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neuro.adem.v1

Acute Disseminated Encephalomyelitis (ADEM)

neurologyacutesubacutepediatricadult
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Encounter flow

12/12 authored

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

Current phase

Frame

Detailed

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

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ADEM phenotype assigned vs MDEM vs MS first attack

Patient inputs (16)

AQP4-IgG — rare in pediatric but document; if positive routes to NMOSD (Wingerchuk 2015 PMID 26092914)

Autoimmune encephalitis panel — anti-NMDAR + LGI1 + CASPR2 + GAD65 + GABA-B + AMPA — differential overlap

Pediatric ADEM workup — strep, mycoplasma serology + PCR, EBV, CMV, mumps, measles (rare with vaccine era)

Pediatric ADEM peak 5-10 y (~80% < 10 y); adult ADEM rarer + worse prognosis (Hardy PMID 27478954)

Post-infectious / post-vaccinal precipitant in 50-75% of classic pediatric ADEM (Tenembaum PMID 12391351; Pohl PMID 27572859)

ENCEPHALOPATHY (behavioral change or altered consciousness) REQUIRED for ADEM diagnosis per IPMSSG 2013 (PMID 23572237) — distinguishes ADEM from multifocal MS first attack

Typically days to <3 mo; >3 mo without new lesions = ADEM; new events ≥3 mo = MDEM (Krupp IPMSSG 2013 PMID 23572237)

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)

Cord MRI — ADEM-LETM with ≥3 vertebral segments triggers AQP4-IgG (NMOSD pivot per Wingerchuk 2015 PMID 26092914)

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 via live CBA — positive in ~60% pediatric ADEM (MOGAD-ADEM phenotype; route to neuro.mogad.v1) (Banwell 2023 PMID 36706773)

Baseline labs — CBC, CMP, LFT before steroid; ESR + CRP (often elevated)

Severe pediatric ADEM may present with seizure / status; EEG monitoring + AED (Pohl PMID 27572859)

Severe ADEM with coma / brainstem-airway compromise → ICU; cyclophosphamide consideration (Hardy PMID 27478954)

Family history of MS / NMOSD / MOGAD informs DDx; pediatric MS overlap consideration

Tumefactive ADEM lesions can cause mass effect / midline shift / increased ICP — hyperosmolar therapy + neurosurgery consult

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Severity triggers (9)

9 need judgement
  • informationallife_threateningpediatric_severe_adem_with_coma_or_seizure
    Pediatric severe ADEM with coma / status epilepticus / brainstem-airway compromise → ICU; cyclophosphamide consideration if refractory
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereclassic_post_infectious_adem_pediatric
    Pediatric post-infectious ADEM — URI/exanthem/vaccination 1-3 wk prior + encephalopathy + multifocal demyelination + HSV PCR negative (IPMSSG 2013 PMID 23572237; Tenembaum PMID 12391351)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereadult_adem_rarer_worse_prognosis
    Adult-onset ADEM — rarer; worse prognosis; more likely evolves to MS (Hardy PMID 27478954)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepost_vaccinal_adem_rare
    Post-vaccinal ADEM — very rare; historically rabies / smallpox / influenza vaccines; report to VAERS
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseveremog_igg_positive_adem
    MOG-IgG+ pediatric ADEM (~60% pediatric ADEM positive) — route to neuro.mogad.v1 for course classification (Banwell 2023 PMID 36706773)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseveremultiphasic_adem_mdem
    MDEM — second ADEM event ≥3 mo after first with new lesions on MRI (IPMSSG 2013 PMID 23572237)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereadem_with_optic_neuritis_ademon
    ADEM with concurrent or sequential ON (ADEM-ON) — check MOG-IgG (often positive); ophthalmology coordination
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereadem_with_letm_check_aqp4
    ADEM with LETM ≥3 vertebral segments — atypical for classic ADEM; AQP4-IgG MANDATORY (NMOSD pivot) + MOG-IgG
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereautoimmune_encephalitis_overlap
    Autoimmune encephalitis (anti-NMDAR / LGI1 / GAD65) — overlap differential with ADEM; AE panel MANDATORY; different chronic management
    Trigger could not be auto-evaluated — needs clinician judgement.

