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Neurology Boards > Peds Epilepsy > Flashcards

Peds Epilepsy Flashcards

1
Q

Benign Familial Neonatal Seizures

  1. Classification
  2. Clinical Picture
  3. Genetic Association
  4. EEG
  5. Treatment
  6. Prognosis
A

“Fifth Day Fits”

  1. Classification - Partial/Generalized
  2. Clinical Picture - Onset: First wk after birth, CP: Clonic or Myoclonic Seizures
  3. Genetic Association- KCNQ2, KCNQ3 mutation
  4. EEG - Focal or multifocal
  5. Treatment - No tx
  6. Prognosis - Usually stop by 6wks, 16% risk of epilepsy
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2
Q

Benign Familial Infantile Seizures

  1. Classification
  2. Clinical Picture
  3. Genetic Association
  4. EEG
  5. Treatment
  6. Prognosis
A
  1. Classification - Partial
  2. Clinical Picture - Focal clonic, eye deviation, cyanosis; Seizures cluster
  3. Genetic Association - None
  4. EEG - Occipital-parietal spikes
  5. Treatment - No tx
  6. Prognosis - Excellent, resolves in 1-2 years
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3
Q

Benign Myoclonic Epilepsy of Infancy

  1. Classification
  2. Clinical Picture
  3. Genetic Association
  4. EEG
  5. Treatment
  6. Prognosis
A
  1. Classification - Generalized
  2. Clinical Picture - Onset: 6mo - 3yr, Brief Generalized Myoclonic activity
  3. Genetic Association - None
  4. EEG - Generalized spike/wave lasting 2-3 seconds
  5. Treatment - Valproate, Lamotrigine
  6. Prognosis - Remission in most cases
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4
Q

Early Myoclonic Encephalopathy

  1. Classification
  2. Clinical Picture
  3. Genetic Association
  4. EEG
  5. Treatment
  6. Prognosis
A
  1. Classification - Generalized
  2. Clinical Picture - Onset: First month of life, Starts with erratic Myoclonic jerks then simple focal seizures then infantile spasms, Multiple metabolic causes identified
  3. Genetic Association - None
  4. EEG - Burst- Suppression evolve to hypsarrhythmia
  5. Treatment - AED: Intractable
  6. Prognosis - poor, 50% died in few wks
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5
Q

Early Infantile Epileptic Encephalopathy (EIEE)/Othahara Syndrome

  1. Classification
  2. Clinical Picture
  3. Genetic Association
  4. EEG
  5. Treatment
  6. Prognosis
A

Early Infantile Epileptic Encephalopathy (EIEE)/Ohtahara Syndrome

  1. Classification - Generalized
  2. Clinical Picture
    - Tonic spasms (hundreds daily), 75% lesions in MRI
    - Starts with erratic Myoclonic jerks then simple focal seizure then infantile spasms
    - Multiple metabolic causes identified
  3. Genetic Association - None
  4. EEG - Burst-Suppression
  5. Treatment - Surgical evaluation
  6. Prognosis - Poor - Can evolve into West Syndrome or Lennox-Gastaut Syndrome
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6
Q

Infantile Spasms/ “West Syndrome”

  1. Classification
  2. Clinical Picture
  3. Genetic Association
  4. EEG
  5. Treatment
  6. Prognosis
A

Infantile Spasms/ “West Syndrome”

  1. Classification - Generalized
  2. Clinical Picture:
    Onset - Infancy
    Infantile Spasms - Sudden jack-knife movement with flexion of the neck, trunk, limbs, and waist occurring in clusters
  3. Genetic Association - N/A
  4. EEG - Hypsarrhythmia (high amplitude, chaotic slow waves with multi focal spikes), Electrodecrement during spasms
  5. Treatment - Corticotrophin, Vigabatrin
  6. Prognosis - Poor
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7
Q

Severe Myoclonic Epilepsy in Infancy / “Dravet Syndrome”

  1. Classification
  2. Clinical Picture
  3. Genetic Association
  4. EEG
  5. Treatment
  6. Prognosis
A
  1. Classification - Focal or Generalized
  2. Clinical Picture -
    Onset: First year, peak 2-8 mo
    Focal or GTC seizures, starts initially in setting of fever or vaccination
    Later: Myoclonic and absence seizures
    Developmental arrest with onset of seizures
  3. Genetic Association - SCN1A mutation
  4. EEG - Slow background, multifocal or generalized spike-and-wave
  5. Treatment - Valproate, Clobazam, Ketogenic Diet
  6. Prognosis - Poor
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8
Q

Benign Epilepsy with Centrotemporal Spikes/“Rolandic Epilepsy”

