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peds.infantile-spasms.v1PRODUCTION
peds.infantile-spasms.v1

Infantile epileptic spasms syndrome (West syndrome)

pediatricssubacutepediatric
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Encounter flow

12/12 authored

Canonical 12-phase frame with authored status for this dossier.

Current phase

Frame

Detailed

Confirm clinical triad: epileptic spasms (clusters, often on awakening) + hypsarrhythmic EEG + developmental regression / arrest; peak 4-7 mo (Go AAN/CNS 2012 PMID 22689735)

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Advance rule
Set
Advance when

Triad confirmed + EEG ordered + pediatric neurology notified

Patient inputs (14)

All AED dosing weight-based (AAP Red Book 2024-2027, Lexicomp Peds)

Preterm / HIE / NICU / TORCH infections raise probability of symptomatic IES (Go AAN/CNS 2012)

Cryptogenic IES has normal pre-onset development; regression / plateau is cardinal (Go AAN/CNS 2012)

TSC autosomal dominant; metabolic IEM family history; familial IES (ARX, CDKL5) (Go AAN/CNS 2012)

Peak 4-7 mo, range 3 mo - 2 yr; older onset suggests alternate epilepsy syndrome (Go AAN/CNS 2012)

Clusters of brief (1-2 sec) spasms in series, often on awakening; document number per cluster + clusters per day (Go AAN/CNS 2012)

Gold standard for hypsarrhythmia + ictal pattern (Go AAN/CNS 2012)

Structural causes — tubers, periventricular leukomalacia, malformations of cortical development (Go AAN/CNS 2012)

Metabolic IEM screen (Go AAN/CNS 2012)

Shorter lag-time = better developmental outcome (Go AAN/CNS 2012); document onset date precisely

ACTH dosing in U/m²/day requires BSA (Go AAN/CNS 2012; Lux UKISS 2005)

Baseline visual fields BEFORE vigabatrin initiation (irreversible peripheral visual field loss is dose- and duration-related) (Go AAN/CNS 2012; FDA black box)

IEM screen (Go AAN/CNS 2012)

ARX, CDKL5, STXBP1, SCN8A, TSC1/TSC2, FOXG1, MEF2C — high yield (Go AAN/CNS 2012)

* = hard-required. Engine cannot meaningfully run until these are filled.

Severity triggers (7)

7 need judgement
  • informationalseverenew_diagnosis_ies_time_critical
    New diagnosis of infantile epileptic spasms syndrome — TIME-CRITICAL treatment window (Go AAN/CNS 2012 PMID 22689735)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseveretsc_associated_ies
    IES with TSC features (hypopigmented macules, cardiac rhabdomyomas, brain MRI tubers, family history) (Go AAN/CNS 2012)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevererefractory_spasms_after_first_line
    Persistent spasms after 2 weeks of first-line hormonal OR vigabatrin (Go AAN/CNS 2012)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepyridoxine_dependent_epilepsy_dx
    Infant < 18 mo with refractory spasms or diagnostic uncertainty — pyridoxine-dependent epilepsy trial (AES 2016 Glauser PMID 26900382; Go AAN/CNS 2012)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverevigabatrin_visual_field_concern
    Vigabatrin visual field changes on ophthalmology surveillance (FDA black box for irreversible peripheral visual field loss)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverehormonal_therapy_serious_side_effects
    ACTH or prednisolone serious side effects: uncontrolled HTN, hyperglycemia requiring insulin, severe infection (PJP, varicella, bacterial sepsis), cardiac toxicity (Lux UKISS 2005)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereevolution_to_other_epilepsy_syndrome
    Spasm cessation but evolution to Lennox-Gastaut syndrome (multiple seizure types + slow spike-wave on EEG) OR other refractory epilepsy syndrome (Go AAN/CNS 2012)
    Trigger could not be auto-evaluated — needs clinician judgement.

