Epilepsies of childhood

epidemiology
1. incidence= 0.5%
  1. but commoner after the 1st year of life
2. prevalence= 0.5%
chronic neurological disorder
1. recurrent unprovoked seizures
  1. transient signs & sx
    1. assoc w/ abnormal, excessive or synchronous neuronal activity
international classification of epilepsy
1. generalised
  1. discharge arises from both hemispheres
    1. bilat synchronous seizure discharge on EEG or varying asymmetry
  2. absence
    1. transient loss of consciousness
      1. w/ an abrupt onset & termination
      2. w/ some flickering of eyelids & minor alteration in mm tone
    2. typical (petit mal)
    3. atypical
    4. precipitating factors
      1. hyperventilation
  3. myoclonic
    1. brief, often repetitive, jerking movements
      1. limbs
      2. neck
      3. trunk
    2. non-epileptic movements
      1. seen physiologically in hiccoughs
  4. tonic-clonic
    1. rhythmic contraction of mm groups ff the tonic phase
    2. in rigid tonic phase (1)
      1. fall to ground
        injury
      2. don't breathe
        become cyanosed
    3. clonic phase (2)
      1. limb jerking
      2. irregular breathing
      3. cyanosis persists
      4. saliva may build up in mouth
      5. tongue biting
      6. urinary incontinence
      7. duration: few seconds to mins
        fb unconsciousness or deep sleep for several hours
  5. atonic
    1. often combined w/ a myoclonic jerk
      1. fb transient loss of muscle tone
        -> sudden fall to the floor or drop of the head
  6. no warning
  7. symmetrical seizure
  8. tonic
    1. generalised increase in tone
2. focal
  1. where seizures arise from 1 or part of 1 hemisphere
    1. manifestations depend on part of the brain where discharge originates
      1. frontal lobe seizures
        involve motor or premotor cortex
        motor phenomena
        may -> clonic movements
        may travel proximally (Jacksonian march)
        asymmetrical tonic seizures can be seen
        hyperkinetic
        can be mistaken for non-epileptic events
        atonic seizures
        may arise from mesial frontal discharge
      2. temporal lobe seizures
        commonest of all epilepsies
        may result in strange warning feelings
        or aura w/
        smell
        taste abnormalities
        sound & shape distortions
        ff spread to pre-motor cortex
        automatisms
        lip-smacking
        plucking @ one's clothing
        walking around in a non-purposeful manner
        deja-vu
        intense feeling of having been in same situation before
        jamais-vu
        never having been in a situation before
        impaired consciousness
        length of event longer than a typical absence
      3. occipital seizures
        cause distortion of vision
      4. parietal lobe seizures
        cause contralat dysaesthesias (altered sensation)
        distorted body
  2. consciousness may be retained or lost
  3. seizure may be followed by generalised tonic-clonic seizure
3. can be unclear esp in < 5 years
diagnosis
1. based on hx
  1. child
  2. eye witnesses
  3. triggers
  4. impairments
    1. educational
    2. psychological
    3. social
2. video
3. examination
  1. skin markers
    1. neurocutaneous syn
    2. neurological abnormalities
4. epilepsy usually has idiopathic cause
  1. but may be presentation or complication
    1. of an underlying neurological disorder
Ix
1. EEG
  1. indication
    1. whenever epilepsy suspected
  2. interpreted to ID a background that is abnormal for child's age
    1. asymmetry or slowing
      1. underlying structural abnormalities
    2. evidence of neuronal hyperexcitability
      1. sharp waves
      2. spike-waves complexes
  3. unless seizure is captured
    1. only adds supportive evidence or not for the diag
  4. many kids w/ have a normal initial EEG
    1. and vice versa
  5. if standard EEG is normal
    1. sleep or sleep-deprived record can help
  6. 24h ambulatory EEG
  7. video-telemetry
  8. subdural electrodes
    1. for assessment prior to surgery
2. structural imaging
  1. e.g. MRI & CT scans
  2. not routinely required for childhood generalised epilepsies
  3. indications
    1. neurological signs btw seizures
    2. focal seizures
    3. those indicated to ID tumour, vascular lesion or area of sclerosis
  4. MRI FLAIR
    1. better detect mesial temporal sclerosis in temporal lobe epilepsy
3. functional imaging
  1. to detect abnormal hypometabolism
    1. suggestive of seizure foci
  2. PET
  3. SPECT
  4. can use alongside
    1. psychological testing
      1. including memory assessment
      2. minimise risk of postop impairment
4. metabolic ix
  1. indications
    1. developmental regression
    2. seizures related to feeds or fasting
5. genetic studies
  1. due to genetic deletions
    1. causing abnormalities of Na+ & other ion chanels
      1. e.g. SCN1A mutations in severe myoclonic epilepsy of infancy
Mx
1. explanation and advice
  1. to help adjustment to the condition
    1. psychological help
  2. those w/ photosensivity
    1. sit at a distance from TV
2. specialist epilepsy nurse
  1. education & advice
    1. lifestyle issues
      1. adolescents
        driving
        only after 1 year free of seizures
        contraception
        pregnancy
        adherence
        seizure precipitants
        alcohol
        poor sleep routines
3. Rx
  1. decision to treat
    1. based on level of inconvenience seizures bring
    2. DON'T institute after single unprovoked seizure
  2. Anti-epileptic drug (AED)
    1. not all seizures require AED
      1. base this decision on
        seizure type
        frequency
        social & educational conseq
        possible s/e of drugs
    2. choose the approp drug for the seizure
      1. inapprop AEDs may be detrimental
        carbamazepine can worsen absence & myoclonic seizures
    3. monotherapy at minimum dosage is desired
    4. drug levels not measured routinely
    5. kids w/ prolonged seizures are given rescue therapy
      1. e.g. rectal diazepam & buccal midalzolam
    6. therapy can usually be discontinued after 2 years free of seizures
  3. tonic-clonic
    1. 2nd line
      1. lamotrigine, topiramate
    2. 1st line
      1. valproate, carbamazepine
  4. absence
    1. 1st line
      1. valproate, ethosuximide
    2. 2nd line
      1. lamotrigine
  5. myoclonic
    1. 1st line
      1. valproate
    2. 2nd line
      1. lamotrigine
  6. focal
    1. 1st line
      1. carbamazepine, valproate
      2. lamotrigine
        most effective
        slow titration
    2. 2nd line
      1. topiramate, levetiracetam, oxacarbazepine, gabapentin, tiagabine, vigabatrin
  7. ketogenic (fat-based) diets
    1. helpful in some kids
    2. mechanism of action poorly understood
  8. vagal nerve stimulation
    1. delivered using externally programmable stimulation
      1. wire around vagal nerve
        on trial
  9. surgery
    1. well localised seizures
      1. demonstrated by good concordance btw EEG, MRI & functional imaging findings
    2. temporal lobectomy
      1. for mesial temporal sclerosis
    3. hemispherotomy or hemispherotomy
      1. isolation of hemisphere which isn't removed so as avoid post-op in space
4. promote independence & confidence
5. informing the school
  1. vigilance for absence episodes
  2. avoiding deep baths, not swimming alone in deep water

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Epilepsies of childhood

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	Created by v.djabatey over 10 years ago