  1. Classification
  2. Clinical Picture
  3. Genetic Association
  4. EEG
  5. Treatment
  6. Prognosis
A
  1. Classification - Partial
  2. Clinical Picture:
    Onset: School age, 15-25% of epilepsy in children
    CP: Nocturnal SPS with clonic contractions of upper face and upper limb, excessive salivation, gurgling or choking sounds
  3. Genetic - None
  4. EEG - Centrotemporal spikes with normal background
  5. Treatment - No treatment, CBZ if frequent seizure
  6. Prognosis - Resolves by puberty 2/3 infrequent seizures
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9
Q

Childhood Epilepsy with Occipital Paroxsyms / Panayiotopoulos

  1. Classification
  2. Clinical Picture
  3. Genetic Association
  4. EEG
  5. Treatment
  6. Prognosis
A
  1. Classification - Partial
  2. Clinical Picture -
    Onset: 1-14 average, 5 years
    CP: Nocturnal autonomic seizures of prolonged duration. Child is conscious but complains about feeling sick, vomits, turns pale, dilated pupils +/- visual seizures, thermoregulatory alterations
  3. Genetic Association - None
  4. EEG - Interictal occipital spikes, Increase in non-REM sleep
  5. Treatment - No treatment needed
  6. Prognosis - 20% develop ictal syncope w/ and wo convulsions
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10
Q

Idiopathic Childhood Occipital Epilepsy of Gastaut

  1. Classification
  2. Clinical Picture
  3. Genetic Association
  4. EEG
  5. Treatment
  6. Prognosis
A
  1. Classification - Partial
  2. Clinical Picture -
    Onset: 3–14 years, Average 8 years
    CP: Episodic blindness or colored luminous discs, visual hallucinations, lasting seconds or minutes; post-ictal migraine in 1/3
  3. Genetic Association - None
  4. EEG - Interictal high amplitude spike and wave complexes occurring with the eyes closed
  5. Treatment - CBZ
  6. Prognosis - 50% with positive FH of epilepsy
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11
Q

Acquired Epileptic Aphasia / “Landau-Kleffner Syndrome”

  1. Classification
  2. Clinical Picture
  3. Genetic Association
  4. EEG
  5. Treatment
  6. Prognosis
A
  1. Classification - Partial
  2. Clinical Picture
    Onset: 3-8 years old
    Focal seizure involving face or arm usually diagnosed initially as BECTS then child develop acquired motor and sensory aphasia
  3. Genetic Association - Bilateral Centrotemporal spikes, increased during sleep
  4. EEG - Valproate, LTG, Steroids. Avoid CBZ & PHT
  5. Treatment - Variable - many children become permanently aphasic
  6. Prognosis
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12
Q

Continuous Spike - Wave Activity during Sleep (CWSW)

  1. Classification
  2. Clinical Picture
  3. Genetic Association
  4. EEG
  5. Treatment
  6. Prognosis
A
  1. Classification - ?
  2. Clinical Picture -
    Onset: 5 years
    CP: Starts with partial or generalized seizures then develop epileptic encephalopathy
  3. Genetic Association
  4. EEG - ESES appears 2-3 years after onset
  5. Treatment - Valproate, Lamotrigine, Steroids
  6. Prognosis - ?
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13
Q

Lennox-Gastaut Syndrome

  1. Classification
  2. Clinical Picture
  3. Genetic Association
  4. EEG
  5. Treatment
  6. Prognosis
A
  1. Classification - Generalized
  2. Clinical Picture - Multiple seizures types (Tonic, Atonic, Absence), MR, Slow Spike Wave (1.5 - 2.5 Hz)
  3. Genetic Association - None
  4. EEG - Interictal: slow spikes and waves, <2.5Hz
  5. Treatment - Valproate for all seizure types, LTG & Felbamate for drop attacks
  6. Prognosis - Poor
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14
Q

Myoclonic-astatic Epilepsy / “Doose Syndrome”

  1. Classification
  2. Clinical Picture
  3. Genetic Association
  4. EEG
  5. Treatment
  6. Prognosis
A
  1. Classification - Generalized
  2. Clinical Picture - Myoclonic, atonic, tonic and absence seizures; Seizures may start in the setting of infection/fever
  3. Genetic Association - None
  4. EEG - Slow background, generalized spike and wave pattern
  5. Treatment - Valproate, LTG, LEV, Ketogenic diet
  6. Prognosis - Poor
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15
Q

Childhood Absence Epilepsy

  1. Classification
  2. Clinical Picture
  3. Genetic Association
  4. EEG
  5. Treatment
  6. Prognosis
A
  1. Classification - Generalized
  2. Clinical Picture - Brief (few seconds) staring episodes with behavioral arrest; May have automatisms. No post ictal state
    Provoked by HV
  3. Genetic Association -
  4. EEG - 3Hz spiel and wave pattern
  5. Treatment - Ethosuximide, Valproate, LTG. D/C if seizures
  6. Prognosis - Excellent, Remits by teen years
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16
Q

