This handout is for infantile epileptic spasms syndrome (west syndrome). Your care team identified this based on: clusters of brief flexor / extensor / mixed spasms in infant 4-7 mo, often on awakening (go aan/cns 2012 pmid 22689735).
Other reasons your team may use this plan: developmental regression or arrest in infant 3 mo - 2 yr — infantile spasms differential (go aan/cns 2012); hypsarrhythmic (chaotic high-voltage interictal) eeg pattern in infant (go aan/cns 2012); hypopigmented macules (tsc) + spasms in infant — tsc-associated infantile spasms (go aan/cns 2012; iciss o'callaghan 2017).
Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.
| Medication | Starting dose | How | When | What it does |
|---|---|---|---|---|
| corticotropin | ACTH (H.P. Acthar Gel) 75-150 U/m²/day IM divided BID × 2 weeks, then taper × 2 weeks. Low-dose 20 U IM daily may be equivalent to high-dose per Go AAN/CNS 2012 (Go AAN/CNS 2012 PMID 22689735) | IM | BID × 2 wk then taper × 2 wk | Go AAN/CNS 2012 PMID 22689735 — ACTH more effective than vigabatrin short-term (excluding TSC); low-dose probably as effective as high-dose; hormonal therapy may be preferred over VGB in cryptogenic to improve developmental outcome (UKISS Lux 2005 PMID 16239177 cryptogenic VABS benefit at 14 mo) |
| prednisolone | 40-60 mg/day PO divided BID-QID × 2 weeks, then taper × 2 weeks (UKISS protocol). Some centers use up to 8 mg/kg/day (Go AAN/CNS 2012; Lux UKISS 2005 PMID 16239177) | PO | BID-QID × 2 wk then taper × 2 wk | Lux UKISS 2005 PMID 16239177 — prednisolone is an effective hormonal alternative to ACTH (similar short-term efficacy in UKISS); cheaper + easier to administer + oral; UKISS used 40 mg/day BID-QID × 2 wk then taper × 2 wk (UKISS Lux 2005 PMID 16239177) |
| vigabatrin | 50 mg/kg/day PO divided BID, titrate over 1-2 weeks to 100-150 mg/kg/day (Go AAN/CNS 2012; Lux UKISS 2005) | PO | BID | Go AAN/CNS 2012 — vigabatrin first-line for TSC-associated IES (more effective in TSC than hormonal); used in combination with hormonal per ICISS O'Callaghan 2017 PMID 27838190 for 72% cessation rate (vs 57% hormonal alone); FDA black box for irreversible peripheral visual field loss — mandatory baseline + q3 mo ophthalmology |
| pyridoxine | 100 mg IV under continuous EEG monitoring (one-time trial); response within minutes confirms pyridoxine-dependent epilepsy. May be followed by 30 mg/kg/day PO maintenance if positive | IV then PO | single IV trial then daily PO if positive | AES 2016 Glauser PMID 26900382 + Go AAN/CNS 2012 — pyridoxine-dependent epilepsy mimics IES; B6 trial is low-cost / low-risk / high-impact when positive (response within minutes); document EEG burst-suppression before + after for definitive trial |
Plan: Infantile epileptic spasms syndrome — TIME-CRITICAL first-line (3 options + combination) (Go AAN/CNS 2012 PMID 22689735; ICISS O'Callaghan 2017 PMID 27838190)
Contact your care team if any of the following happen:
Call 911 or go to the nearest emergency room right away if you have:
Pediatric neurology / epileptology q1-3 mo; developmental pediatrics; early intervention; genetic counseling if positive; family education on safety / monitoring / triggers (Go AAN/CNS 2012; Lux UKISS 2005)
Guideline: Go AAN/CNS 2012 PMID 22689735 (evidence-based guideline update: medical treatment of infantile spasms) + Lux UKISS 2005 PMID 16239177 (UK Infantile Spasms Study) + Darke 2010 PMID 20457702 (UKISS 4-yr follow-up) + O'Callaghan ICISS 2017 PMID 27838190 (combination hormonal + vigabatrin)