Acute Transverse Myelitis
Phase C shard-3 neuro expansion wave-8 (2026-05-14): authored at INTEGRATED tier — manifest file forward-declared (stub manifest in prisma/seed/manifests permits pointer resolve; PRODUCTION promotion requires full manifest + RxNav-validated terminology). 10 phenotype severity_triggers span the full transverse myelitis spectrum: idiopathic_tm (TMCWG 2002 PMID 12236201) / nmosd_tm (AQP4+; longitudinally extensive ≥3 vertebral segments; Wingerchuk 2015 PMID 26092914; route to ms-flare NMOSD pathway) / mogad_tm (MOG+; Banwell 2023 PMID 36706773; route to ms-flare MOGAD pathway) / post_infectious_tm (Pidcock 2007 PMID 17470749) / sarcoid_tm (ACE + biopsy) / sle_tm (route to rheum.sle-flare.core.v1; cyclophosphamide + rituximab) / sjogren_tm (rituximab) / paraneoplastic_tm (cancer workup) / spinal_cord_infarct (anterior cord; vascular RFs; NOT steroid responsive — route to neuro.ischaemic-stroke.v1) / dural_av_fistula_chronic (embolization; NOT steroid responsive). 5 setting playbooks: home (rehab maintenance + recurrence warning) → ed (STAT MRI exclude compression + LP + AQP4/MOG send-out + start methylprednisolone) → icu (cervical TM respiratory + autonomic + PLEX + cyclophosphamide for severe AI) → inpatient (steroid pulse + PLEX + workup + DMT initiation + rehab) → outpatient (DMT via ms-flare engine + rehab + recurrence surveillance + complications mgmt). Schema-blocked downstream: calc.asia (ASIA Impairment Scale A-E), calc.scim (Spinal Cord Independence Measure) — not in clinical-tools-registry; calc.edss available and used for MS-spectrum cases; surfaced in depth bundle as schema-blocked tickets. Regimen axis encoded with 4 steps: high-dose IV methylprednisolone 1 g × 3-5 d (TMCWG 2002 PMID 12236201; ms-flare ONTT-scaffolding) → PLEX 5 sessions q48h × 10 d for steroid-refractory severe (ApolyDS Magaña 2011 PMID 10589540 Class I) → cyclophosphamide 1 g/m² monthly for severe AI/vasculitic + rituximab/eculizumab/satralizumab/inebilizumab for NMOSD-AQP4+ (route via ms-flare NMOSD pathway) → antibiotic for confirmed infectious (Lyme doxycycline / ceftriaxone — CDC PMID 27931077; HSV/VZV acyclovir; schistosoma praziquantel) + supportive (bowel/bladder catheterization, DVT prophylaxis, spasticity Rx baclofen/tizanidine, pressure ulcer prevention, PT/OT/SLP rehab). Sibling differentiation maps to neuro.ms-flare.core.v1 (primary NMOSD-TM + MOGAD-TM pathway — ms-flare engine has the steroid + PLEX + IVIG + NMOSD-specific DMT scaffolding), neuro.gbs.core.v1 (sensory level pivot — TM has sensory level + bilateral cord-level deficit; GBS has ascending without level + areflexia + albuminocytologic dissociation), rheum.sle-flare.core.v1 (SLE-TM overlap — 5% of SLE develop TM; SLE engine drives cyclophosphamide / rituximab; TM engine drives cord workup + steroid + PLEX) — all sibling engines are PRODUCTION-registered. Critical safety: STAT MRI cord with gadolinium to EXCLUDE COMPRESSIVE myelopathy (epidural abscess / hematoma / mass / fracture / disc — surgical emergency; time-to-decompression determines neurological outcome) FIRST before TM diagnosis; CSF AQP4-IgG + MOG-IgG send-out BEFORE steroid (live cell-based assay; steroid can lower titer); cyclophosphamide CONTRAINDICATED in pregnancy; eculizumab requires meningococcal vaccine FIRST; rituximab requires HBV/VZV/TB screen FIRST; baclofen NEVER abrupt-stop (withdrawal syndrome with hyperthermia + AMS + autonomic instability); DVT prophylaxis + bladder decompression + pressure ulcer prevention MANDATORY in immobile TM.
