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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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24
Q

Adverse effects Lamotrigine

(5)

A
  1. Rash (SJS)
  2. Headache
  3. ataxia
  4. somnolence
  5. vivid dreams
25
Adverse effects: Oxcarbazepine (6)
1. Fatigue / somnolence 2. Dizziness 3. ataxia 4. double vision 5. hyponatremia 6. rash
26
Adverse Effects: Topiramate (8)
1. Somnolence 2. dizziness 3. ataxia 4. parasthesia 5. Mental slowing 6. weight loss 7. renal stones 8. Acute angle-closure glaucoma
27
Adverse Effects: Zonisamide (5)
1. Renal stones 2. dizziness 3. ataxia 4. mental slowing 5. weight loss 6. renal stones (basically topiramate minus somnolence and parathesias)
28
Adverse effects: Keppra (5)
1. somnolence 2. dizziness 3. headache 4. personality change / aggressiveness 5. depression / SI
29
Adverse Effects: Lacosamide
1. Dizziness 2. ataxia 3. diplopia 4. nausea 5. depression
30
Adverse Effects: Vigabatrin (4)
1. Somnolence 2. headache 3. agitation 4. visual field loss
31
Adverse Effects: Tiagabine (4)
1. Dizziness / somnolence 2. depression 3. confusion 4. ataxia
32
Adverse Effects: Gabapentin / Pregabalin (6)
1. Somnolence 2. dizziness 3. ataxia 4. tremor 5. edema 6. weight gain
33
Adverse Effects: Clobazam (5)
1. Dizziness 2. Somnolence 3. ataxia 4. diplopia 5. dysarthria 6. (rare) SJS / TEN
34
Adverse Effects: Rufiamide (6)
1. Dizziness\Drowsiness 2. Nausea 3. headache 4. rash 5. mood changes 6. Shortening of QTc (get EKG before starting)
35
Adverse Effects: Perampanel (4)
1. Fatigue 2. dizziness 3. ataxia 4. FDA warning for psychiatric effects (homicidal ideation)
36
Adverse Effects Ezogabine / Retigabine
1. Dizziness 2. Drowsiness 3. slurred speech 4. FDA warning (Skin discoloration / retinal pigment changes)
37
Antiseizure medications used for _generalized_ epilepsy: (6)
1. leviteracetam 2. topiramate 3. valproate 4. ethosuximide 5. zonisamide 6. lamotrigine
38
Major Risk factors for SUDEP: | (10)
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
Benign Familial Neonatal Seizures VS Benign Familial Neonata-Infantile seizures
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
Early myoclonic encephalopathy 1. Onset 2. Interictal EEG 3. Seizure types 4. Etiology (2)
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
Ohtahara syndrome 1. Onset: 2. EEG: 3. Seizure types (MC + 2 more) 4. Etiology (2)
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
CECTS 1. Onset 2. Seizure types (6) 3. EEG
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
Panayiotopoulos syndrome verus Gastaut type epilepsy
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
Criteria For Neurofibramatosis Type 2
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
Treatment for Rasmussen's Encephalitis (4)
1. IVIG 2. Plasmaphoresis 3. Steroids 4. Hemispherectomy
46
Gelastic Seizures are (for the boards) one-to-one with \_\_\_\_, and is treated with \_\_\_\_\_
Gelastic Seizures are (for the boards) one-to-one with **_Hypothalamic Hammartoma_**, and is treated with **_resection or ablation_**
47
What is this?
Hypothalamic Hammartoma
48
Workup for BRUE (6)
1. screen for GERD 2. urinalsysis / culture 3. (consider) neuroimaging) 4. (consider) EEG 5. pneumoimaging 6. WBC
49
Typical age range for febrile seizures
6 months to 5 years Peak 1-2 y.o.
50
Seizure auras and location dysphasic psychic dysmnesic aura cognitive psychic aura Illusions
1. dys**_p_**hasic aura = left **_p_**erisylvian area 2. dys**_m_**nesic aura = **_m_**esobasal 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
Key factor differentiating CDLK5 from Rhett Syndrome (2)
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
Common Progressive myoclonic epilepsies and their respective chromosomes
"SLUMiN it" Sialidosis (6p) Lafora disease (6p - EPM2B and 6q - EPM2A) Unverricht-Lundborg disease (EPM1 21q) MERRF (mitochondrial lysine) NCL (multiple genes / chromosmes)
53
Unverricht-Lundborg Disease Inheritance Defect in cystatin B on chromosome 21 age of onset Additional features (2)
"_U_, **_L_**ittle _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 SL**_U_**MiN it later on Autosomal recessive age 6-18 ("little sis") defect in **_cys_**tatin 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 **_SLUM_**i_N_)
54
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?
Cimetidine (transient) erythromycin Grapefruit juice
55
Hallmark features of Autosomal Dominant Epilepsy with Auditory features as well
Auditory phenomena (Humming, buzzing,,,,,,,, ringing) Receptive aphasia
56
Medications to avoid with epilepsy
there's a LOAD of medicines that cause seizures to HATCH * *_L_**ithium * *_O_**piates * *_A_**ntidepressants * *_D_**opamine agonists **_H_**ypnotics **_A_**ntipsychotics **_T_**riptans _C_**_H_**olinesterase Inhibitors

Peds neuro boards, V2 (29 decks)

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