and get toxicity even if normal carbamazepine levels

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17
Q

oxcarbazepine

A

Oxcarb: not autoinducer; can titrate faster
-no epoxide
-fast inactivation of VGSC

After OXC administration, a peak concentration of the parent drug is detectable in plasma, with a half-life of 1-2.5 hours, and also in the cerebrospinal fluid.This OXC peak concentration may produce an effect in brain before transformation to its active metabolite

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18
Q

Felbamate

A

FBM, reserving it for medically refractory epilepsy such as Lennox Gastaut syndrome

inhibits the cytochrome P-450 system thus resulting in significant interactions with other AEDs and in most instances increasing the levels of PHT, CBZ epoxide and VPA

hepatotoxic

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19
Q

eslicarbazepine

A

ESL is rapidly and extensively metabolized by hepatic esterases to eslicarbazepine. This active metabolite has a linear pharmacokinetic profile,
-a low binding to plasma proteins (<40%)
-half-life of 20-24 hours

  • excreted by kidneys in an unchanged form or as glucuronide conjugates.

once a day and has a low potential for drug-drug interactions

ESL and OXC exert their effects through an identical metabolite, which is eslicarbazepine

-OXC affects fast inactivation of VGSC
-OXC is transformed to equal amounts of both stereoisomers-inactive and active

  • ESL administration, the agent is rapidly converted to eslicarbazepine and is not even detectable in plasma
  • ESL is mainly transformed to the active metabolite, less exposure to inactive metabolite
20
Q

pregnancy and ASM

A

ASM clearance increases and plasma concentration decreases as pregnancy advance. This occurs especially for LTG, LEV and OXC [83, 91], which may be because of the strong enhancement of maternal glucuronidation and hydrolysis during pregnancy, through which the three ASMs are metabolized

-ESPECIALLY LTG

21
Q

benzos

A

agonist at GABAa channels - open chloride channels causing hyper-polarization
-liver metabolism, renal excretion

intranasal midazolam contraindications: acute narrow-angle glaucoma

-if overdose->flumenazil

22
Q

lacosamide

A

inhibits slow VG Na channel
-stabilizes hyperpolarized membranes
-inhibits CRMP2

23
Q

SMA semiology

A

fencers posture - extended arm contralateral to seizing hemisphere (pointing/flexed arm ipsilateral to seizure)

24
Q

Aicardi syndrome

A

X L dominant - girls
agenesis corpus callusum
infantile spasms
retinal blindness-chlorioretinal laucnae
EEG-burst suppression; asynchrony

25
Q

Doose

A

normal cognitive development; myoclonic astatic (falls w/ loss of tone)
parietal abnormality EEG
Tx: VPA

26
Q

otohara

A

1 day-3 months of age
-burse suppression

27
Q

benign myoclonic epilepsy of infancy

A

4 mo-3 yr
males>females
-no clusters: brief spasms (vs infantile spasm)
interictal EEG normal; imaging normal
-usually no developmental delay
Tx: VPA

28
Q

Benign neonatal seizures

A

neonate “fifth day fits” - resolve by 4-6 wks
apnic spells, partial clonic seizure unilateral or BL
EEG: “theta pointu alternant” - discontinuous async unreactive delta activity + sharps
-no Tx, spontaneous resolve, neuro development normal

29
Q

Panayiotopoulous syndrome / early onset childhood occipital epilepsy

A

4-5 yrs age (up to 8); vis hallucinations auras while awakening; seizures at night

30
Q

last onset chilhood epilepsy -Gastaut type -

A

8 yrs - visual aura then hemiclonic convulsions + migraine

31
Q

LGS

A

1.5-2 Hz spike wave + multiple seizure types + development impair
-3-5 yrs typical presentation
-Tx: VPA

32
Q

Landau-Kleffner

A

5-7 yrs
-word deafness, normal hearing
-progress to global aphasia
-various seizure types
Tx: VPA, LTG

33
Q

AD frontal lobe epilepsy

A

start in childhood
-hypermotor seizures during non-REM sleep
+/- aura
-Tx: carbamazepine, oxcarbazepine
mutation CNRNA4, CHRNB2 - nicotinic ACh receptor

34
Q

electrical status epilepticus during slow wave sleep

A

usually 4-5 yrs
-psychomotor impairment
-multiple seizure types
~Landau Kleffner but more global regression

35
Q

temporal lobe semiology

A

dystonic posture contralateral to seizing hemisphere + hand automatisms ipsilateral

36
Q

hypothalamic hamartoma

A

gelastic seizures from hypothalamus
+/- precocious puberty

37
Q

first seizure

A

guidelines: EEG + CTH/MRI (not labs/LP/tox screen)

-ASM Tx: reduce seizure recurrence in next 2 yrs (not prognosis or QOL)

38
Q

REM sleep

A

90 min after sleep onset

39
Q

sleep structures

A

sleep spindles - after 5 months of age

40
Q

sleep apnea

A

apnea/hypopnea indices
- moderate sleep apnea - 15-30/hr
severe - >30/hr

central - no respiratory drive - comorbid with HF can improve if Tx HR

41
Q

non-REM parasomnia vs nightmares

A

non-REM: sleep terrors, confusional arousals, sleep walking - slow-wave sleep/N3 sleep

-sleep terrors: confusion if awaken
-autonomic Sx

sleep walking: slow wave sleep

sleep terrors vs nightmares- dreams remembered; last 1/3 of night; less autonomic Sx, not confused when awoken

42
Q

REM parasomnia

A

acting out specific scenes

43
Q

Kleine-Levin syndrome

A

recurrent hypersomnia - adolescence - days to weeks - sleep 18-20 hrs a day; increased appetite, irritable, hypersexuality

44
Q

narcolepsy

A

+ cataplexy - loss hypocretin neurons in lateral hypothalamus - low hypocretin levels

no cataplexy - hypocretin levels normal

45
Q

restless leg syndrome

A

Tx-DA agonist - pramipexole; ropinirole; gabapentin/pregabalin; opioids

46
Q

Ictal vomiting/spitting

A

Nondominent hemisphere

47
Q

Dravet

A

No sodium channel blockers
SCN1A sodium channel mutation

Cheng-Ching (13 decks)

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