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peds.infantile-spasms.v1

Infantile epileptic spasms syndrome (West syndrome)

pediatricssubacutepediatricoutpatientinpatienttransition
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NEW pediatric dossier (lane-D 2026-05-26). Manifest authored at prisma/seed/manifests/peds.infantile-spasms.v1.ts. All RxCUIs RxNav-verified (forward + reverse) on 2026-05-26: corticotropin 376, prednisolone 8638, vigabatrin 14851, pyridoxine 684879. Cosyntropin 2890 also verified as alternative to corticotropin. PMIDs verified via PubMed MCP on 2026-05-26: 22689735 Go AAN/CNS 2012 (canonical evidence-based guideline update); 16239177 Lux UKISS 2005 Lancet Neurology; 20457702 Darke 2010 Arch Dis Child UKISS 4-yr follow-up; 27838190 O'Callaghan ICISS 2017 Lancet Neurology combination hormonal + vigabatrin; 26900382 Glauser AES 2016 status epilepticus cross-reference. Workup IDs omitted as not in registry: infantile_spasms, epilepsy_genetics, west_syndrome — used safe registry workups: workup.first_seizure, workup.encephalopathy, workup.status_epilepticus. Calculator gap: Hypsarrhythmia EEG calc + spasm-cessation rate calc not in registry; used calc.bsa (resolves; required for ACTH dosing in U/m²/day) + calc.gcs. Subacute pediatric engine: outpatient + inpatient + transition setting playbooks. TIME-CRITICAL treatment window emphasized throughout. Three first-line options per Go AAN/CNS 2012: ACTH (corticotropin), prednisolone, vigabatrin. Combination per ICISS O'Callaghan 2017 has 72% cessation rate vs 57% hormonal alone. Vigabatrin FDA black box for irreversible peripheral visual field loss — mandatory baseline + q3 mo ophthalmology surveillance + REMS program enrollment. Sibling differentiation from peds.status_epilepticus.v1 (semiology + EEG + treatment paradigm).

Entry points (5)

  • symptom
    Clusters of brief flexor / extensor / mixed spasms in infant 4-7 mo, often on awakening (Go AAN/CNS 2012 PMID 22689735)
    clusters_of_brief_spasms_infant
  • symptom
    Developmental regression or arrest in infant 3 mo - 2 yr — infantile spasms differential (Go AAN/CNS 2012)
    developmental_regression_or_arrest
  • imaging
    Hypsarrhythmic (chaotic high-voltage interictal) EEG pattern in infant (Go AAN/CNS 2012)
    hypsarrhythmic_eeg_pattern
  • history
    Hypopigmented macules (TSC) + spasms in infant — TSC-associated infantile spasms (Go AAN/CNS 2012; ICISS O'Callaghan 2017)
    tsc_skin_findings_with_spasms
  • symptom
    Head drops, salaam attacks, leg jackknifing in clusters in infant — IES vs non-epileptic differential (Go AAN/CNS 2012)
    head_drops_or_salaam_attacks

Required inputs (14)

  • agerequired
    demographic • used at FRAME
    Peak 4-7 mo, range 3 mo - 2 yr; older onset suggests alternate epilepsy syndrome (Go AAN/CNS 2012)
  • weightrequired
    demographic • used at CONTEXT
    All AED dosing weight-based (AAP Red Book 2024-2027, Lexicomp Peds)
  • body_surface_arearequired
    demographic • used at TREATMENT
    ACTH dosing in U/m²/day requires BSA (Go AAN/CNS 2012; Lux UKISS 2005)
  • birth_history_perinatal_complicationsrequired
    history • used at CONTEXT
    Preterm / HIE / NICU / TORCH infections raise probability of symptomatic IES (Go AAN/CNS 2012)
  • developmental_milestones_to_daterequired
    history • used at CONTEXT
    Cryptogenic IES has normal pre-onset development; regression / plateau is cardinal (Go AAN/CNS 2012)
  • family_history_epilepsy_tsc_metabolicrequired
    history • used at CONTEXT
    TSC autosomal dominant; metabolic IEM family history; familial IES (ARX, CDKL5) (Go AAN/CNS 2012)
  • spasm_cluster_pattern_and_frequencyrequired
    history • used at FRAME
    Clusters of brief (1-2 sec) spasms in series, often on awakening; document number per cluster + clusters per day (Go AAN/CNS 2012)
  • lag_time_from_onset_to_diagnosisrequired
    history • used at RISK_STRATIFICATION
    Shorter lag-time = better developmental outcome (Go AAN/CNS 2012); document onset date precisely
  • video_eeg_overnightrequired
    imaging • used at INITIAL_WORKUP
    Gold standard for hypsarrhythmia + ictal pattern (Go AAN/CNS 2012)
  • mri_brain_with_seizure_protocolrequired
    imaging • used at INITIAL_WORKUP
    Structural causes — tubers, periventricular leukomalacia, malformations of cortical development (Go AAN/CNS 2012)
  • metabolic_panel_ammonia_lactaterequired
    lab • used at INITIAL_WORKUP
    Metabolic IEM screen (Go AAN/CNS 2012)
  • serum_amino_acids_urine_organic_acids
    lab • used at BRANCHING_WORKUP
    IEM screen (Go AAN/CNS 2012)
  • genetic_panel_iss_ies
    lab • used at BRANCHING_WORKUP
    ARX, CDKL5, STXBP1, SCN8A, TSC1/TSC2, FOXG1, MEF2C — high yield (Go AAN/CNS 2012)
  • ophthalmology_baseline_visual_fieldsrequired
    imaging • used at TREATMENT
    Baseline visual fields BEFORE vigabatrin initiation (irreversible peripheral visual field loss is dose- and duration-related) (Go AAN/CNS 2012; FDA black box)

