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

Epilepsy syndrome

View Details
logo
Parent item
Seizure classification
Sub-item
Epilepsy syndrome on neonatal period
Epilepsy syndrome on infancy
Epilepsy syndrome on childhood
Epilepsy syndrome on adolescence/adulthood

General

  • An epilepsy syndrome represents a complex of clinical features, signs and symptoms that together define a distinctive, recognizable clinical seizure disorder
  • Some syndromes are highly correlated with a single specific aetiology (eg, SCN1A mutations in Dravet syndrome), whereas others may be due to a broad range of causes (eg, West syndrome or Lennox-Gastaut syndrome).
  • Many syndromes can be identified based on age at onset, seizure type(s), electroencephalogram (EEG) characteristics, aetiology, and associated comorbidities.
  • Epilepsydiagnosis.org has parameters for diagnosis and videos

Paediatrics epilepsy syndrome

Type
Presentation
Pathognomonic EEG
Treatment
Juvenile myoclonic epilepsy
Myoclonic jerks after waking.
- Absence seizures may present years before myoclonic jerks.
- Generalized tonic clonic seizures may present years after myoclonic jerks.
Usually presents in puberty and may be inherited.
EEG: Polyspike discharge (4–6 Hz) and slow waves
1. Valproic acid (risk of teratogenicity in pregnant women)
2. Alternatives: levetiracetam, lamotrigine, topiramate
West syndrome/infantile spasms
- Salaam seizures (rapid flexion of head, torso, limbs/less often extension)
- Associated with cognitive impairment.
- Usually presents in first year of life.
- Must exclude tuberous sclerosis.
EEG: Hypsarrhythmia (very disorganized background activity + high-amplitude irregular spikes waves)
1. ACTH (high CRH is a possible cause, with ACTH and steroids possibly suppressing them)
2. Steroids
Lennox–Gastaut syndrome
- Usually atonic/drop attacks but also tonic and other types
- Cognitive impairment
- Children
- Exclude tuberous sclerosis
- Up to 50 seizures/d
EEG: Slow background rhythm + spike-wave bursts (frequency < 2.5 Hz)
1. Drug resistant (try valproic acid, lamotrigine, topiramate)
2. Vagus nerve stimulator
3. Callosotomy (reduces drop attacks)
Rasmussen’s syndrome
- Seizures + one enlarged hemisphere
- Encephalitis (probably autoimmune)
- Children
1. Various, nonspecific, findings related to clinical progression
2. EEG: Persistent high-amplitude delta activity in inflamed hemisphere
1. Frequently resistant to antiepileptics
2. Steroids
3. IV immunoglobulins/plasmapheresis
4. Hemispherectomy

4 stages of development

  • Infancy
    • West syndrome/infantile spasms
      • Infantile spasm/hyperarrthymias/mental retardation
  • Childhood
    • Lennox-Gastaut syndrome: Drop attacks atonic seizures
    • Landau-Kleffner syndrome : Aphasia/neuropsych deterioration → seizure
    • Childhood absence epilepsy: frequent seizures impair attention and learning and may lead to an inaccurate diagnosis of attention-deficit disorder or daydreaming
    • Epileptic encephalopathy with continuous spike-and-wave during sleep (CSWS): seizure then neuropsych deterioration
    • Benign focal epilepsy with centrotemporal spikes (BECTS): seizure at night or before waking
  • Adolesent/adult
    • Juvenile myoclonic epilepsy
      • 3 phases over years: absence/myoclonic jerks/tonic-clonic
    • Juvenile absence epilepsy
      • Absence seizures triggered by hyperventilate and lack of sleep
notion image