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Epilepsy/Neurology
Epilepsy/Neurology Review for American Board of Pediatrics (ABP) Board Certification Exam
25
Medical
Professional
09/16/2012

Additional Medical Flashcards

 


 

Cards

Term
Which AED is most useful for both myoclonic jerks and convulsions in juvenile myoclonic epilepsy?
Definition
Valproic Acid (Depakote/Depakene)
Term

Which AED is useful only for generalized seizures, especially absence seizures in 

childhood absence epilepsy?

Definition
Ethosuximide
Term
What is the drug of choice for treatment of infantile spasms? 
Definition

Adrenocorticotropic hormone (ACTH) initially;

valproic acid for maintenance therapy 

Term

Benign Rolandic Epilepsy

(Benign Childhood Epilepsy with Centrotemporal Spikes)

Definition
  • Onset: 4-12 years (Peak: 8-10 years)
  • Infrequent Seizures
    • Face and arm/Sensorimotor
    • Nocturnal Generalized Tonic-Clonic (80% have only nighttime seizures)
 
EEG: Centrotemporal Spikes/Sharp Waves
 
Spontaneous remission by age 16
 
Good response to carbamazepine, oxcarbazepine or gabapentin, but Tx usually not necessary
Term
Drug of choice for Absence Seizure
Definition

Ethosuximide

(Can also use valproic acid)


Valproic Acid is preferred is generalized Tonic-Clonic seizures are also present

Term
Childhood Absence Epilepsy
Definition
  • School-Aged Onset
  • EEG: Generalized 3 Hz spike and wave
  • Good response to VPA, ESM, LTG, TPM, ZNS
  • May (usually) remit spontaneously

 

Term
Etiologic causes that may lead to infantile spasms include: (3)
Definition
  • Idiopathic
  • Genetic and Metabolic
  • Focal Brain Malformations
Term

Infantile Spasms

(West Syndrome if includes Dev. Delay)

Definition
  • Onset in first 2 years (usu. 3-9 months)
  • EEG: Hypsarrhythmia
  • Developmental plateau or regression
  • MR is part of def. of West Syndrome, but not all infants with infantile spasms and hypsarrhythmia have intellectual impairment
  • Tx: ACTH, VGB, VPA, Prednisone
Prognosis is better when no known etiology (40%)
Term
Juvenile Myoclonic Epilepsy
Definition
  • Onset Common in Adolescents (12-18 years)
  • Myoclonic and generalized TC sz ON AWAKENING
  • 10% of all epilepsies
  • Tx: Valproic Acid (Lamotrigine and Topiramate are newer alternatives)
  • Excellent Prognosis but Discontinuation of AEDs is NOT RECOMMENDED
Term

Initial AED choices for Focal Onset Sz

(Partial, Secondary Generalized)

Definition

CBZ, OXC

(LTG, LEV)

Term

Initial AED choices for Generalized Onset Sz

Tonic-Clonic, Myoclonic, Atonic/Tonic

Definition

VPA, LTG, TPM

(ZNS, FBM)

Term

Initial AED choices for Generalized Onset Sz

Absence

Definition
ESM, VPA, LTG
Term

Initial AED choices for Generalized Onset Sz

Infantile Spasms

Definition

ACTH, VGB

(TPM, ZNS)

Term
West Syndrome
Definition
  • Infantile Spasms (Hypasrrhythmia)
  • Mental Retardation
  • May be flexor (salaam, jackknife or head nodding), extensor, or mixed (Most Common)
  • Spasms usu. occur when awake, upon awakening, or in drowsiness period before sleep
  • Tx: ACTH or Prednisone
Prognosis is better when no known etiology
Term

West Syndrome

(Etiology)

Definition
  • Perinatal (hypoxia-ischemia, infection, prematurity or hemorrhage)
  • Genetic (Tuberous Sclerosis or Aicardi Syndrome)
  • Malformations of cortical development
  • Inborn errors (e.g.- PKU)
  • Idiopathic (40% of cases)
Testing: MRI, Dilated eye exam, Wood's lamp eval, Metabolic/Genetic eval

Prognosis is better when no known etiology
Term
Lennox-Gastaut Syndrome
Definition
  • Generalized Epilepsy (multiple types, including atonic or myoclonic attacks
  • Onset in childhood
  • MR
  • EEG: Generalized slow spike and slow wave complexes and background slowing
  • Seizures often intractable; may require several antiepileptic meds
  • Ketogenic diet or vagusnerve stimulation may be helpful
  • Tx: Valproic Acid (VPA)
Term
Temporal Lobe Epilepsy
Definition
  • Onset: School-age or teenage years
  • Typically Complex Partial with automatisms
  • EEG: Temporal Lobe Sharp Waves
  • May be intractable
  • Rate of spontaneous remission is low
  • MRI critical to rule out structural lesion/mass
  • Carbamazepine is first-line Tx
  • Refractory cases may be candidates for epilepsy surgery
Term

Initial AED choices for

Lennox-Gastaut Syndrome

Definition

Clonazepam

Valproate (VPA)

Term

Initial AED choices for

Juvenile Myoclonic Epilepsy

Definition
Valproate (VPA)
Term

Initial AED choices for

Benign Focal Epilepsy of Childhood

Definition

Carbamazepine (CBZ)

Gabapentin (GBP)

Term

Carbamazepine

(CBZ)

Definition
  • Best initial choice for simple or complex partial sz
  • Also effective for generalized tonic-clonic sz
  • Minimal adverse effects on cognition
  • Drawbacks: Short half-life; lack of IV form
  • Adverse Effects
    • Bone Marrow Suppression (w/ granulocytopenia)
    • hyponatremia (2o to SIADH)
    • Dosage related effects
      • ataxia, diplopia, lethargy
Term

Ethosuximide

(ESM)

Definition
  • Initial Tx of absence seizures
  • VPA more appropriate is GTC sz also present
  • Advantages:
    • Few serious side effects (rare bone marrow suppression)
    • Long half-life
  • Drawbacks:
    • GI Upset
    • Possibility of some cognitive effects
Term

Felbamate

(FBM)

Definition
  • Broad-spectrum activity against partial sz, 2o generalized TC sz, and the multiple generalized sz types of Lennox-Gastaut
  • Strikingly free of adverse effects on cognition or alertness
  • RISK OF FATAL APLASTIC ANEMIA OR HEPATOTOXICITY
  • CBC and LFT every 2 weeks
  • Decreases clearance of PHT, VPA, CBZ epoxide (metabolically-active metabolite of CBZ)
  • FBM clearance increased by CBZ and PHT
  • FBM clearance decreased by VPA
Term

Gabapentin

(GBP, Neurontin)

Definition
  • Partial and secondarily generalized TC seizures
  • Low toxicity; minimal cognitive impairment
  • No drug-drug interactions (not plasma bound; excreted unmetabolized by the kidneys)
  • Adverse Effects:
    • Transient dizziness
    • Lethargy
    • Ataxia
    • Weight gain
    • Paradoxical aggression
Term

Lamotrigine

(LTG, Lamictal)

Definition
  • Effective against partial or secondarily generalized TC seizures, absence seizures, and generalized seizure-types, including Lennox-Gastaut
  • Low toxicity profile; minimal cognitive impairment
  • Adverse effects
    • Lethargy, Ataxia, HA
    • SEVERE skin rash (SJS) in as many as 1/100 (esp if on VPA or LTG introduced quickly)
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