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Peds neuro boards, V2 > Epilepsy > Flashcards

Epilepsy Flashcards

1
Q

Otahara syndrome:

  1. Onset
  2. Seizure type
  3. other features
  4. EEG (interictal)
  5. EEG (ictal)
A
  1. Onset: first 10 days of life
  2. Seizure types: Multiple clusters of tonic spasms
  3. Other features: hypotonia, developmental arrest
  4. EEG Interictal: burst suppression
  5. High amplitude spike-and-wave followed by generalized fast activity (figure)
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2
Q

What is this?

A

Forehead plaque (Tuberous sclerosis)

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3
Q

Most common abnormal Brain finding in patients with temporal lobe epilepsy

A
  1. Mesial temporal sclerosis
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4
Q

most common gene associated with epilepsy of infancy with migrating focal seizures

A

KCNT1

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5
Q

Rate of depression in epilepsy

A

30%

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6
Q

Common EEG abnormalities with Temporal lobe epilepsies

A
  1. Temporal spikes and sharp waves (duh)
  2. Temporal delta slowing (TIRDA)
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7
Q

Most common genetic cause for frontal lobe epilepsy

A 
Autosomal dominant frontal nocturnal epilepsy:
acetylcholine receptor (Ach-R) mutation
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8
Q

Epilepsy localization by semiology: Frontal lobe

  1. Fencer posture
  2. hyperkinetic behaviors (rocking, bicycling, pelvic thrusting, back arching)
  3. Focal clonic movements
A
  1. Fencer Posture = Supplementary motor area (tonic / dystonic features)
  2. Hyperkinetic behaviors = Dorsolateral / orbitofrontal
  3. Focal clonic = posterior frontal
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9
Q

Channels (2) and receptors (2) most commonly affected in childhood absence epilepsy

A
  • Calcium and chloride channels
  • GABA-A and GABA-B receptors
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10
Q

Treatments for absence epilepsy (4) and drugs to avoid (4)

A

Treatment:

  1. Ethosuximide (first line)
  2. valproate
  3. lamotrigine
  4. levetiracetam

Avoid:

  1. carbamazepibne
  2. vigabatrin
  3. tiagabine
  4. phenytoin
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11
Q

Treatments (5) and drugs to avoid (2) in Juvenile myoclonic epilepsy

A

Treatment

  1. Valproate (first line if not a woman of childbearing age)
  2. levetiracetam
  3. lamotrigine
  4. topiramate
  5. zonisamide

Drugs to avoid

  1. carbamazepine
  2. oxcarbazepine
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12
Q

Genes associated with juvenile myoclonic epilepsy (per BtB)

(3)

A
  1. EFHC1
  2. GABRA1
  3. CLCH2
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13
Q

Age-range an peak onsest for Lennox-Gastaut Syndrome

A

1-8 years

Peak is 3-5 years

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14
Q

Drug known to increase drop-attacks in LGS

A

Carbemazepine

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15
Q

Typical age of onset of Landau-Kleffner syndrome

A

2-11 years

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16
Q

Mechanisms of action:

  1. Phenobarbital
  2. Phenytoin
  3. Carbamazepine
  4. Ethosuximide
  5. Valproic acid
A
  1. Phenobarbital: GABA enhancer
  2. Phenytoin: Na+ blocker
  3. Carbamazepine: Na+ blocker
  4. Ethosuximide: Ca++ blocker
  5. Valproic Acid: Na+ blocker, Ca++ blocker, GABA enhancer
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17
Q

Side Effects:

Phenytoin (7)

A
  1. Drowsiness
  2. Nausea
  3. Ataxia
  4. Gingival hyperplasia
  5. Rash
  6. Neuropathy
  7. Cardiac suppression (was originally used as an anti-arrhythmic
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18
Q

Side Effects:

Carbamazepine (6)

A
  1. Dizziness
  2. Nausea
  3. Ataxia
  4. Double vision
  5. thrombocytopenia
  6. Leukopenia
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19
Q

Side Effects:

Phenobarbital (2)

A
  1. Sedation
  2. “mental slowing”
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20
Q

Side Effects:

Ethosuximide (5)

A
  1. Drowsiness
  2. Confusion
  3. Insomnia
  4. Headache
  5. Ataxia
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21
Q

Side Effects: Valproate

“CNS” (4)
“GI” (5)
“Female” (2)
Other (1)

A
  1. “CNS-related”
    1. Drowsiness
    2. Dizziness
    3. Ataxia
    4. Tremor
  2. GI
    1. nausea
    2. weight gain
    3. Transaminitis
    4. Pancreatitis
    5. Eosinophilic colitis
  3. “Female”
    1. Teratogenicity
    2. PCOS
  4. Other: hair loss
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22
Q
A
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23
Q
A
24
Q

