Secondary Progressive Multiple Sclerosis
Encounter flow
12/12 authoredCanonical 12-phase frame with authored status for this dossier.
Frame
Confirm SPMS conversion from RRMS — ≥6 mo progression independent of relapse (Lublin 2014); active vs non-active classification
SPMS confirmed with active/non-active status
Patient inputs (19)
VZV-naive → vaccinate before S1P modulator (AAN 2024)
HBV reactivation on anti-CD20 (AAN 2024)
TB screen before immunosuppressive DMT (AAN 2024)
SPMS typically 10-20 y after RRMS diagnosis; usually 40s-50s
Depression highly comorbid in progressive MS; suicide risk elevated
Active SPMS (with relapse or new MRI lesion) qualifies for siponimod; non-active SPMS has limited DMT options
Confirm prior RRMS to establish SPMS conversion (vs PPMS primary progression)
Lublin 2014 — ≥6 mo progression without relapse defines SPMS conversion
Active SPMS = new T2 / gad+; baseline + annual (AAN 2024)
Lymphopenia surveillance on siponimod / cladribine
Siponimod hepatotoxicity (1% LFT >3× ULN); ocrelizumab LFT monitoring
EDSS upward trajectory marks progression; schema-blocked
Siponimod CYP2C9 *3/*3 contraindicates (EXPAND PMID 29576505)
Siponimod contraindicates 2nd/3rd degree AV block, recent MI/stroke/HF (EXPAND PMID 29576505)
Spasticity dominant phenotype — drives baclofen / tizanidine / BTX / pump decisions
Neurogenic bladder common — anticholinergic / BTX / catheterization
Cord atrophy strong disability predictor in progressive MS
SDMT preferred MS cognitive screen; schema-blocked
Fatigue dominant phenotype — amantadine / modafinil
* = hard-required. Engine cannot meaningfully run until these are filled.
Severity triggers (8)
- informationalsevereactive_spms_relapse_or_mri_activityActive SPMS — relapse in past 2 y OR new T2/gad+ lesion + progression — siponimod EXPAND-eligible (PMID 29576505)Trigger could not be auto-evaluated — needs clinician judgement.
- informationalsevereedss_progression_dominantEDSS step progression confirmed at 3 + 6 mo without intervening relapse — PIRA (progression independent of relapse activity)Trigger could not be auto-evaluated — needs clinician judgement.
- informationalmoderatenon_active_spms_symptomatic_focusNon-active SPMS — no relapse or MRI activity in past 2 y — symptomatic + rehab dominant; consider DMT discontinuation (AAN 2024)Trigger could not be auto-evaluated — needs clinician judgement.
- informationalmoderatepseudobulbar_affect_dextromethorphan_quinidinePseudobulbar affect — uncontrolled laughing/crying episodes inappropriate to mood (TRANSIT trial)Trigger could not be auto-evaluated — needs clinician judgement.
- informationalmoderatespasticity_dominant_baclofen_tizanidineSpasticity dominant phenotype — limits ADL + gait + sleep — baclofen / tizanidine / BTX / intrathecal pump ladderTrigger could not be auto-evaluated — needs clinician judgement.
- informationalmoderateneurogenic_bladder_anticholinergic_or_btxNeurogenic bladder — detrusor overactivity / DSD / retention — oxybutynin / mirabegron / BTX intradetrusor / catheterizationTrigger could not be auto-evaluated — needs clinician judgement.
- informationalmoderatecognitive_decline_neurorehabCognitive decline — SDMT decline / processing speed / memory — cognitive rehab + treat depression + minimize anticholinergic burdenTrigger could not be auto-evaluated — needs clinician judgement.
- informationalmildfatigue_dominant_amantadine_or_modafinilFatigue dominant phenotype — amantadine first-line; modafinil second; sleep hygiene + treat OSATrigger could not be auto-evaluated — needs clinician judgement.
Workflow calculators
Run this disease's risk and dosing calculators inline.