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Recommended regimen

ADEM acute treatment — IV methylpred + IVIG + PLEX (IPMSSG 2013 PMID 23572237; PMID 22476745)
axis: adem_acute_treatmentstep 1 - Step 1 — IV methylprednisolone pulse (first-line; Tenembaum PMID 12391351)
Selected step "Step 1 — IV methylprednisolone pulse (first-line; Tenembaum PMID 12391351)" — Confirmed ADEM per IPMSSG 2013 criteria (PMID 23572237) — encephalopathy + multifocal demyelination + HSV PCR negative
  • methylprednisolone (pediatric)
    first line
    corticosteroid_pulse
    30 mg/kg/d IV × 3-5 d (max 1 g/d) • IV • daily × 3-5 d (max: 1 g/d (5 g cumulative))
    triggers: acute_adem_pediatric
    First-line acute pulse in pediatric ADEM; 70-90% recovery; weight-based dosing capped at 1 g/d (Tenembaum PMID 12391351; Pohl PMID 27572859)
    rxcui 6902
  • methylprednisolone (adult)
    first line
    corticosteroid_pulse
    1 g IV daily × 3-5 d • IV • daily × 3-5 d (max: 5 g cumulative)
    triggers: acute_adem_adult
    Adult ADEM dosing same as MS-flare; rarer + worse prognosis (Hardy PMID 27478954)
    rxcui 6902
  • prednisone (oral taper)
    add on
    corticosteroid_oral_taper
    1-2 mg/kg/d PO (pediatric, max 60 mg) × 1-2 wk then taper over 4-6 wk • PO • daily taper
    triggers: post_pulse_taper_adem
    Oral taper after IV pulse; relapse during taper rare in monophasic ADEM but watched
    rxcui 8640

outpatient playbook — drug actions (5)

  1. 1. prednisone taper supervision
    per taper • PO • taper
    trigger: Post-discharge taper
    Monitor side effects + adherence
  2. 2. levetiracetam (post-seizure)
    Pediatric 30-60 mg/kg/d; Adult 1000-1500 mg BID • PO • BID
    trigger: Post-ADEM seizure history
    AED management; consider taper at 12 mo if seizure-free + normal EEG
  3. 3. observation if monophasic
    no DMT • n/a • n/a
    trigger: Monophasic at 12 mo
    Discharge from chronic surveillance
  4. 4. route to neuro.mogad.v1 if MOG+ + relapsing
    per neuro.mogad.v1 axis • per axis • per axis
    trigger: MOG-IgG+ + ≥2 events ≥3 mo apart
    MOGAD-ADEM phenotype with relapsing course
  5. 5. route to neuro.ms-rrms.v1 if MS conversion
    per neuro.ms-rrms.v1 axis • per axis • per axis
    trigger: New T2 lesions meeting McDonald 2017 DIS+DIT (rare in pediatric, more common in adult ADEM)
    Adult ADEM may presage MS (Hardy PMID 27478954)

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Post URI/exanthem/vaccination 1-3 wk + encephalopathy + multifocal demyelination — classic pediatric ADEM (IPMSSG 2013 PMID 23572237; Tenembaum PMID 12391351); Encephalopathy — required for ADEM diagnosis per IPMSSG 2013 (PMID 23572237); MRI multifocal hyperintense T2/FLAIR lesions — typically large + asymmetric + supratentorial dominant + may enhance.

Objective

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

Assessment

**Acute Disseminated Encephalomyelitis (ADEM)** (neuro.adem.v1).
Phenotype framing: 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
Scope: 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

No severity triggers fired against current inputs.

