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neuro.adem.v1

Acute Disseminated Encephalomyelitis (ADEM)

neurologyacutesubacutepediatricadultacuteinpatientoutpatient
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Phase C shard-3 neuro wave-11 (2026-05-15): authored at SCAFFOLDED — no ADEM-specific workup in clinical-tools-registry.ts (only workup.ms_flare registered for the shared steroid + IVIG/PLEX pulse scaffolding). 9 phenotypes: classic_post-infectious_pediatric / adult_rarer_worse / post-vaccinal_rare / MOG-IgG_positive (route to MOGAD) / multiphasic_MDEM / ADEM-ON / ADEM-LETM (check AQP4) / pediatric_severe_with_coma_or_seizure / autoimmune_encephalitis_overlap. 5 setting playbooks: home (surveillance + developmental) → outpatient (MS-NMO-MOGAD clinic at 3-6-12 mo to classify monophasic vs MDEM vs MS) → ed (HSV PCR mandatory + acyclovir empiric + STAT MRI) → inpatient (IV pulse + IVIG + PLEX + acyclovir until HSV PCR negative + rehab planning) → icu (coma, status, fulminant Hurst variant, tumefactive ICP). 8 PMID evidence anchor: Krupp IPMSSG 2013 (23572237) + Banwell MOGAD 2023 (36706773) + Tenembaum 2002 (12391351) + Pohl 2016 (27572859) + steroid+IVIG+PLEX (22476745) + Hardy adult (27478954) + Wingerchuk NMOSD DDx (26092914) + Apoly DS PLEX (21242498). Schema-blocked: calc.edss / calc.modified_rankin_scale / workup.adem_panel / workup.encephalopathy_workup / workup.mog_igg_cba / workup.aqp4_igg_index — not in clinical-tools-registry; surfaced in depth bundle. Critical safety: HSV PCR MANDATORY before assigning ADEM (mimic exclusion); acyclovir empiric until HSV PCR returns; pediatric methylpred 30 mg/kg/d (max 1 g); MOG-IgG via live CBA (not ELISA); do NOT initiate chronic DMT after monophasic event (70-90% pediatric monophasic); cyclophosphamide CONTRAINDICATED in pregnancy + reserved for fulminant; surveillance MRI at 3-6-12 mo to classify monophasic vs MDEM vs MS conversion; pediatric neuropsych baseline (residua in 30-50%). Sibling differentiation routes to neuro.encephalitis.hsv.v1 (HSV mimic — MANDATORY exclusion), neuro.ms-flare.core.v1 (acute pulse shared scaffolding), neuro.transverse-myelitis.v1 (ADEM-LETM check AQP4), neuro.mogad.v1 (MOG+ pediatric ADEM same-commit peer), neuro.nmosd.v1 (rare AQP4+ pediatric pivot same-commit peer). Promotion to INTEGRATED requires registered ADEM workup (e.g., workup.adem_panel, workup.encephalopathy_workup) + IPMSSG 2013 criteria cascade in clinical-tools-registry.

Entry points (11)