Recommended regimen
SPMS DMT — siponimod first-line for active SPMS; symptomatic dominant for non-active (AAN 2024; EXPAND PMID 29576505)- siponimodfirst lineS1P_modulator0.25 mg titrate to 2 mg PO daily over 6 days (CYP2C9 *1/*3 or *2/*3 → 1 mg max) • PO • daily (max: 2 mg/day (1 mg if CYP2C9 *1/*3 or *2/*3))triggers: active_SPMSEXPAND (Kappos Lancet 2018 PMID 29576505 NEEDS_SOURCE_REVIEW) — first FDA-approved DMT for active SPMS; 21% relative risk reduction in 3-mo CDP; first-dose 6-h ECG monitoringrxcui 2121085
- ocrelizumab (off-label active SPMS)second lineanti_CD20_mAb300 mg IV × 2 then 600 mg q6 months • IV • q6 monthstriggers: active_SPMS_alt_to_siponimodOPERA extension supports off-label use in active SPMS; OPERA PMID 28002679rxcui 1876366
- cladribine (active SPMS)second linepurine_antimetaboliteWeight-based 3.5 mg/kg cumulative oral • PO • pulsed years 1+2triggers: active_SPMS_oral_preferenceCLARITY extension supports active SPMS; PMID 20089960rxcui 44157
outpatient playbook — drug actions (11)
- 1. siponimod0.25 mg titrate to 2 mg PO daily • PO • dailytrigger: Active SPMSEXPAND PMID 29576505; CYP2C9-driven dose
- 2. ocrelizumab (off-label active SPMS)600 mg IV q6 mo • IV infusion • q6 motrigger: Active SPMS altOPERA extension
- 3. baclofen10-20 mg PO TID • PO • TIDtrigger: SpasticityAAN 2024
- 4. tizanidine2-8 mg PO TID • PO • TIDtrigger: Spasticity baclofen-intolerantAAN 2024
- 5. oxybutynin5 mg PO BID-TID • PO • BID-TIDtrigger: Neurogenic bladderAUA 2024
- 6. mirabegron50 mg PO daily • PO • dailytrigger: Anticholinergic-intolerantAUA 2024
- 7. amantadine100 mg PO BID • PO • BIDtrigger: FatigueAAN 2024
- 8. modafinil100-200 mg PO morning • PO • morningtrigger: Amantadine-intolerantAAN 2024
- 9. sertraline50-100 mg PO daily • PO • dailytrigger: DepressionAPA 2024
- 10. dextromethorphan-quinidine (Nuedexta)20/10 mg PO BID • PO • BIDtrigger: Pseudobulbar affectTRANSIT FDA-approved
- 11. dalfampridine10 mg PO BID • PO • BIDtrigger: Gait impairmentFDA-approved; eGFR ≥80; no seizure hx
Auto-drafted A&P note
outpatientSubjective
- Possible entry pathways: RRMS patient with insidious progression ≥6 mo independent of relapses (Lublin 2014 PMID 24871874 NEEDS_SOURCE_REVIEW); EDSS step progression confirmed at 3 + 6 mo without intervening relapse — PIRA; Disproportionate cord/brain atrophy on annual MRI — neurodegenerative pattern.
Objective
- No vitals, labs, or imaging entered for this encounter.
Assessment
**Secondary Progressive Multiple Sclerosis** (neuro.ms-spms.v1). Phenotype framing: Active SPMS (relapse / new MRI) vs non-active SPMS; RRMS-still vs SPMS-conversion (often retrospective); PIRA tracking; consider PPMS misclassification (rare after established RRMS) Scope: Confirm SPMS conversion from RRMS — ≥6 mo progression independent of relapse (Lublin 2014); active vs non-active classification No severity triggers fired against current inputs.