Plan

Regimen axis: **ADEM acute treatment — IV methylpred + IVIG + PLEX (IPMSSG 2013 PMID 23572237; PMID 22476745)** — step "Step 1 — IV methylprednisolone pulse (first-line; Tenembaum PMID 12391351)".
1. methylprednisolone (pediatric) 30 mg/kg/d IV × 3-5 d (max 1 g/d) IV daily × 3-5 d (corticosteroid_pulse, first line) — First-line acute pulse in pediatric ADEM; 70-90% recovery; weight-based dosing capped at 1 g/d (Tenembaum PMID 12391351; Pohl PMID 27572859)
2. methylprednisolone (adult) 1 g IV daily × 3-5 d IV daily × 3-5 d (corticosteroid_pulse, first line) — Adult ADEM dosing same as MS-flare; rarer + worse prognosis (Hardy PMID 27478954)
3. prednisone (oral taper) 1-2 mg/kg/d PO (pediatric, max 60 mg) × 1-2 wk then taper over 4-6 wk PO daily taper (corticosteroid_oral_taper, add on) — Oral taper after IV pulse; relapse during taper rare in monophasic ADEM but watched

Setting playbook (outpatient) — Post-discharge MS-NMO-MOGAD clinic — monophasic vs MDEM classification at 3-6-12 mo; pediatric developmental surveillance; AED management; route to neuro.mogad.v1 if MOG+/relapsing (IPMSSG 2013 PMID 23572237; Banwell 2023 PMID 36706773)
4. prednisone taper supervision per taper PO taper — Post-discharge taper (Monitor side effects + adherence)
5. levetiracetam (post-seizure) Pediatric 30-60 mg/kg/d; Adult 1000-1500 mg BID PO BID — Post-ADEM seizure history (AED management; consider taper at 12 mo if seizure-free + normal EEG)
6. observation if monophasic no DMT n/a n/a — Monophasic at 12 mo (Discharge from chronic surveillance)
7. route to neuro.mogad.v1 if MOG+ + relapsing per neuro.mogad.v1 axis per axis per axis — MOG-IgG+ + ≥2 events ≥3 mo apart (MOGAD-ADEM phenotype with relapsing course)
8. route to neuro.ms-rrms.v1 if MS conversion per neuro.ms-rrms.v1 axis per axis per axis — New T2 lesions meeting McDonald 2017 DIS+DIT (rare in pediatric, more common in adult ADEM) (Adult ADEM may presage MS (Hardy PMID 27478954))

Non-pharmacologic actions:
- Pediatric developmental + neuropsych referral
- School IEP + accommodations
- PT / OT / SLP referral
- Cognitive + behavioral therapy if residua
- Mental health referral if PHQ-9 ≥10
- Bone health (DEXA, vitamin D)
- Vaccination plan post-steroid

AVOID / contraindication checks:
- HSV_PCR_MANDATORY_before_assigning_ADEM (mimic exclusion)
- Acyclovir_empiric_until_HSV_PCR_returns (avoid catastrophic HSV miss)
- Pediatric_methylpred_30_mg_per_kg_per_d_max_1g_per_d
- MOG_IgG_via_LIVE_CBA_not_ELISA
- Sample_MOG_AQP4_BEFORE_steroid_if_feasible
- Do_NOT_initiate_chronic_DMT_after_monophasic_event (70 90% monophasic in pediatric)
- Cyclophosphamide_CONTRAINDICATED_in_pregnancy_use_only_for_fulminant
- Mannitol_serum_osm_under_320_serum_Na_watch_during_hyperosmolar
- AED_LEV_first_line_for_seizures
- Surveillance_MRI_at_3_6_12_mo_to_classify_monophasic_vs_MDEM
- Pediatric_neuropsych_baseline_for_cognitive_residua_30_to_50pct