12-phase flow (12)

  1. 1FRAME
    Confirm clinical triad: epileptic spasms (clusters, often on awakening) + hypsarrhythmic EEG + developmental regression / arrest; peak 4-7 mo (Go AAN/CNS 2012 PMID 22689735)
    inputs: age, spasm_cluster_pattern_and_frequency
    advance: Triad confirmed + EEG ordered + pediatric neurology notified
  2. 2ENTRY
    Caregiver reports clusters of brief flexor / extensor / mixed spasms (head drops, salaams, leg jackknifing) most prominent on awakening, often initially dismissed as colic / startle; developmental regression (Go AAN/CNS 2012)
    inputs: age
    advance: Entry trigger captured + family-recorded video reviewed + neurology referral made
  3. 3CONTEXT
    Birth history (preterm / HIE / NICU / TORCH), milestones to date, family history of epilepsy / TSC / metabolic disease, prior viral illness, skin findings (hypopigmented macules of TSC) (Go AAN/CNS 2012)
    inputs: weight, body_surface_area, birth_history_perinatal_complications, developmental_milestones_to_date, family_history_epilepsy_tsc_metabolic
    advance: Context complete + etiology hypothesis formulated
  4. 4RED_FLAGS
    Recurrent untreated spasms beyond 1-2 weeks (lag time = worse outcome per Go AAN 2012); developmental regression; hypsarrhythmia on EEG; TSC features; structural brain abnormality (Go AAN/CNS 2012)
    inputs: lag_time_from_onset_to_diagnosis
    advance: Treatment urgency established + TIME-CRITICAL window flagged
  5. 5INITIAL_WORKUP
    Overnight video EEG (gold standard for hypsarrhythmia + ictal pattern); MRI brain with seizure protocol (structural causes); metabolic panel + ammonia + lactate + serum amino acids + urine organic acids; ophthalmology baseline visual fields BEFORE vigabatrin (Go AAN/CNS 2012; FDA black box for VGB)
    inputs: video_eeg_overnight, mri_brain_with_seizure_protocol, metabolic_panel_ammonia_lactate, ophthalmology_baseline_visual_fields
    actions: workup.first_seizure, workup.encephalopathy, panel.metabolic
    advance: EEG + MRI + metabolic workup complete; ophthalmology baseline obtained
  6. 6BRANCHING_WORKUP
    TSC workup if hypopigmented macules + cardiac rhabdomyomas (FoH echo + skin Wood's lamp + brain MRI for tubers); LP if metabolic concern (CSF glycine, lactate, neurotransmitter metabolites); pyridoxine trial 100 mg IV under EEG monitoring for pyridoxine-dependent epilepsy DDx; genetic panel (Go AAN/CNS 2012)
    inputs: serum_amino_acids_urine_organic_acids, genetic_panel_iss_ies
    advance: Etiology workup launched + multidisciplinary input documented
  7. 7DIFFERENTIAL
    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)
    advance: Differential narrowed + IES confirmed by EEG + clinical pattern
  8. 8RISK_STRATIFICATION
    Etiology (cryptogenic better prognosis vs symptomatic); TSC etiology = vigabatrin first-line; lag time to treatment (shorter is better); EEG pattern (Go AAN/CNS 2012; Lux UKISS 2005)
    inputs: lag_time_from_onset_to_diagnosis
    advance: Risk tier + etiology category documented
  9. 9TREATMENT
    TIME-CRITICAL — start within 1-2 weeks of identification. Three first-line options per Go AAN/CNS 2012 + ICISS 2017: (A) ACTH 75-150 U/m²/day IM divided BID × 2 wk then taper × 2 wk (low-dose may be equivalent per Go 2012); (B) Prednisolone 40-60 mg/day PO divided × 2 wk then taper × 2 wk (cheaper, easier); (C) Vigabatrin 50 mg/kg/day PO BID titrate to 100-150 mg/kg/day (preferred for TSC). Combination per ICISS 2017: hormonal + vigabatrin → 72% cessation vs 57% hormonal alone. Pyridoxine trial 100 mg IV before / alongside if any diagnostic uncertainty (Go AAN/CNS 2012; ICISS 2017)
    inputs: weight, body_surface_area, ophthalmology_baseline_visual_fields
    advance: First-line + combination plan documented + ophthalmology baseline complete
  10. 10DISPOSITION
    Inpatient admission for initial ACTH / prednisolone initiation + EEG monitoring + family education + vigabatrin baseline visual fields; outpatient for chronic vigabatrin maintenance (Go AAN/CNS 2012)
    advance: Disposition + admission for initiation documented
  11. 11MONITORING
    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)
    advance: Monitoring orders documented
  12. 12FOLLOWUP
    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)
    advance: Follow-up + early intervention + genetic counseling documented