Entry points (8)
- symptomBilateral motor + sensory deficit referable to single spinal cord level (TMCWG 2002 PMID 12236201)bilateral_motor_sensory_deficit_with_level
- symptomSensory level (clear demarcation between normal and abnormal sensation) — TMCWG 2002 cardinal featuresensory_level_present
- symptomBowel / bladder dysfunction (retention, incontinence, urgency) — autonomic involvement; cord pathologybowel_bladder_dysfunction
- symptomBack pain at the level of the cord lesion (inflammatory radicular pain)back_pain_at_level
- symptomMax severity within 4 h to 21 d of onset (TMCWG 2002 PMID 12236201)rapid_progression_4h_21d
- symptomRecent or concurrent optic neuritis — NMOSD pivot (Wingerchuk 2015 PMID 26092914)optic_neuritis_recent_or_concurrent
- symptomRecent viral / bacterial illness or vaccination — post-infectious TM (Pidcock 2007 PMID 17470749)recent_viral_or_bacterial_illness
- historyKnown SLE / Sjögren / sarcoid / Behçet → autoimmune TM workupknown_autoimmune_disease
Required inputs (20)
- agerequireddemographic • used at CONTEXTAdult TM peaks 10-19 + 30-39; NMOSD female 9:1; MOGAD bimodal peds + young adult; older age + vascular RFs → spinal cord infarct (TMCWG 2002 PMID 12236201)
- sexdemographic • used at CONTEXTNMOSD female predominant (9:1); SLE / Sjögren female predominant; MOGAD slight female (Banwell 2023 PMID 36706773)
- symptom_onset_timingrequiredsymptom • used at FRAMEMax severity within 4 h to 21 d = TMCWG 2002 criterion; <4 h = vascular (cord infarct); >21 d = chronic (dural AV fistula, compressive, demyelinating slow progressive)
- cord_level_localizationrequiredsymptom • used at CONTEXTCervical (C3-C5 → diaphragm + respiratory) / thoracic / lumbar localization drives respiratory + autonomic risk + intubation threshold
- asia_grade_baselinerequiredsymptom • used at RISK_STRATIFICATIONASIA Impairment Scale A-E (A = complete; B-D = incomplete; E = normal) baseline drives severity + prognosis + PLEX trigger; schema-blocked calculator
- autonomic_featuresrequiredsymptom • used at CONTEXTBowel / bladder dysfunction, sweating asymmetry, hypotension / dysreflexia (cervical/high thoracic) → cord involvement, autonomic risk, ICU consideration
- respiratory_status_fvc_nifrequiredsymptom • used at RED_FLAGSCervical TM (C3-C5) → diaphragm involvement; FVC < 20 mL/kg or NIF magnitude < 30 → intubate
- recent_infection_vaccinationrequiredhistory • used at CONTEXTPost-infectious TM after viral / bacterial illness or vaccination (Pidcock 2007 PMID 17470749); also drives infectious workup
- autoimmune_disease_historyrequiredhistory • used at CONTEXTKnown SLE / Sjögren / sarcoid / Behçet / MS / NMOSD / MOGAD drives autoimmune workup + DMT decisions
- malignancy_historyrequiredhistory • used at CONTEXTParaneoplastic TM (anti-CRMP5, anti-amphiphysin, anti-Hu) — drives cancer screening
- travel_exposure_tick_schistosomiasisrequiredhistory • used at CONTEXTLyme (tick endemic; CDC PMID 27931077); schistosoma (freshwater bathing in endemic); HTLV-1/2 (Caribbean / Japan); strongyloides (immunosuppression); brucellosis; TB
- mri_cord_with_gad_statrequiredimaging • used at INITIAL_WORKUPSTAT MRI cervical + thoracic cord with gadolinium — exclude compressive lesion (epidural abscess, hematoma, mass, fracture, disc — SURGICAL EMERGENCY); document lesion length (≥3 vertebral segments → NMOSD pivot — Wingerchuk 2015 PMID 26092914); cord edema; gad enhancement = active inflammation
- mri_brain_with_gadrequiredimaging • used at INITIAL_WORKUPMRI brain — rule out MS lesions (T2 white matter, periventricular, Dawson fingers); MOGAD cortical encephalitis (Banwell 2023 PMID 36706773)