Monitoring

Regimen monitoring:
- Daily neuro exam + GCS during acute admission
- EEG continuous if cortical lesions or seizures
- Serial MRI brain at 3 mo + 6 mo + 12 mo (monophasic vs MDEM classification)
- Pediatric neurodevelopmental + neuropsych at discharge + 6 mo + 12 mo
- AED level + LFT if long-term AED
- MOG-IgG persistence at 6-12 mo (Banwell 2023 PMID 36706773)
- Repeat AQP4-IgG if pediatric ADEM-like AQP4+ initial (rare; rule out NMOSD)
- Bone health (DEXA + vitamin D + calcium) if prolonged steroid
- School performance + behavioral surveillance long-term (residua in 30-50%)

Setting (outpatient) monitoring:
- Surveillance MRI at 3 + 6 + 12 mo
- Pediatric neuropsych at 6 + 12 mo
- AED level if applicable
- MOG-IgG at 6-12 mo

Follow-up plan: 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
- Close-out criterion: Long-term plan + specialty referrals + surveillance MRI scheduled

Monitoring phase: 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

Disposition

Current setting: outpatient — Post-discharge MS-NMO-MOGAD clinic — monophasic vs MDEM classification at 3-6-12 mo; pediatric developmental surveillance; AED management; route to neuro.mogad.v1 if MOG+/relapsing (IPMSSG 2013 PMID 23572237; Banwell 2023 PMID 36706773)

Disposition criteria:
- Discharge from chronic surveillance after 24 mo monophasic + no new lesions
- Continue if relapsing (route to neuro.mogad.v1 or neuro.ms-rrms.v1)

Escalation triggers (move to higher acuity):
- New event → ED + STAT MRI (MDEM vs MS conversion)
- Cognitive regression → developmental specialist + repeat MRI
- AED breakthrough → medication adjust
- MOG-IgG+ + recurrence → route to neuro.mogad.v1

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] Pediatric severe ADEM with coma / status epilepticus / brainstem-airway compromise → ICU; cyclophosphamide consideration if refractory
- [SEVERE] Pediatric post-infectious ADEM — URI/exanthem/vaccination 1-3 wk prior + encephalopathy + multifocal demyelination + HSV PCR negative (IPMSSG 2013 PMID 23572237; Tenembaum PMID 12391351)
- [SEVERE] Adult-onset ADEM — rarer; worse prognosis; more likely evolves to MS (Hardy PMID 27478954)

Citations

- Krupp / IPMSSG 2013 ADEM diagnostic criteria + Banwell 2023 MOGAD criteria (for MOG-IgG+ ADEM pivot) + Tenembaum 2002 pediatric ADEM natural history + Pohl 2016 long-term outcomes [PMID:23572237](https://pubmed.ncbi.nlm.nih.gov/23572237/)
- Cited evidence (PMID 36706773) [PMID:36706773](https://pubmed.ncbi.nlm.nih.gov/36706773/)
- Cited evidence (PMID 12391351) [PMID:12391351](https://pubmed.ncbi.nlm.nih.gov/12391351/)
- Cited evidence (PMID 27572859) [PMID:27572859](https://pubmed.ncbi.nlm.nih.gov/27572859/)
- Cited evidence (PMID 22476745) [PMID:22476745](https://pubmed.ncbi.nlm.nih.gov/22476745/)

Last reconciled with current guidelines: 2026-05-22.
References
  • Krupp / IPMSSG 2013 ADEM diagnostic criteria + Banwell 2023 MOGAD criteria (for MOG-IgG+ ADEM pivot) + Tenembaum 2002 pediatric ADEM natural history + Pohl 2016 long-term outcomes — PMID:23572237
  • Cited evidence (PMID 36706773) — PMID:36706773
  • Cited evidence (PMID 12391351) — PMID:12391351
  • Cited evidence (PMID 27572859) — PMID:27572859
  • Cited evidence (PMID 22476745) — PMID:22476745