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Childhood epilepsy syndromes
In children each epilepsy syndrome is defined by age, seizure type and EEG, and the drug choice depends on it: infantile spasms — urgent, ACTH or steroids and vigabatrin; absence — ethosuximide; rolandic — usually no treatment; JME — lifelong, and some drugs make it worse.
Why it matters in the exam
Infantile epileptic spasms (West syndrome): an infant aged 3–12 months with brief contractions in clusters, usually on waking, and developmental regression; the EEG shows hypsarrhythmia. This is urgent — delayed treatment harms development. Treatment: ACTH or high-dose prednisolone, and vigabatrin is first choice in tuberous sclerosis, in which ash-leaf macules are sought with a Wood's lamp.
Childhood absence epilepsy: a girl aged 4–10 with "staring" spells of seconds many times a day, triggered by hyperventilation, and an EEG with 3 Hz spike-wave; ethosuximide is first choice when there are only absences. Carbamazepine and oxcarbazepine worsen absence and JME. Rolandic epilepsy (with centrotemporal spikes): a child aged 3–13 with nocturnal focal seizures of the face and throat, drooling and speech arrest with preserved awareness; it remits by puberty and often needs no treatment.
JME: an adolescent with morning myoclonic "jerks" (drops the cup) and generalised seizures after sleep deprivation or alcohol; lifelong treatment, valproate is effective but in girls of childbearing age levetiracetam or lamotrigine is preferred because of teratogenicity. Lennox-Gastaut: many seizure types including tonic, slow spike-wave and intellectual disability. Dravet (SCN1A): prolonged, one-sided febrile seizures in the first year; sodium channel blockers worsen it. In a prolonged seizure: a benzodiazepine after 5 minutes (buccal, intranasal or IM midazolam, or IV lorazepam 0.1 mg/kg), and if it continues — levetiracetam 60 mg/kg, fosphenytoin 20 mg PE/kg or valproate 40 mg/kg.
Key points
- West: 3–12 months, hypsarrhythmia, urgent treatment.
- Absence: 3 Hz, ethosuximide.
- Rolandic: nocturnal facial seizures, usually no treatment.
- JME: morning jerks, lifelong treatment.
- Seizure over 5 minutes → benzodiazepine, then second line.
Summary table
| Syndrome | Age and picture | EEG | Treatment |
|---|---|---|---|
| Infantile spasms (West) | 3–12 months, clusters of spasms on waking, regression | Hypsarrhythmia | ACTH or prednisolone; vigabatrin (first in tuberous sclerosis) |
| Childhood absence | 4–10, many brief stares, triggered by hyperventilation | 3 Hz spike-wave | Ethosuximide; valproate or lamotrigine |
| Rolandic (centrotemporal spikes) | 3–13, nocturnal facial seizures, drooling, speech arrest | Centrotemporal spikes | Usually no treatment; remits by puberty |
| JME | Adolescents, morning jerks, generalised seizures after sleep loss | 4–6 Hz polyspikes | Levetiracetam, lamotrigine or valproate; lifelong |
| Lennox-Gastaut | Childhood, tonic and multiple seizures, intellectual disability | Slow spike-wave | Multiple drugs; treatment-resistant |
| Dravet | First year, prolonged one-sided febrile seizures | Normal at first; SCN1A | Avoid sodium blockers; stiripentol, fenfluramine, cannabidiol |
A free exam question
אצל ילוד בן שבוע שנולד במועד ש מעקב ההיריון שלו היה דל, הופיעו .פרכוס כללי ופריחה פטכיאלית בבדיקה גופנית לציין צהבת ,הפטוספלנומגליה, ו כוריורטיניטיס. בב דיקות העזר לציין הידרוצפלוס ב- US מוח והסתיידויות אינטרה- ( קרניאליות intra-cranial calcifications ) ב- MRI איזה מבין הבאים הוא המחולל הסביר ביותר של ?מחלתו (the question as examined, in Hebrew)
- א. Herpes simplex virus
- ב. Toxoplasmosis
- ג. Treponema pallidum
- ד. Zika virus
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Sources
- ILAE Classification and Definition of Epilepsy Syndromes with Onset in Neonates and Infants, and in Childhood, 2022 — Syndrome definitions
- American Epilepsy Society Guideline: Treatment of Convulsive Status Epilepticus in Children and Adults, 2016 (with ESETT 2019 evidence) — Benzodiazepines and second-line agents
- Nelson Textbook of Pediatrics, 22nd edition (2024) — Seizures in Childhood
Sources are given for further reading; the text is written afresh and not copied from them.
Reviewed by the Examqo AI Professor · 2026-11-06. The text may contain errors; when in doubt, check the source.