Plan
Regimen axis: **SPMS DMT — siponimod first-line for active SPMS; symptomatic dominant for non-active (AAN 2024; EXPAND PMID 29576505)** — step "Step 1 — Active SPMS (relapse + progression) — siponimod EXPAND". 1. siponimod 0.25 mg titrate to 2 mg PO daily over 6 days (CYP2C9 *1/*3 or *2/*3 → 1 mg max) PO daily (S1P_modulator, first line) — EXPAND (Kappos Lancet 2018 PMID 29576505 NEEDS_SOURCE_REVIEW) — first FDA-approved DMT for active SPMS; 21% relative risk reduction in 3-mo CDP; first-dose 6-h ECG monitoring 2. ocrelizumab (off-label active SPMS) 300 mg IV × 2 then 600 mg q6 months IV q6 months (anti_CD20_mAb, second line) — OPERA extension supports off-label use in active SPMS; OPERA PMID 28002679 3. cladribine (active SPMS) Weight-based 3.5 mg/kg cumulative oral PO pulsed years 1+2 (purine_antimetabolite, second line) — CLARITY extension supports active SPMS; PMID 20089960 Setting playbook (outpatient) — MS clinic q3-6 mo — DMT (siponimod for active SPMS), symptomatic management, neurorehab coordination, depression + cognition screening, advance directives at EDSS ≥7 (AAN 2024) 4. siponimod 0.25 mg titrate to 2 mg PO daily PO daily — Active SPMS (EXPAND PMID 29576505; CYP2C9-driven dose) 5. ocrelizumab (off-label active SPMS) 600 mg IV q6 mo IV infusion q6 mo — Active SPMS alt (OPERA extension) 6. baclofen 10-20 mg PO TID PO TID — Spasticity (AAN 2024) 7. tizanidine 2-8 mg PO TID PO TID — Spasticity baclofen-intolerant (AAN 2024) 8. oxybutynin 5 mg PO BID-TID PO BID-TID — Neurogenic bladder (AUA 2024) 9. mirabegron 50 mg PO daily PO daily — Anticholinergic-intolerant (AUA 2024) 10. amantadine 100 mg PO BID PO BID — Fatigue (AAN 2024) 11. modafinil 100-200 mg PO morning PO morning — Amantadine-intolerant (AAN 2024) 12. sertraline 50-100 mg PO daily PO daily — Depression (APA 2024) 13. dextromethorphan-quinidine (Nuedexta) 20/10 mg PO BID PO BID — Pseudobulbar affect (TRANSIT FDA-approved) 14. dalfampridine 10 mg PO BID PO BID — Gait impairment (FDA-approved; eGFR ≥80; no seizure hx) Non-pharmacologic actions: - PT/OT bid-weekly programs - Cognitive rehab if SDMT decline - Neuropsychology referral if cognitive impairment - Spasticity clinic (BTX, pump consideration) - Urology + urodynamics for refractory bladder - Pelvic floor PT - SLP for dysphagia + speech - Vocational rehab + disability paperwork - Wheelchair + assistive device fitting - Caregiver support + respite - Advance directives + capacity assessment at EDSS ≥7 - Palliative referral at severe disability AVOID / contraindication checks: - CYP2C9_3_3_contraindicates_siponimod (EXPAND PMID 29576505) - Siponimod_first_dose_6h_ECG_monitoring (EXPAND PMID 29576505) - Siponimod_contraindicate_AV_block_recent_MI_stroke_HF (FDA label) - VZV_vaccination_before_siponimod (AAN 2024) - Macular_edema_screen_baseline_and_q3_mo_initial_siponimod (AAN 2024) - LFT_q3_to_6_mo_on_siponimod (AAN 2024) - HBV_screen_before_anti_CD20 (AAN 2024) - TB_screen_before_DMT (AAN 2024) - Lymphocyte_count_nadir_monitor_siponimod_cladribine (AAN 2024) - Cladribine_6mo_washout_before_conception (AAN 2024)
Monitoring