  • symptom
    Post URI/exanthem/vaccination 1-3 wk + encephalopathy + multifocal demyelination — classic pediatric ADEM (IPMSSG 2013 PMID 23572237; Tenembaum PMID 12391351)
    classic_post_infectious_adem_pediatric
  • symptom
    Encephalopathy — required for ADEM diagnosis per IPMSSG 2013 (PMID 23572237)
    encephalopathy_behavioral_change_or_altered_consciousness
  • symptom
    MRI multifocal hyperintense T2/FLAIR lesions — typically large + asymmetric + supratentorial dominant + may enhance
    multifocal_demyelination_on_mri
  • symptom
    Adult-onset ADEM — rarer; worse prognosis; more likely evolves to MS (Hardy PMID 27478954)
    adult_adem_rarer_worse_prognosis
  • symptom
    Post-vaccinal ADEM (very rare; historically rabies / smallpox / influenza)
    post_vaccinal_adem_rare
  • symptom
    MOG-IgG+ pediatric ADEM — route to neuro.mogad.v1 for course classification (~60% of pediatric ADEM are MOG-IgG+; Banwell 2023 PMID 36706773)
    mog_igg_positive_adem
  • symptom
    Second ADEM event ≥3 mo after first (with new lesions on MRI) — multiphasic disseminated encephalomyelitis (MDEM)
    multiphasic_adem_mdem
  • symptom
    ADEM with concurrent ON (ADEM-ON) — recognized phenotype; check MOG-IgG
    adem_with_optic_neuritis_ademon
  • symptom
    ADEM with LETM ≥3 vertebral segments — check AQP4-IgG (NMOSD pivot) and MOG-IgG
    adem_with_letm
  • symptom
    Severe pediatric ADEM with coma / status epilepticus / brainstem-airway involvement → ICU + cyclophosphamide consideration
    pediatric_severe_adem_with_coma_or_seizure
  • symptom
    Autoimmune encephalitis (anti-NMDAR / LGI1 / GAD65) — overlap differential; AE panel mandatory
    autoimmune_encephalitis_overlap

Required inputs (16)

  • agerequired
    demographic • used at CONTEXT
    Pediatric ADEM peak 5-10 y (~80% < 10 y); adult ADEM rarer + worse prognosis (Hardy PMID 27478954)
  • recent_infection_or_vaccination_1_to_3_wk_priorrequired
    history • used at CONTEXT
    Post-infectious / post-vaccinal precipitant in 50-75% of classic pediatric ADEM (Tenembaum PMID 12391351; Pohl PMID 27572859)
  • encephalopathy_documentedrequired
    symptom • used at FRAME
    ENCEPHALOPATHY (behavioral change or altered consciousness) REQUIRED for ADEM diagnosis per IPMSSG 2013 (PMID 23572237) — distinguishes ADEM from multifocal MS first attack
  • time_to_max_severityrequired
    symptom • used at FRAME
    Typically days to <3 mo; >3 mo without new lesions = ADEM; new events ≥3 mo = MDEM (Krupp IPMSSG 2013 PMID 23572237)
  • mri_brain_with_gad_multifocal_lesionsrequired
    imaging • used at INITIAL_WORKUP
    STAT MRI brain with gad — multifocal T2/FLAIR hyperintense lesions; typically large, asymmetric, supratentorial dominant; may show variable gad enhancement; basal ganglia + thalamus involvement common (IPMSSG 2013 PMID 23572237)
  • mri_cord_with_gadrequired
    imaging • used at INITIAL_WORKUP
    Cord MRI — ADEM-LETM with ≥3 vertebral segments triggers AQP4-IgG (NMOSD pivot per Wingerchuk 2015 PMID 26092914)
  • lp_csf_cell_count_protein_glucose_ocb_hsv_pcrrequired
    lab • used at INITIAL_WORKUP
    LP — CSF pleocytosis (often >50 cells), protein elevated, OCB usually NEGATIVE or transient (vs MS 90%+ persistent); HSV PCR MANDATORY (mimic exclusion); enterovirus + VZV + adenovirus PCR if pediatric
  • mog_igg_serum_live_cell_based_assayrequired
    lab • used at INITIAL_WORKUP
    MOG-IgG via live CBA — positive in ~60% pediatric ADEM (MOGAD-ADEM phenotype; route to neuro.mogad.v1) (Banwell 2023 PMID 36706773)
  • aqp4_igg_serum_cell_based_assayrequired
    lab • used at BRANCHING_WORKUP
    AQP4-IgG — rare in pediatric but document; if positive routes to NMOSD (Wingerchuk 2015 PMID 26092914)
  • autoimmune_ae_panel_nmda_lgi1_caspr2_gadrequired
    lab • used at BRANCHING_WORKUP
    Autoimmune encephalitis panel — anti-NMDAR + LGI1 + CASPR2 + GAD65 + GABA-B + AMPA — differential overlap
  • infectious_panel_pediatricrequired
    lab • used at BRANCHING_WORKUP
    Pediatric ADEM workup — strep, mycoplasma serology + PCR, EBV, CMV, mumps, measles (rare with vaccine era)
  • cbc_cmp_lft_esr_crprequired
    lab • used at INITIAL_WORKUP
    Baseline labs — CBC, CMP, LFT before steroid; ESR + CRP (often elevated)
  • seizure_or_status_epilepticusrequired
    symptom • used at RED_FLAGS
    Severe pediatric ADEM may present with seizure / status; EEG monitoring + AED (Pohl PMID 27572859)
  • coma_or_brainstem_compromiserequired
    symptom • used at RED_FLAGS
    Severe ADEM with coma / brainstem-airway compromise → ICU; cyclophosphamide consideration (Hardy PMID 27478954)
  • icp_or_mass_effect
    imaging • used at RED_FLAGS
    Tumefactive ADEM lesions can cause mass effect / midline shift / increased ICP — hyperosmolar therapy + neurosurgery consult
  • family_history_demyelinating
    history • used at CONTEXT
    Family history of MS / NMOSD / MOGAD informs DDx; pediatric MS overlap consideration

