Neurosurgery notes/Functional/Seizure/Seizure classification/Epilepsy syndrome/Epilepsy syndrome on childhood

Epilepsy syndrome on childhood

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Epilepsy syndrome

Lennox-Gastaut syndrome

  • Rare condition that begins in childhood as atonic seizures (“drop attacks”). → develops into tonic seizures with mental retardation.
  • S&S
    • Polymorphic,
    • Difficult to treat medically,
    • 50 per day.
    • May also present with status epilepticus.
  • EEG
      • notion image
  • Treatment
    • Valproic acid
      • 50% of patients have reduced seizures
    • Corpus callosotomy may reduce the number of atonic seizures.
    • Lennox-Gestaut is one of the few epilepsies particularly responsive to DBS of the centromedian nucleus of thalamus

Childhood absence epilepsy

  • SS
    • Brief (4-20 s) unresponsive, staring episodes
      • The frequent seizures impair attention and learning and may lead to an inaccurate diagnosis of attention-deficit disorder or daydreaming.
      • The majority have accompanying non-stereotyped motor activity (e.g. blinking, myoclonus of eyelid/mouth, oral automatisms, picking/rubbing movements of hands/feet) and are termed complex absence seizures.
  • EEG
      • Ictal EEG: Dome and spike 3 Hz
          • notion image
      • Interictal EEG: Normal
          • notion image
  • Numbers
    • 8-15% of all childhood epilepsies;
    • Onset 4-10 years;
    • History of febrile seizures in up to 30%,
    • Family history of epilepsy in 15%;
    • May be induced by hyperventilation.
  • Treatment
    • Ethosuximide
  • Outcome
    • Absence seizures usually remit in adolescence
      • But a significant number of children go on to have generalized tonic-clonic seizures as adults.

Landau-Kleffner syndrome

  • AKA: acquired epileptic aphasia
  • SS
    • Onset is usually acute and presents initially as difficulty comprehending language, followed by verbal agnosia.
    • Subsequently, expressive language is also affected and may lead to mutism.
      • In a child with previous normal language development,
    • Paroxysmal EEG abnormalities, and epileptic seizures.
    • Associated symptoms include
      • Psychomotor and behavioural disturbances consisting of
        • Motor hyperactivity
        • Impulsivity
        • Aggressive behaviour
  • Numbers
    • Slight male predominance
    • Presents at between 3 and 8 years of age
  • Investigation
    • EEG
      • Background activity during wakefulness is usually normal;
      • Severe and variable abnormalities during sleep.
    • Neuroimaging without any structural brain lesion is required for diagnosis
    • PET scans
      • Regions of hypometabolism predominantly in the temporal lobes
  • Treatment
    • Seizures are usually well controlled with traditional antiepileptic drugs but the neuropsychological manifestations have a more variable response.
    • Valproate, clobazam, and ethosuximide, either monotherapy or in polytherapy, are successful in controlling seizures and are occasionally successful in reversing language regression.
      • Carbamazepine, phenobarbital, and phenytoin can lead to worsening of seizures and conversion to electrical status epilepticus during slow sleep because they may increase cortical synchronization
    • Corticosteroids can provide dramatic improvement in language, cognition, and behaviour in some children.

Epileptic encephalopathy with continuous spike-and-wave during sleep (CSWS)

  • Seizures are the first symptom in 70-80% of children, followed by neuropsychological regression that may be global or selective regression of cognitive functions,
      • Features
      Landau Kleffner
      Epileptic encephalopathy with continuous spike and wave during sleep
      1st symptom
      Aphasia
      Seizure
      2nd symptom
      Seizure
      Aphasia/cognitive regression
  • Acquired aphasia characteristic of Landau-Kleffner syndrome is opposite where you get speech aphasia first then seizure

Benign focal epilepsy with centrotemporal spikes (BECTS)

  • Aka
    • Benign Rolandic epilepsy
  • Number
    • Most frequent focal epilepsy syndrome in childhood.
    • Age at onset ranges from 3 to 13 years of age
      • Peak incidence 8 years
  • SS
    • Seizures present usually at night, often shortly after falling asleep or before waking up.
    • The present as somatosensory aura with perioral paraesthesia's, a sensation of choking, and jaw or tongue stiffness.
    • Hemifacial or hemibody motor seizures, frequently with unilateral clonic, but also tonic or tonic-clonic, activity are seen in up to 34% of patients.
    • Motor features involve the face, lips, tongue, pharynx, and larynx, and this may be associated with speech arrest.
    • Generalized tonic-clonic seizures without focal onset have been described in 54%.
  • The characteristic interictal EEG finding is a distinct high-amplitude, diphasic spike with prominent after going slow wave typically in C3/C4 or T3/T4 electrodes.
      • Spikes are more frequently observed during sleep, and sleep activation of spikes is a salient feature of BECTS.
      notion image
  • Outcome
    • The condition remits spontaneously in almost all patients around the age of 16 years hence indications for treatment include seizures during daytime, repeated generalized tonic-clonic seizures, prolonged seizures, and status epilepticus as well as seizure onset before the age of 4 years.
  • Carbamazepine may control seizures in up to 65% of patients

Others

  • Febrile seizures plus (can start in infancy)
  • Panayiotopoulos syndrome
  • Epilepsy with myoclonic atonic (previously astatic) seizures
  • Benign epilepsy with centrotemporal spikes
  • Autosomal-dominant nocturnal frontal lobe epilepsy
  • Late-onset childhood occipital epilepsy (Gastaut type)
  • Epilepsy with myoclonic absences