🔷 Epileptic Encephalopathies (EE)

Definition

• A group of severe childhood epilepsies where epileptic activity itself causes progressive cognitive, behavioral, or developmental decline
• Seizures + EEG abnormalities are toxic to the brain

⭐ Core Features

• 👶 Early onset (infancy/early childhood)
• ⚡ Frequent, severe, drug-resistant seizures
• 📈 Abundant interictal discharges
• 🧠 Developmental delay/regression
• 📉 Abnormal background EEG
• 🎗️ High risk of long-term neurocognitive disability

🍼 1. Ohtahara Syndrome (Early Infantile EE)

• ⏳ Neonatal onset
• ⚡ Tonic spasms, frequent
• 📉 EEG: Burst–suppression
• 🔄 Evolves to West syndrome

Treatment

• 💉 High-dose steroids
• 💊 Vigabatrin (TS)
• 🍽️ Ketogenic diet
• Levetiracetam, topiramate
• 🔪 Surgery (focal dysplasia)

👶 2. West Syndrome (Infantile Spasms)

Triad

• ⚡ Epileptic spasms
• 🧠 Developmental regression
• 📉 Hypsarrhythmia

Onset: 3–12 months

Treatment

• ⭐ ACTH / high-dose prednisolone
• 💊 Vigabatrin (TS)
• 🍽️ Ketogenic diet
• Levetiracetam, topiramate
• 🧪 Treat metabolic causes (B6, PLP, biotin)

🧩 3. Lennox–Gastaut Syndrome (LGS)

• ⏳ Onset: 1–8 years
• ⚡ Seizures: atonic, atypical absences, tonic, myoclonic
• 📉 EEG:
  • <3 Hz slow spike-wave
  • Paroxysmal fast activity in sleep

Treatment

• Valproate
• Clobazam
• Lamotrigine
• Rufinamide
• Topiramate
• 🍽️ Ketogenic diet
• 🔌 VNS
• ✂️ Corpus callosotomy

🔥 4. Dravet Syndrome

• 🧬 SCN1A mutation
• Onset after prolonged febrile seizure
• Fever-sensitive, drug-resistant

❌ Avoid sodium channel blockers:
CBZ, LTG, PHT

Treatment

• Valproate
• Clobazam
• Topiramate
• Stiripentol
• Cannabidiol
• Fenfluramine
• 🍽️ Ketogenic diet

🌙 5. Doose Syndrome (Myoclonic–Astatic Epilepsy)

• Onset 2–6 years
• Myoclonic, atonic, absence seizures
• EEG: 2–3 Hz spike-wave

Treatment

• Valproate
• ⭐ Ketogenic diet (most effective)
• Levetiracetam
• Clobazam

🌙 6. CSWS / ESES

• ⭐ Spike-wave index >85% during sleep
• Language regression, cognitive decline
• Onset 2–8 years

Treatment

• High-dose steroids / IVMP
• Clobazam, diazepam
• Valproate
• Levetiracetam
• Sulthiame
• 🔪 Surgery if focal lesion

🗣️ 7. Landau–Kleffner Syndrome (LKS)

• 🎧 Acquired aphasia + auditory agnosia
• Abnormal EEG during sleep

Treatment

• Steroids
• Benzodiazepines
• Valproate
• Speech therapy
• Subpial transections (refractory)

🧬 8. Metabolic / Pyridoxine-Dependent EE

• Neonatal refractory seizures
• Consider metabolic causes

Workup / Treatment

• Pyridoxine (B6) trial
• Pyridoxal phosphate
• Folinic acid
• Biotin (biotinidase deficiency)

💡 High-Yield Treatment Principles

• ⚡ Treat early to prevent developmental harm
• 🔬 Evaluate metabolic and genetic causes
• ⭐ ACTH/steroids for spasms
• 🍽️ Ketogenic diet for drug-resistant EE
• 🔪 Surgery for focal structural lesions
• ❌ Avoid sodium channel blockers in SCN1A disorders