Generalized epilepsy with febrile seizures plus (GEFS+)

  1. Classification
  2. Clinical Picture
  3. Genetic Association (4)
  4. EEG
  5. Treatment
  6. Prognosis
A
  1. Classification - Generalized
  2. Clinical Picture - Febrile seizures, Myoclonic, a static, tonic-clonic, absence
    Strong family hx with variable penetrance
  3. Genetic Association - AD, SCNA1, SCN1B, SCN2A, GABARG2
  4. EEG - Normal, generalized or focal spike and wave
  5. Treatment
  6. Prognosis - Variable presentation within families from benign to catastrophic
17
Q

AD Nocturnal Frontal Lobe Epilepsy

  1. Classification
  2. Clinical Picture
  3. Genetic Association
  4. EEG
  5. Treatment
  6. Prognosis
A
  1. Classification - Partial
  2. Clinical Picture -
    Onset: Variable, Infants to Elderly
    CP: Brief hypermotor seizures (identical to those from SMA) that tend to cluster and occur mainly during sleep. Includes fist clenching, arm throwing, leg cycling, yelling, moaning, sleep walking
  3. Genetic Association - CHRNA (nicotinic Ach Rc)
  4. EEG - Frontal SW
  5. Treatment - Nictoine patch, Carbamazepine, Clonazepam
  6. Prognosis - Can be misdiagnosed as night terrors or somnambulism
18
Q

Eye lid Myoclonic with absences (Jeavons Syndrome)

  1. Classification
  2. Clinical Picture
  3. Genetic Association
  4. EEG
  5. Treatment
  6. Prognosis
A
  1. Classification - Generalized
  2. Clinical Picture
    - Onset: around 6 yo
    - CP: Eyelid Myoclonic (jerking eyelid w/ jerky deviation of eyeballs & head for seconds) with and without absences; eye closure-induced seizures and EEG paroxysms; photosensitive
  3. Genetic Association: n/a
  4. EEG: Brief 3 to 6Hz, Generalized poly-spike and wave discharge
  5. Treatment : Valproate, clonazepam, levetiracetam
  6. Prognosis: N/A
19
Q

Juvenile Myoclonic Epilepsy

  1. Classification
  2. Clinical Picture
  3. Genetic Association
  4. EEG
  5. Treatment
  6. Prognosis
A
  1. Classification - Generalized
  2. Clinical Picture -
    - Onset: teenage (8-24), Myoclonic seizures on awakening; GTC - absence seizure; Myoclonus can be triggered by reading, talking, photic stimulation
  3. Genetic Association : None
  4. EEG: Generalized 4-6 Hz polyspike and wave discharges in 75% patients
  5. Treatment: Valproate, LTG, LEV, Topiramamte, ZNS
  6. Prognosis : Good but requires lifelong treatment
20
Q

Idiopathic Photosensitive Occipital Lobe Epilepsy

  1. Classification
  2. Clinical Picture
  3. Genetic Association
  4. EEG
  5. Treatment
  6. Prognosis
A
  1. Classification: Reflex epilepsy
  2. Clinical Picture:
    Onset: During puberty
    Occipital lobe seizures provoked by visual stimuli (TV, video games)
  3. Genetic Association : N/a
  4. EEG: Occipital or generalized spike and wave discharges enhanced with eye closure
  5. Treatment: avoid triggers, VPA
  6. Prognosis: Good
21
Q

Rasmussen Encephalitis

  1. Classification
  2. Clinical Picture
A
  1. Classification: Partial
  2. Clinical Picture:
    - Intractable and progressive focal seizures, hemiparesis, cognitive regression
    - Slowly progressive cortical atrophy on MRI
    - Ab to glutamate receptor 3
22
Q

Autosomal dominant frontal lobe epilepsy

  1. Classification
  2. Clinical Picture
  3. Genetic Association
  4. EEG
  5. Treatment
  6. Prognosis
A
  1. Classification: Partial
  2. Clinical Picture:
    Hyperkinetic seizures at sleep week transition. May have aura of fear
  3. Genetic Association: CHRNA4, CHRNB2
  4. EEG: Normal or frontal spikes
  5. Treatments: Carbamazepine
  6. Prognosis: Good response to treatment
23
Q

Name 4 seizures that start in the setting of viral illness or fever?

A
  1. Febrile
  2. GEFS+
  3. Dravet
  4. Doose
24
Q

Name 3 seizures that are associated with spasms?

A
  1. Ohtahara
  2. West
  3. AICARDI
25
Q

What are the causes of Myoclonic seizures in children?

A

Early Myoclonic encephalopathies (EME, EIEE)
Part of generalized epileptic syndrome (Doose, Dravet, LGX)
Non-Progressive Myoclonic epilepsies (Benign neonatal Myoclonic epilepsy, familial Myoclonic epilepsy)
Progressive Myoclonic epilepsies

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