Patient handout

Neuromyelitis Optica Spectrum Disorder (NMOSD)

PRODUCTION

1. Your condition

This handout is for neuromyelitis optica spectrum disorder (nmosd). Your care team identified this based on: aqp4-igg positive (cell-based assay) + ≥1 core clinical event per wingerchuk 2015 (pmid 26092914).

Other reasons your team may use this plan: letm ≥3 contiguous vertebral segments on mri — cardinal nmosd feature (wingerchuk 2015 pmid 26092914); severe on — bilateral simultaneous, or severe unilateral with poor recovery + chiasmal lesion — nmosd phenotype; intractable hiccups + nausea/vomiting ≥48 h afebrile — area postrema dorsal medulla lesion (wingerchuk 2015 pmid 26092914).

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
methylprednisolone	1 g IV daily × 3-5 d (typically 5 d)	IV	daily × 3-5 d	First-line acute pulse; reduces inflammation; same scaffolding as MS-flare ONTT-style; sample AQP4 BEFORE steroid if feasible (steroid lowers titer)
prednisone	60 mg PO daily × 2 wk then taper over 4-8 wk	PO	daily taper	Slow oral taper after IV pulse to bridge to DMT initiation; NMOSD relapses often rebound during steroid taper

Plan: NMOSD acute relapse + chronic AQP4+ DMT (PREVENT eculizumab PMID 31050279 / N-MOmentum inebilizumab PMID 31495497 / SAkura satralizumab PMID 31774956)

3. When to call your provider

Contact your care team if any of the following happen:

Breakthrough relapse on DMT → consider switch to higher-efficacy biologic
New ON / LETM / area postrema → ED + steroid pulse + PLEX trigger
IgG <500 + recurrent infection on B-cell depletion → IVIG / pause
Hepatotoxicity (LFT >3× ULN) on AZA/MMF → pause + workup
Severe lymphopenia on biologic → pause + workup
Pregnancy confirmed → MS-NMO + MFM coordination
Fever / systemic infection on eculizumab → urgent w/u (meningococcal sepsis)

4. When to seek emergency care

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

AQP4-IgG positive with ≥1 core clinical event (ON / LETM / area postrema / brainstem / diencephalic / cerebral) per Wingerchuk 2015 (PMID 26092914)
Intractable hiccups + nausea/vomiting ≥48 h afebrile + dorsal medulla lesion — area postrema syndrome (Wingerchuk 2015 PMID 26092914)
Acute myelitis with LETM ≥3 contiguous vertebral segments — strongly suggests NMOSD vs MS (Wingerchuk 2015 PMID 26092914)
Severe ON — bilateral simultaneous or severe unilateral with NLP / chiasmal involvement / poor recovery — NMOSD phenotype
Brainstem syndrome with characteristic NMOSD lesion (periependymal 4th ventricle, dorsal medulla)
Pediatric NMOSD — overlaps with ADEM phenotype; AQP4-IgG+ children rarer; rituximab off-label first-line
Pregnancy in known NMOSD — relapse rate increases in postpartum window (first 6 mo); plan DMT continuity (eculizumab Cat B preferred; AZA Cat D but used)

5. Follow-up

Rehab (PT/OT/SLP); ophthalmology q3-6 mo if ON; urology for neurogenic bladder; PCP for vaccinations + bone health; mental health screen (depression highly comorbid); pregnancy planning + postpartum surveillance (60% relapse peak); MS-NMO specialty clinic q3-6 mo

6. Sources

Guideline: Wingerchuk 2015 IPND criteria (PMID 26092914) + PREVENT (Pittock NEJM 2019 PMID 31050279) + N-MOmentum (Cree Lancet 2019 PMID 31495497) + SAkura (Yamamura NEJM 2019 PMID 31774956) + AAN 2024 NMOSD