12-phase flow (12)

  1. 1FRAME
    Confirm ADEM per IPMSSG 2013 criteria (PMID 23572237) — (1) polyfocal CNS clinical event, (2) ENCEPHALOPATHY required, (3) MRI multifocal hyperintense lesions, (4) no new clinical or MRI findings ≥3 mo after onset (single event); MDEM if second event ≥3 mo apart
    inputs: encephalopathy_documented, time_to_max_severity
    advance: ADEM phenotype assigned vs MDEM vs MS first attack
  2. 2ENTRY
    Acute presentation with encephalopathy + multifocal deficits + post-infectious/post-vaccinal precipitant typically 1-3 wk prior; STAT MRI + LP
    inputs: age, recent_infection_or_vaccination_1_to_3_wk_prior
    advance: Pathway activated (acute pediatric vs adult vs autoimmune AE differential)
  3. 3CONTEXT
    Capture precipitant (URI / exanthem / vaccination 1-3 wk prior), prior demyelinating events (rule out MDEM vs MS), family history, immunocompetence, vaccination status
    inputs: recent_infection_or_vaccination_1_to_3_wk_prior, family_history_demyelinating
    advance: ADEM-relevant context captured
  4. 4RED_FLAGS
    Coma / status epilepticus / brainstem with airway-swallow compromise → ICU; tumefactive lesion with mass effect / increased ICP → hyperosmolar + neurosurgery; HSV encephalitis mimic (HSV PCR+ → route to neuro.encephalitis.hsv.v1); severe respiratory failure
    inputs: seizure_or_status_epilepticus, coma_or_brainstem_compromise, icp_or_mass_effect
    actions: workup.ms_flare
    advance: Critical airway / seizure / ICP / HSV-mimic escalation triaged
  5. 5INITIAL_WORKUP
    STAT MRI brain + cord with gad — multifocal T2/FLAIR lesions typically large + asymmetric + supratentorial + thalamic/basal ganglia + cord (rule out compressive); LP (cell count, protein, glucose, OCB, IgG index, HSV-PCR MANDATORY, enterovirus + VZV + adenovirus); CBC + CMP + LFT + ESR + CRP + glucose; pregnancy test; MOG-IgG (live CBA) + AQP4-IgG (CBA); autoimmune AE panel (IPMSSG 2013 PMID 23572237; Banwell 2023 PMID 36706773)
    inputs: mri_brain_with_gad_multifocal_lesions, mri_cord_with_gad, lp_csf_cell_count_protein_glucose_ocb_hsv_pcr, mog_igg_serum_live_cell_based_assay, cbc_cmp_lft_esr_crp
    actions: panel.csf, panel.cbc, panel.renal, panel.lft, panel.inflammation
    advance: MRI + LP returned (or pending); HSV PCR pending with empiric acyclovir
  6. 6BRANCHING_WORKUP
    HSV PCR → encephalitis pivot if +; MOG-IgG → MOGAD-ADEM pivot if + (most pediatric ADEM); AQP4-IgG → NMOSD pivot if + (rare in ADEM phenotype); autoimmune AE panel for anti-NMDAR / LGI1 etc; infectious serology + PCR (mycoplasma, EBV, CMV, strep); paraneoplastic if older + atypical (Banwell 2023 PMID 36706773; Wingerchuk 2015 PMID 26092914)
