Patient handout

Primary Progressive Multiple Sclerosis

PRODUCTION

1. Your condition

This handout is for primary progressive multiple sclerosis. Your care team identified this based on: progressive paraparesis or cord syndrome from onset ≥1 y without relapse (lublin 2014 pmid 24871874 needs_source_review).

Other reasons your team may use this plan: cervical cord-dominant progressive syndrome — mri shows cord atrophy + cord lesions, minimal brain; cognitive-dominant ppms — frontal/subcortical pattern without relapse; middle-age male with progressive neurological decline — typical ppms demographic (m:f ~1:1, mean onset 40s).

2. Your medications

Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.

Medication	Starting dose	How	When	What it does
ocrelizumab	300 mg IV × 2 (2 weeks apart) then 600 mg IV q6 months	IV	q6 months	ORATORIO (Montalban NEJM 2017 PMID 28002688 NEEDS_SOURCE_REVIEW) — first and only FDA-approved DMT for PPMS; 24% RR reduction 12-wk CDP; ARR + MRI improvement; hypogammaglobulinemia surveillance
rituximab (off-label)	1000 mg IV × 2 (2 weeks apart) then q6 months	IV	q6 months	PROMISE (Hawker Ann Neurol 2009 PMID 19847908 NEEDS_SOURCE_REVIEW) — negative overall but signal in younger inflammatory subgroup; off-label in MS

Plan: PPMS DMT — ocrelizumab ORATORIO first-line for active PPMS (PMID 28002688)

3. When to call your provider

Contact your care team if any of the following happen:

Rare acute relapse → route to neuro.ms-flare.core.v1
Continued progression on ocrelizumab → cyclophosphamide consideration
Severe pressure injury → admit
Aspiration / pulmonary decline → admit
Refractory spasticity → intrathecal pump
Refractory bladder → BTX + cath program
Severe depression → ED + psych
Infusion reaction → stop + emergency Rx

4. When to seek emergency care

Call 911 or go to the nearest emergency room right away if you have:

Classic PPMS — middle-age male with insidious progressive paraparesis from onset ≥1 y without relapse (Lublin 2014 PMID 24871874)
Active PPMS — gad+ lesion OR new T2 in past 12 mo OR rare relapse → ocrelizumab ORATORIO-eligible (PMID 28002688)
EDSS 4-6.5 + age ≤55 + active disease → ocrelizumab eligible per ORATORIO inclusion criteria (PMID 28002688)
Cervical cord-dominant progressive syndrome — atrophy + lesion + minimal brain involvement; spastic quadriparesis / paresis predominant
Progressive paraparesis dominant — gait + bowel/bladder + sexual dysfunction; rule out vascular myelopathy / B12 deficiency / hereditary spastic paraparesis / NMOSD
NMOSD mimic — LETM > 3 cord segments OR bilateral optic neuritis → AQP4-IgG + MOG-IgG urgent; AVOID IFN/natalizumab (worsen NMOSD)(life-threatening)

5. Follow-up

PT/OT/SLP; spasticity clinic + intrathecal baclofen pump; pulmonary FVC surveillance; palliative + advance directives at EDSS ≥7; caregiver support (AAN 2024)

6. Sources

Guideline: AAN 2024 MS DMT guideline + ECTRIMS 2024 + ORATORIO ocrelizumab PPMS (Montalban NEJM 2017 PMID 28002688)