- lp_csf_cell_count_protein_glucose_ocb_igg_indexrequiredlab • used at INITIAL_WORKUPCSF cell count (lymphocytic pleocytosis), protein (mildly elevated), glucose (normal), OCB (matched in TM, unmatched in MS), IgG index — TMCWG 2002 inflammation evidence
- aqp4_igg_serum_csfrequiredlab • used at BRANCHING_WORKUPAQP4-IgG (live cell-based assay) → NMOSD; positive triggers different DMT (eculizumab / satralizumab / inebilizumab / rituximab) (Wingerchuk 2015 PMID 26092914)
- mog_igg_serumrequiredlab • used at BRANCHING_WORKUPMOG-IgG (live cell-based assay) → MOGAD; different DMT consideration (Banwell 2023 PMID 36706773)
- ana_dsdna_ss_a_ss_b_ace_rpr_hiv_lyme_b12_htlvlab • used at BRANCHING_WORKUPAutoimmune + infectious panel — ANA / dsDNA (SLE), SS-A/SS-B (Sjögren), ACE (sarcoid + gallium scan + biopsy), RPR (syphilis), HIV, Lyme (CDC PMID 27931077), B12 (subacute combined degeneration mimic), HTLV-1/2 (HAM/TSP — Caribbean / Japan endemic)
- paraneoplastic_panellab • used at BRANCHING_WORKUPAnti-CRMP5, anti-amphiphysin, anti-Hu → paraneoplastic; drives cancer screening (CT chest/abdomen/pelvis + PET)
- glucose_baselinerequiredlab • used at INITIAL_WORKUPBaseline glucose before high-dose steroid pulse — steroid-induced hyperglycemia common
- pregnancy_statusrequiredhistory • used at CONTEXTPregnancy alters drug selection (steroid OK after first trimester; cyclophosphamide CONTRAINDICATED; rituximab category C; doxycycline CONTRAINDICATED → amoxicillin/cefuroxime for Lyme); ob consult
12-phase flow (12)
- 1FRAMEAcute / subacute bilateral spinal cord dysfunction with sensory level + inflammation evidence (TMCWG 2002 PMID 12236201); EXCLUDE COMPRESSIVE first via STAT MRIinputs: symptom_onset_timing, cord_level_localizationadvance: TMCWG criteria framework activated
- 2ENTRYED presentation with new bilateral weakness + sensory level + bowel/bladder dysfunction — STAT spinal MRI within 2 h to exclude compressive lesioninputs: ageadvance: TM pathway activated with STAT imaging
- 3CONTEXTCapture recent infection / vaccination, autoimmune history, malignancy, travel (Lyme, schistosoma, HTLV, TB), sexual history, immunosuppression, family history, pregnancyinputs: recent_infection_vaccination, autoimmune_disease_history, malignancy_history, travel_exposure_tick_schistosomiasis, pregnancy_status, autonomic_featuresadvance: Context captured
- 4RED_FLAGSCompressive myelopathy (mass / hematoma / abscess / fracture / disc — STAT decompression neurosurgery emergency); respiratory failure with cervical TM (FVC < 20 mL/kg or NIF magnitude < 30 → intubate); autonomic instability (high cord with autonomic dysreflexia); ileus + retention requiring decompressioninputs: respiratory_status_fvc_nif, mri_cord_with_gad_statactions: workup.acute_weaknessadvance: Compression excluded + respiratory + autonomic risk triaged
- 5INITIAL_WORKUPSTAT MRI cervical + thoracic cord with gadolinium (exclude compressive; document lesion length ≥3 vertebral segments → NMOSD pivot; cord edema; gad enhancement = active inflammation) → MRI brain with gad (MS lesion screen; MOGAD cortical encephalitis) → LP (cell count, protein, glucose, OCB, IgG index, viral PCRs, AQP4-IgG, MOG-IgG) → CBC + BMP + ESR + CRP + glucose + pregnancy testinputs: mri_cord_with_gad_stat, mri_brain_with_gad, lp_csf_cell_count_protein_glucose_ocb_igg_index, glucose_baselineactions: panel.csf, panel.cbc, panel.renal, panel.lft, panel.inflammationadvance: Workup back; compressive excluded; TMCWG criteria met