Regimen monitoring: - CBC lymphocyte count q3 to 6 mo (AAN 2024) - LFT q3 to 6 mo (AAN 2024) - OCT or dilated fundus baseline and q3 mo initial siponimod (FDA label) - annual brain MRI (AAN 2024) - annual cervical cord MRI (AAN 2024) - EDSS at each visit PIRA tracking (schema-blocked — see depth bundle) - SDMT cognitive screen annually (schema-blocked) - urodynamics if bladder dysfunction (AUA 2024) Setting (outpatient) monitoring: - EDSS + MSFC components q3-6 mo (schema-blocked) - PIRA confirmation 3- and 6-mo step progression - MRI annually - CBC + LFT q3-6 mo - OCT q3 mo initial on siponimod - PVR + UA q6 mo if catheterizing Follow-up plan: PT/OT/SLP; spasticity clinic + intrathecal baclofen pump consideration if refractory; pulmonary surveillance (FVC); palliative + advance directives at EDSS ≥7; caregiver support (AAN 2024) - Close-out criterion: Multidisciplinary follow-up scheduled Monitoring phase: CBC + LFT q3-6 mo; annual MRI; EDSS / SDMT / T25FW / 9HPT (schema-blocked) at each visit; pressure injury surveillance; UA + bladder scan q6 mo; depression + cognitive screen annually (AAN 2024)
Disposition
Current setting: outpatient — MS clinic q3-6 mo — DMT (siponimod for active SPMS), symptomatic management, neurorehab coordination, depression + cognition screening, advance directives at EDSS ≥7 (AAN 2024) Disposition criteria: - Continue indefinite MS clinic q3-6 mo - Home health intensification at progressive decline - Long-term care at EDSS ≥7.5 with caregiver inability - Hospice at severe disability + recurrent complications Escalation triggers (move to higher acuity): - Acute relapse → route to neuro.ms-flare.core.v1 - Rapid EDSS jump → neuro + MRI urgent - Severe pressure injury stage III/IV → wound + admit - Aspiration / pulmonary decline → SLP + pulmonology - Refractory spasticity → intrathecal baclofen pump - Refractory bladder → BTX + cathter program - Severe depression / suicidality → ED + psych
Earlier-Return Triggers
Return-precaution thresholds (watch for): - [SEVERE] Active SPMS — relapse in past 2 y OR new T2/gad+ lesion + progression — siponimod EXPAND-eligible (PMID 29576505) - [SEVERE] EDSS step progression confirmed at 3 + 6 mo without intervening relapse — PIRA (progression independent of relapse activity) - [MODERATE] Non-active SPMS — no relapse or MRI activity in past 2 y — symptomatic + rehab dominant; consider DMT discontinuation (AAN 2024)
Citations
- AAN 2024 MS DMT guideline + ECTRIMS 2024 + EXPAND siponimod (Kappos Lancet 2018 PMID 29576505) [PMID:29576505](https://pubmed.ncbi.nlm.nih.gov/29576505/) - Cited evidence (PMID 28002679) [PMID:28002679](https://pubmed.ncbi.nlm.nih.gov/28002679/) - Cited evidence (PMID 32757523) [PMID:32757523](https://pubmed.ncbi.nlm.nih.gov/32757523/) - Cited evidence (PMID 29545067) [PMID:29545067](https://pubmed.ncbi.nlm.nih.gov/29545067/) - Cited evidence (PMID 24871874) [PMID:24871874](https://pubmed.ncbi.nlm.nih.gov/24871874/) Last reconciled with current guidelines: 2026-05-22.
- AAN 2024 MS DMT guideline + ECTRIMS 2024 + EXPAND siponimod (Kappos Lancet 2018 PMID 29576505) — PMID:29576505
- Cited evidence (PMID 28002679) — PMID:28002679
- Cited evidence (PMID 32757523) — PMID:32757523
- Cited evidence (PMID 29545067) — PMID:29545067
- Cited evidence (PMID 24871874) — PMID:24871874