Adverse effects Lamotrigine

(5)

A
  1. Rash (SJS)
  2. Headache
  3. ataxia
  4. somnolence
  5. vivid dreams
25
Q

Adverse effects:

Oxcarbazepine (6)

A
  1. Fatigue / somnolence
  2. Dizziness
  3. ataxia
  4. double vision
  5. hyponatremia
  6. rash
26
Q

Adverse Effects:

Topiramate (8)

A
  1. Somnolence
  2. dizziness
  3. ataxia
  4. parasthesia
  5. Mental slowing
  6. weight loss
  7. renal stones
  8. Acute angle-closure glaucoma
27
Q

Adverse Effects:

Zonisamide (5)

A
  1. Renal stones
  2. dizziness
  3. ataxia
  4. mental slowing
  5. weight loss
  6. renal stones

(basically topiramate minus somnolence and parathesias)

28
Q

Adverse effects:

Keppra (5)

A
  1. somnolence
  2. dizziness
  3. headache
  4. personality change / aggressiveness
  5. depression / SI
29
Q

Adverse Effects:

Lacosamide

A
  1. Dizziness
  2. ataxia
  3. diplopia
  4. nausea
  5. depression
30
Q

Adverse Effects:

Vigabatrin (4)

A
  1. Somnolence
  2. headache
  3. agitation
  4. visual field loss
31
Q

Adverse Effects:

Tiagabine (4)

A
  1. Dizziness / somnolence
  2. depression
  3. confusion
  4. ataxia
32
Q

Adverse Effects:

Gabapentin / Pregabalin (6)

A
  1. Somnolence
  2. dizziness
  3. ataxia
  4. tremor
  5. edema
  6. weight gain
33
Q

Adverse Effects:

Clobazam (5)

A
  1. Dizziness
  2. Somnolence
  3. ataxia
  4. diplopia
  5. dysarthria
  6. (rare) SJS / TEN
34
Q

Adverse Effects:

Rufiamide (6)

A
  1. Dizziness\Drowsiness
  2. Nausea
  3. headache
  4. rash
  5. mood changes
  6. Shortening of QTc (get EKG before starting)
35
Q

Adverse Effects:

Perampanel (4)

A
  1. Fatigue
  2. dizziness
  3. ataxia
  4. FDA warning for psychiatric effects (homicidal ideation)
36
Q

Adverse Effects

Ezogabine / Retigabine

A
  1. Dizziness
  2. Drowsiness
  3. slurred speech
  4. FDA warning (Skin discoloration / retinal pigment changes)
37
Q

Antiseizure medications used for generalized epilepsy:

(6)

A
  1. leviteracetam
  2. topiramate
  3. valproate
  4. ethosuximide
  5. zonisamide
  6. lamotrigine
38
Q

Major Risk factors for SUDEP:

(10)

A
  1. Uncontrolled epilepsy
  2. Epilepsy of an early onset
  3. Multiple ASMs
  4. Subtherapeutic levels of ASMs
  5. Young age (20-40)
  6. Male Sex
  7. Neurologic comorbidities
  8. Sleep / nocturnal seizures
39
Q

Benign Familial Neonatal Seizures VS Benign Familial Neonata-Infantile seizures

A

Benign familial neonatal seizures (BFNS):

  1. Autosomal Dominant
  2. KCN2 and KCN3
  3. Onset: Typically DoL 2-8 but can be up to 6 months
  4. Interictal EEG: normal
  5. Ictal eeg: Asymmetric Spike-wave

Benign familial neonatal-infantile Seizures

  1. ***
  2. SCN2A
  3. Onset: 2 days to 7 months (peak 2-3 months)
  4. Seizure type
    1. focal motor with yead / eye deviation
    2. often with 2/2 generalization
  5. Interictal EEG: normal
  6. Ictal EEG: onset typically in posterior quadrant
40
Q

Early myoclonic encephalopathy

  1. Onset
  2. Interictal EEG
  3. Seizure types
  4. Etiology (2)
A
  1. Onset: first 3 months
  2. EEG: burst suppression mostly during sleep, awake: multifocal spikes
  3. Seizure types
    1. myoclnic (mostly face and eyelids), migrates
    2. tonic
    3. focal
  4. Etiology
    1. Metabolic
    2. IEM
41
Q

Ohtahara syndrome

  1. Onset:
  2. EEG:
  3. Seizure types (MC + 2 more)
  4. Etiology (2)
A
  1. Onset: first 3 months
  2. EEG: Burst suppression
  3. Seizure types
    1. Mostly tonic spasms
    2. myoclonic
    3. focal seizures
  4. Etiology
    1. CNS malformations
    2. Genetic
42
Q

