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neuro.ms-flare.core.v1

Multiple Sclerosis Flare

neurologysubacuteadultoutpatientinpatient
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Phase B deepening (2026-05-14): MS flare phenotypes (optic_neuritis, transverse_myelitis, brainstem_syndrome, cerebellar_hemispheric_attack, uhthoff_pseudoflare, steroid_refractory_severe, nmosd_suspected, mogad_suspected, jcv_high_index_natalizumab, breakthrough_relapse_on_dmt) encoded as severity_triggers — pivot from variant-specific engines because NMOSD/MOGAD/ADEM/DMT-escalation companion engines are Phase C/D expansion targets. 5 setting playbooks span the full journey: home (relapse vs Uhthoff differentiation + infection screen) → ed (NMOSD/MOGAD-distinguishing features + STAT MRI + LP with AQP4/MOG send-out + first dose IV methylpred) → icu (rare; severe TM with respiratory/autonomic compromise or steroid complications) → inpatient (full 1 g × 3–5 d pulse + PLEX if refractory + glucose control + DVT/PPI/PJP prophylaxis + antibody-result follow-up) → outpatient (DMT-escalation discussion → future companion engine, MRI surveillance, JCV q6 mo on natalizumab, AQP4/MOG re-classification, EDSS q6 mo). Acute pulse: IV methylprednisolone 1 g × 3–5 d (ONTT PMID 1734247) OR oral methylprednisolone 1250 mg × 3–5 d (Le Page non-inferior). PLEX 5 cycles q48h for steroid-refractory severe deficit (Apoly DS Magaña Neurology 2011 PMID 11309833 NEEDS_SOURCE_REVIEW; AAN 2011 Class I). Secondary rescue: IVIG / cyclophosphamide. Antibody send-outs (AQP4-IgG live cell-based assay + MOG-IgG live cell-based assay) are mandatory at first demyelinating event or atypical flare; positive result pivots chronic management to NMOSD or MOGAD pathway. AVOID interferon, natalizumab, fingolimod in AQP4+ NMOSD — they WORSEN disease (Wingerchuk 2015 PMID 26092914). 14-PMID anchor set: 2017 McDonald (29275977; 2024 revision pending publication — NEEDS_SOURCE_REVIEW), ONTT Beck 1992 (1734247), Apoly DS Magaña 2011 (11309833 NEEDS_SOURCE_REVIEW), OPERA Hauser 2017 (28002679), ASCEND Kapoor 2018 (29545067 NEEDS_SOURCE_REVIEW), HERMES Hauser 2008 (18272891), DEFINE Gold 2012 (22992073), CONFIRM Fox 2012 (22992072), CLARITY Giovannoni 2010 (20089960), NMOSD criteria Wingerchuk 2015 (26092914), PREVENT Pittock 2019 (31050279), SAkura satralizumab (31774956 + 32333898 NEEDS_SOURCE_REVIEW), N-MOmentum Cree 2019 (31495497 NEEDS_SOURCE_REVIEW), MOGAD criteria Banwell 2023 (36706773). All marked NEEDS_SOURCE_REVIEW per shard convention; see _briefs/neuro.ms-flare.core.v1.depth.md §1. Schema-blocked calculators surfaced in depth bundle: EDSS (Expanded Disability Status Scale; Kurtzke 1983), ARR (annualized relapse rate), MSSS (Multiple Sclerosis Severity Score; Roxburgh 2005), MSFC (MS Functional Composite = Timed 25-Foot Walk + 9-Hole Peg Test + PASAT/SDMT; Cutter 1999). Currently encoded as plain-English required_assessments strings. Sibling differentiation: GBS (existing real engine), NMOSD (future neuro.nmosd.v1), MOGAD (future neuro.mogad.v1), ADEM (future neuro.adem.v1), CNS vasculitis, neurosarcoidosis, CNS Lyme, B12 deficiency / subacute combined degeneration, Susac, CADASIL. sibling_engine_id is not registry-resolved; descriptive identifiers chosen so future engines can adopt them directly.

Entry points (4)

  • symptom
    New neurological deficit in known MS lasting ≥24h (AAN 2024)
    new_neurologic_deficit_in_known_ms
  • symptom
    Acute monocular vision loss with painful eye movement + RAPD (ONTT 1992 PMID 1734247)
    optic_neuritis
  • symptom
    Sensory level / motor level / bowel-bladder dysfunction (AAN 2024)
    transverse_myelitis_pattern
  • symptom
    Acute brainstem / cerebellar syndrome — pivot for NMOSD (AAN 2024; Wingerchuk 2015 PMID 26092914)
    cerebellar_brainstem_syndrome

Required inputs (13)