    inputs: aqp4_igg_serum_cell_based_assay, autoimmune_ae_panel_nmda_lgi1_caspr2_gad, infectious_panel_pediatric
    advance: Etiology classified (idiopathic ADEM / MOGAD-ADEM / NMOSD-mimic / autoimmune AE / infectious mimic)
  7. 7DIFFERENTIAL
    Classic ADEM (post-infectious/post-vaccinal) / MOGAD-ADEM (MOG+; pivot) / NMOSD-mimic (AQP4+; pivot) / HSV encephalitis (pivot) / autoimmune encephalitis (anti-NMDAR) / MS first attack with later DIS/DIT (Krupp 2013) / acute hemorrhagic leukoencephalitis (Hurst) — fulminant ADEM variant / CLIPPERS / paraneoplastic / vasculitis
    advance: Final phenotype assigned with confidence
  8. 8RISK_STRATIFICATION
    Severity by GCS + lesion burden + tumefactive mass effect + need for ICU; pediatric vs adult prognosis (adult worse — Hardy PMID 27478954); MOG-IgG status drives long-term DMT decision (monophasic vs relapsing course)
    advance: Severity stratified + treatment intensity decided
  9. 9TREATMENT
    ACUTE: IV methylprednisolone 30 mg/kg/d × 3-5 d pediatric (max 1 g/d) OR 1 g/d × 3-5 d adult + IVIG 2 g/kg over 2-5 d if steroid-incomplete; PLEX 5 sessions q48h for severe steroid + IVIG-refractory (Apoly DS PMID 21242498); cyclophosphamide pulse for fulminant / refractory (rare). RECOVERY: 70-90% in pediatric (Tenembaum PMID 12391351; Pohl PMID 27572859). SUPPORTIVE: ICP control if tumefactive; AED if seizures; SLP if dysphagia; rehab early. NO CHRONIC IMMUNOSUPPRESSION unless multiphasic / MDEM / MOG-IgG-positive (route to neuro.mogad.v1)
    advance: Acute pulse + supportive started; chronic decision deferred to course classification
  10. 10DISPOSITION
    Admit pediatric neurology / pediatric ICU if coma / status / brainstem; admit adult neurology floor or ICU per severity; outpatient pediatric MS-NMO-MOGAD clinic q3 mo post-discharge for course classification; rehab pre-discharge planning
    advance: Disposition documented
  11. 11MONITORING
    Daily neuro exam + GCS during acute admission; EEG continuous if seizures; serial MRI at 3 mo + 6 mo + 12 mo to confirm monophasic vs MDEM (no new lesions = monophasic ADEM); pediatric developmental surveillance (cognitive + behavioral deficits common); MOG-IgG persistence at 6-12 mo informs relapse risk
    actions: panel.cbc, panel.lft
    advance: Monitoring schedule active
  12. 12FOLLOWUP
    Rehab (PT/OT/SLP); pediatric neuropsych baseline + serial (cognitive + behavioral deficits in 30-50%); ophthalmology if ON; AED management if seizure history; route to neuro.mogad.v1 if MOG-IgG+ + recurrence; surveillance MRI at 3-6-12 mo to classify monophasic vs MDEM; pediatric developmental specialist long-term
    advance: Long-term plan + specialty referrals + surveillance MRI scheduled