- 6BRANCHING_WORKUPNMOSD if AQP4-IgG+ (Wingerchuk 2015 PMID 26092914 — route to ms-flare engine NMOSD pathway); MOGAD if MOG-IgG+ (Banwell 2023 PMID 36706773 — route to ms-flare engine MOGAD pathway); sarcoid if elevated ACE + gallium scan + biopsy granulomas; SLE if ANA/dsDNA+ (route to rheum.sle-flare.core.v1); Sjögren if SS-A/SS-B+ + Schirmer + lip biopsy; infectious if viral PCR + or Lyme serology+ (doxycycline 14-28 d adult; CDC PMID 27931077) or schistosoma if travel + eos (praziquantel + steroid); paraneoplastic if anti-CRMP5 / anti-amphiphysin / anti-Hu+ (CT chest/abdomen/pelvis + PET cancer workup)inputs: aqp4_igg_serum_csf, mog_igg_serum, ana_dsdna_ss_a_ss_b_ace_rpr_hiv_lyme_b12_htlv, paraneoplastic_paneladvance: Etiology assigned
- 7DIFFERENTIALIdiopathic TM (TMCWG 2002 PMID 12236201) / NMOSD-TM (AQP4+; Wingerchuk 2015) / MOGAD-TM (MOG+; Banwell 2023 PMID 36706773) / post-infectious / sarcoid (Pidcock 2007 PMID 17470749) / SLE / Sjögren / paraneoplastic / spinal cord infarct (anterior cord syndrome; vascular RF) / chronic dural AV fistula (progressive over months; spinal angiography) / radiation myelopathyadvance: Phenotype assigned
- 8RISK_STRATIFICATIONASIA Impairment Scale A-E baseline (schema-blocked); cord level (cervical / thoracic / lumbar); longitudinally extensive ≥3 vertebral segments (NMOSD pivot); steroid response trajectory; recurrence risk (NMOSD 60% in 1 y untreated — Wingerchuk 2015 PMID 26092914)inputs: asia_grade_baselineadvance: Severity stratified + DMT decision approached
- 9TREATMENTHigh-dose IV methylprednisolone 1 g daily × 3-5 d (TMCWG 2002 scaffolding; ms-flare ONTT-style) → PLEX 5 sessions q48h × 10 d if steroid-refractory severe (ApolyDS Magaña 2011 PMID 10589540 — Class I) → IV cyclophosphamide 1 g/m² monthly for severe AI/vasculitic TM → eculizumab / satralizumab / inebilizumab / rituximab for NMOSD-AQP4+ (route to ms-flare engine NMOSD pathway) → IV/PO antibiotic if confirmed infectious (Lyme doxycycline 14-28 d / ceftriaxone IV; CDC PMID 27931077; syphilis penicillin G; HSV/VZV acyclovir; schistosoma praziquantel + steroid; TB RIPE) → supportive (bowel/bladder catheterization, DVT prophylaxis LMWH, spasticity Rx baclofen/tizanidine/dantrolene, pressure ulcer prevention, PT/OT/SLP from day 1)inputs: glucose_baselineadvance: Steroid pulse + PLEX trigger + DMT decision + supportive started
- 10DISPOSITIONAdmit ICU if cervical TM with FVC < 20 mL/kg or NIF magnitude < 30, ascending pattern, autonomic instability, PLEX needed; admit floor if thoracic / lumbar without respiratory or autonomic compromise; outpatient infusion only after acute phase + ASIA stableadvance: Disposition documented
- 11MONITORINGDaily neuro exam + ASIA grading; FVC/NIF q4h if cervical; bowel/bladder log; pressure ulcer surveillance; glucose q6h on steroid; mood/sleep (steroid psychosis); DMT-specific labs if NMOSD/MOGAD treatment started (CBC, CD19, IgG levels for rituximab; meningococcal vaccine for eculizumab)advance: Monitoring plan documented
- 12FOLLOWUPRehab 6-12 mo (PT / OT / SLP); NMOSD / MOGAD DMT initiation if AQP4+/MOG+ (route to ms-flare engine for regimen scaffolding); MS clinic q3 mo if MRI brain demyelinating lesions; autoimmune clinic if SLE / Sjögren / sarcoid; recurrent TM warning (NMOSD 60% in 1 y untreated; Wingerchuk 2015 PMID 26092914); bladder mgmt + bowel regimen + spasticity Rx + pressure ulcer surveillance + sexual function counseling + psych follow-upadvance: Long-term plan documented + rehab arranged