  • agerequired
    demographic • used at CONTEXT
    Younger relapses respond better; pregnancy alters DMT choice (AAN 2024)
  • ms_subtyperequired
    history • used at DIFFERENTIAL
    RRMS / SPMS / PPMS — only RRMS/active SPMS respond to immunomodulation (AAN 2024)
  • current_dmtrequired
    history • used at TREATMENT
    Existing DMT informs escalation (anti-CD20, S1P, natalizumab) vs switch decision (AAN 2024)
  • last_relapse_date
    history • used at TREATMENT
    Relapse frequency drives DMT escalation per ECTRIMS 2024
  • edss_at_baseline
    symptom • used at RISK_STRATIFICATION
    EDSS pre-flare anchors recovery target (AAN 2024)
  • mri_brain_spine_with_contrastrequired
    imaging • used at INITIAL_WORKUP
    Active gadolinium-enhancing lesion confirms relapse vs pseudo-relapse; long cord lesion ≥3 segments pivots to NMOSD (Wingerchuk 2015 PMID 26092914)
  • urinalysisrequired
    lab • used at INITIAL_WORKUP
    Rule out UTI as pseudo-relapse trigger (Uhthoff) (AAN 2024)
  • temperaturerequired
    vital • used at CONTEXT
    Fever / Uhthoff phenomenon = pseudo-relapse, not true flare (AAN 2024)
  • glucose
    lab • used at TREATMENT
    Steroid pulse raises glucose; baseline before methylprednisolone (AAN 2024)
  • jc_virus_index
    lab • used at TREATMENT
    JCV+ on natalizumab → PML risk; gates DMT switch (AAN 2024)
  • aqp4_igg
    lab • used at BRANCHING_WORKUP
    AQP4-IgG positive → NMOSD (different management — eculizumab/satralizumab/inebilizumab/rituximab) (Wingerchuk 2015 PMID 26092914; Pittock 2019 PREVENT PMID 31050279)
  • mog_igg
    lab • used at BRANCHING_WORKUP
    MOG-IgG positive → MOGAD (often monophasic; bilateral optic neuritis; cortical encephalitis) (Banwell 2023 PMID 36706773)
  • pregnancy_statusrequired
    history • used at TREATMENT
    Pregnancy excludes most DMTs; methylprednisolone OK after first trimester (AAN 2024)

12-phase flow (12)

  1. 1FRAME
    Confirm true relapse (≥24h, no fever/infection) vs pseudo-relapse (AAN 2024)
    inputs: temperature, urinalysis
    advance: pseudo-relapse triggers excluded
  2. 2ENTRY
    Recognise new deficit in known MS or first demyelinating event (CIS/RIS) (AAN 2024)
    inputs: age, ms_subtype
    advance: entry trigger captured
  3. 3CONTEXT
    DMT history, pregnancy, JCV index, vaccinations, comorbidities (AAN 2024)
    inputs: current_dmt, pregnancy_status, jc_virus_index
    advance: context captured
  4. 4RED_FLAGS
    Spinal cord syndrome with bowel/bladder, brainstem with airway/swallow, severe optic neuritis (no light perception) — admit + STAT MRI (AAN 2024)
    advance: no inpatient-level deficit or admitted
  5. 5INITIAL_WORKUP
    MRI brain + cervical/thoracic cord with gad; urinalysis; CBC/CMP; pregnancy test if indicated (2017 McDonald PMID 29275977)
    inputs: mri_brain_spine_with_contrast, urinalysis
    advance: active gad+ lesion documented
  6. 6BRANCHING_WORKUP
    AQP4-IgG (NMOSD), MOG-IgG (MOGAD), LP for OCB + IgG index when atypical longitudinally extensive cord lesion or bilateral optic neuritis — different DMT (Wingerchuk 2015 PMID 26092914; Banwell 2023 PMID 36706773)
    inputs: aqp4_igg, mog_igg
    advance: NMOSD/MOGAD ruled in or out
  7. 7DIFFERENTIAL
    True relapse vs pseudo-relapse vs NMOSD/MOGAD vs PML on natalizumab vs ADEM (AAN 2024)
    inputs: ms_subtype
    advance: classification assigned
  8. 8RISK_STRATIFICATION
    Severity (functional impact, EDSS change), recovery trajectory (AAN 2024)
    inputs: edss_at_baseline
    advance: severity documented
  9. 9TREATMENT
    Methylprednisolone 1 g IV daily × 3–5 d (or PO equivalent per Le Page); PLEX for steroid-refractory severe (Apoly DS PMID 11309833); DMT escalation per ECTRIMS 2024
    inputs: glucose, pregnancy_status
    advance: pulse therapy started + DMT decision triaged
  10. 10DISPOSITION
    Outpatient infusion vs inpatient admission for severe deficit / IV access / fall risk (AAN 2024)
    advance: level-of-care set
  11. 11MONITORING
    Glucose during steroid course, mood/sleep, recovery trajectory at 4 + 12 wk; DMT-specific labs (CBC, JCV, IgG) (AAN 2024)
    inputs: glucose
    advance: monitoring plan documented
  12. 12FOLLOWUP
    MS clinic at 4–6 wk; rehab/PT/OT; symptomatic Rx (spasticity, fatigue, bladder); annual MRI brain ± cord; vaccine planning before B-cell depletion (AAN 2024)
    advance: MS clinic + rehab follow-up scheduled