CECTS

  1. Onset
  2. Seizure types (6)
  3. EEG
A
  1. Onset: 18 months - 13 years (peak 5-10 years)
  2. Seizure types
    1. Nocturnal Focal motor onset with bilateral spread to Tonic clonic
    2. Diurnal focal seizures from rolandic area, often with preserved consciousness
    3. Classic features
      1. Hemi-facial seizures (30%)
      2. Speech arrest (40%)
      3. Hypersalivation (30%)
      4. other oropharyngeal symptoms (53%)
  3. EEG
    1. …centro-temporal spikes
43
Q

Panayiotopoulos syndrome verus Gastaut type epilepsy

A

Panayiotpoulos (early) type

  1. Onset: 1-14 years, peak 4-5 years
  2. Seizure clues
    1. Pallor
    2. Flushing
    3. vomiting
    4. subtype involves minutes of flaccid hypotonia

Gastaut (late) type

  1. Onset: 4-14 years, peak 8 years
  2. seizure clues
    1. Visual hallucination
44
Q

Criteria For Neurofibramatosis Type 2

A

Need one of the following

  1. Bilateral eightth nerve masses
  2. First degree relative with NF-2 AND unilateral eighth nerve mass
    - or-

Two or more of the following

  1. Neurofibroma
  2. meningioma
  3. glioma
  4. schwannoma
  5. juvenile posterior subscapular lens opacity
45
Q

Treatment for Rasmussen’s Encephalitis (4)

A
  1. IVIG
  2. Plasmaphoresis
  3. Steroids
  4. Hemispherectomy
46
Q

Gelastic Seizures are (for the boards) one-to-one with ____, and is treated with _____

A

Gelastic Seizures are (for the boards) one-to-one with Hypothalamic Hammartoma, and is treated with resection or ablation

47
Q

What is this?

A

Hypothalamic Hammartoma

48
Q

Workup for BRUE (6)

A
  1. screen for GERD
  2. urinalsysis / culture
  3. (consider) neuroimaging)
  4. (consider) EEG
  5. pneumoimaging
  6. WBC
49
Q

Typical age range for febrile seizures

A

6 months to 5 years

Peak 1-2 y.o.

50
Q

Seizure auras and location

dysphasic psychic

dysmnesic aura

cognitive psychic aura

Illusions

A
  1. dysphasic aura = left perisylvian area
  2. dysmnesic aura = mesobasal temporal lobe
  3. cognitive psychic aura = frontal association cortex (“cognitive association”)
  4. Illusions = left superior temporal neocortex (“these illusions are super new, but temporary)
51
Q

Key factor differentiating CDLK5 from Rhett Syndrome (2)

A
  1. CDLK5 deficiency syndrome patients never show normal developmental milestones
  2. CDLK5 patients usually have early onset seizures (within first 3 months, as opposed to 6-18 months)
52
Q

Common Progressive myoclonic epilepsies and their respective chromosomes

A

“SLUMiN it”

Sialidosis (6p)
Lafora disease (6p - EPM2B and 6q - EPM2A)
Unverricht-Lundborg disease (EPM1 21q)
MERRF (mitochondrial lysine)
NCL (multiple genes / chromosmes)

53
Q

Unverricht-Lundborg Disease

Inheritance
Defect in cystatin B on chromosome 21
age of onset
Additional features (2)

A

“U, Little Cys. Be Smart and don’t get High until you’re 21, or else you’ll have to sweat or you’re bound to be SLUMiN it later on

Autosomal recessive
age 6-18 (“little sis”)
defect in cystatin B on chromosome 21
intelligence often preserved until late in course
may have high SSEPs
axillary biopsy may show membrane bound vacuoles on ecrine sweat glands

Form of progressive myoclonic epilepsy (acronym SLUMi_N_)

54
Q

Patient with Seizures (or bipolar, or trigeminal nerualgia) is treated with Carbemazepine presents with toxicity despite no changes in dose

What are 3 substances which could have caused this?

A

Cimetidine (transient)
erythromycin
Grapefruit juice

55
Q

Hallmark features of Autosomal Dominant Epilepsy with Auditory features as well

A

Auditory phenomena (Humming, buzzing,,,,,,,, ringing)

Receptive aphasia

56
Q

Medications to avoid with epilepsy

A

there’s a LOAD of medicines that cause seizures to HATCH

  • *L**ithium
  • *O**piates
  • *A**ntidepressants
  • *D**opamine agonists

Hypnotics
Antipsychotics
Triptans
CHolinesterase Inhibitors

Peds neuro boards, V2 (29 decks)

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