Workflow calculators

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Recommended regimen

Infantile epileptic spasms syndrome — TIME-CRITICAL first-line (3 options + combination) (Go AAN/CNS 2012 PMID 22689735; ICISS O'Callaghan 2017 PMID 27838190)
axis: peds_ies_first_line_three_options
Selected axis "Infantile epileptic spasms syndrome — TIME-CRITICAL first-line (3 options + combination) (Go AAN/CNS 2012 PMID 22689735; ICISS O'Callaghan 2017 PMID 27838190)" by default fallback (first axis)
  • corticotropin
    first line
    ACTH_hormonal_therapy
    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 (max: max 150 U/m²/day per Go AAN/CNS 2012; protocols vary; consult institutional protocol)
    triggers: cryptogenic_IES, non_TSC_IES_with_developmental_regression, caregiver_capacity_for_IM_injections
    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)
    rxcui 376
  • prednisolone
    first line
    oral_corticosteroid_hormonal_therapy
    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 (max: max 60 mg/day per UKISS Lux 2005 PMID 16239177 (alternative protocol up to 8 mg/kg/day; AAP Lexicomp Peds))
    triggers: cryptogenic_IES_alternative_to_ACTH, caregiver_cannot_administer_IM, ACTH_unavailable_or_cost_prohibitive
    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)
    rxcui 8638
  • vigabatrin
    first line
    GABA_transaminase_inhibitor_AED
    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 (max: max 150 mg/kg/day per Go AAN/CNS 2012 PMID 22689735 / UKISS Lux 2005 PMID 16239177 (typically capped at 3000 mg/day in older infants))
    triggers: TSC_associated_IES_first_line, cryptogenic_IES_caregiver_preference_over_hormonal, second_line_after_hormonal_failure
    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
    rxcui 14851
  • pyridoxine
    rescue
    vitamin_B6_metabolic_trial
    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 (max: max single IV trial 200 mg; max maintenance PO 30 mg/kg/day per Go AAN/CNS 2012 / AES 2016 Glauser PMID 26900382 / Lexicomp Peds)
    triggers: infant_under_18_mo_with_refractory_spasms_or_diagnostic_uncertainty
    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
    rxcui 684879

outpatient playbook — drug actions (1)

  1. 1. no medication initiated outpatient — TIME-CRITICAL admission for diagnosis confirmation + treatment initiation
    N/A • N/A • N/A
    trigger: Suspected IES at outpatient visit
    Go AAN/CNS 2012 — shorter lag time = better outcome; admit immediately for diagnostic workup + treatment initiation

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Clusters of brief flexor / extensor / mixed spasms in infant 4-7 mo, often on awakening (Go AAN/CNS 2012 PMID 22689735); 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).

Objective

- No vitals, labs, or imaging entered for this encounter.

Assessment

**Infantile epileptic spasms syndrome (West syndrome)** (peds.infantile-spasms.v1).
Phenotype framing: IES / West syndrome vs benign myoclonic epilepsy of infancy vs Dravet syndrome vs Ohtahara syndrome vs Aicardi syndrome vs pyridoxine-dependent epilepsy vs nonepileptic stereotypies / shudder spells / colic vs hyperekplexia (Go AAN/CNS 2012)
Scope: Confirm clinical triad: epileptic spasms (clusters, often on awakening) + hypsarrhythmic EEG + developmental regression / arrest; peak 4-7 mo (Go AAN/CNS 2012 PMID 22689735)

No severity triggers fired against current inputs.

Plan

Regimen axis: **Infantile epileptic spasms syndrome — TIME-CRITICAL first-line (3 options + combination) (Go AAN/CNS 2012 PMID 22689735; ICISS O'Callaghan 2017 PMID 27838190)**.
1. 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 (ACTH_hormonal_therapy, first line) — 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)
2. 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 (oral_corticosteroid_hormonal_therapy, first line) — 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)
3. 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 (GABA_transaminase_inhibitor_AED, first line) — 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
4. 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 (vitamin_B6_metabolic_trial, rescue) — 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

Setting playbook (outpatient) — Recognize IES early, confirm diagnosis with EEG + neurology, initiate TIME-CRITICAL treatment (admit for inpatient initiation), family education + developmental support
5. no medication initiated outpatient — TIME-CRITICAL admission for diagnosis confirmation + treatment initiation N/A N/A N/A — Suspected IES at outpatient visit (Go AAN/CNS 2012 — shorter lag time = better outcome; admit immediately for diagnostic workup + treatment initiation)

Non-pharmacologic actions:
- Same-day pediatric neurology / epileptology referral (Go AAN/CNS 2012)
- Urgent overnight video EEG scheduling (Go AAN/CNS 2012)
- Urgent MRI brain with seizure protocol scheduling (Go AAN/CNS 2012)
- Inpatient admission for treatment initiation (Go AAN/CNS 2012)
- Family education on IES + treatment options (Go AAN/CNS 2012)
- Early intervention referral (state EI program) (AAP)
- Developmental pediatrics referral (AAP)
- Genetic counseling referral if family history (Go AAN/CNS 2012)

AVOID / contraindication checks:
- Vigabatrin_FDA_black_box_irreversible_peripheral_visual_field_loss_baseline_and_q3mo_ophthalmology (FDA; Go AAN/CNS 2012)
- Vigabatrin_REMS_program_required_in_US (FDA)
- ACTH_side_effects_HTN_hyperglycemia_infection_risk_irritability_weight_gain_immunosuppression (Go AAN/CNS 2012)
- Prednisolone_side_effects_HTN_hyperglycemia_irritability_growth_suppression_immunosuppression (Lux UKISS 2005)
- TSC_first_line_vigabatrin_not_hormonal_unless_severe_or_co_morbid (Go AAN/CNS 2012)
- Hormonal_therapy_immunosuppression_consider_PJP_prophylaxis_per_institutional_protocol (Lux UKISS 2005)
- Live_vaccine_deferral_during_and_after_hormonal_therapy (CDC ACIP)
- Hormonal_therapy_BP_glucose_weight_infection_surveillance_during_4_week_course (Lux UKISS 2005)

Monitoring

Regimen monitoring:
- Repeat overnight video EEG at 2 weeks (cessation of hypsarrhythmia is goal) (Go AAN/CNS 2012)
- Ophthalmology visual field exam at baseline + q3 mo while on vigabatrin (FDA black box)
- BP + glucose + weight + signs of infection + irritability weekly during 4-week ACTH or prednisolone course (Lux UKISS 2005)
- Developmental assessment q3 mo (Lux UKISS 2005; Darke 2010 PMID 20457702)
- Serial neurology / epileptology q1-3 mo for first year (Go AAN/CNS 2012)
- Genetic + metabolic results reviewed at follow-up (Go AAN/CNS 2012)

Setting (outpatient) monitoring:
- Track time from symptom onset to diagnosis (lag time) (Go AAN/CNS 2012)
- Track time from diagnosis to treatment initiation (Go AAN/CNS 2012)
- Family-recorded video log of spasm clusters (Go AAN/CNS 2012)

Follow-up plan: 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)
- Close-out criterion: Follow-up + early intervention + genetic counseling documented

Monitoring phase: Repeat EEG at 2 weeks (cessation of hypsarrhythmia is treatment goal); serial development assessment; vigabatrin visual field exams q3 mo by ophthalmology; ACTH side effects (HTN, hyperglycemia, infection risk, irritability, weight gain, immunosuppression); prednisolone side effects (Go AAN/CNS 2012; FDA black box for VGB)

Disposition

Current setting: outpatient — Recognize IES early, confirm diagnosis with EEG + neurology, initiate TIME-CRITICAL treatment (admit for inpatient initiation), family education + developmental support

Disposition criteria:
- Admit inpatient for diagnostic workup + treatment initiation (Go AAN/CNS 2012)

Escalation triggers (move to higher acuity):
- Refractory spasms or developmental regression → urgent admit for treatment initiation (Go AAN/CNS 2012)
- Refractory status epilepticus → ED + route to peds.status_epilepticus.v1 (AES 2016 Glauser PMID 26900382)
- Suspected TSC → cardiology echo + skin Wood's lamp + brain MRI + dermatology + ophthalmology (Go AAN/CNS 2012)
- Suspected IEM → metabolism / genetics + IEM workup (Go AAN/CNS 2012)

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [SEVERE] New diagnosis of infantile epileptic spasms syndrome — TIME-CRITICAL treatment window (Go AAN/CNS 2012 PMID 22689735)
- [SEVERE] IES with TSC features (hypopigmented macules, cardiac rhabdomyomas, brain MRI tubers, family history) (Go AAN/CNS 2012)
- [SEVERE] Persistent spasms after 2 weeks of first-line hormonal OR vigabatrin (Go AAN/CNS 2012)

Citations

- 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) [PMID:22689735](https://pubmed.ncbi.nlm.nih.gov/22689735/)
- Cited evidence (PMID 16239177) [PMID:16239177](https://pubmed.ncbi.nlm.nih.gov/16239177/)
- Cited evidence (PMID 20457702) [PMID:20457702](https://pubmed.ncbi.nlm.nih.gov/20457702/)
- Cited evidence (PMID 27838190) [PMID:27838190](https://pubmed.ncbi.nlm.nih.gov/27838190/)
- Cited evidence (PMID 26900382) [PMID:26900382](https://pubmed.ncbi.nlm.nih.gov/26900382/)

Last reconciled with current guidelines: 2026-05-26.
References
  • 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) — PMID:22689735
  • Cited evidence (PMID 16239177) — PMID:16239177
  • Cited evidence (PMID 20457702) — PMID:20457702
  • Cited evidence (PMID 27838190) — PMID:27838190
  • Cited evidence (PMID 26900382) — PMID:26900382