Patient handout

Anti-NMDA Receptor Encephalitis

PRODUCTION

1. Your condition

This handout is for anti-nmda receptor encephalitis. Your care team identified this based on: young female (15-45 y) with subacute psychiatric prodrome (psychosis, mania, paranoia) → seizures → movement disorders → autonomic instability + ams (graus 2016 ircns pmid 26906964).

Other reasons your team may use this plan: biphasic neurological relapse 1-6 wk after hsv encephalitis — psychiatric + dyskinesia + new seizures (armangué 2018 pmid 30049614); orofacial dyskinesia + autonomic storms (bp/hr/temperature lability) + ams — icu phenotype (dalmau); pediatric anti-nmdar — often post-hsv triggered; less teratoma association (pmid 34301820).

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 × 5 d adult (30 mg/kg/d × 5 d pediatric, max 1 g/d)	IV	daily × 5 d	First-line acute pulse; combine with IVIG or PLEX; Graus 2016 PMID 26906964; Titulaer 2013 PMID 23290630
IVIG	0.4 g/kg/day × 5 d (total 2 g/kg)	IV	daily × 5 d	First-line combo with steroid; preferred in pediatric + pregnancy; Titulaer 2013 PMID 23290630
plasmapheresis (PLEX)	5 cycles q48h over 10 d	IV/large-bore	q2 days × 5	Alternative to or addition to IVIG for severe presentation; large-bore IV / temporary line; ~50-70% improvement
ovarian teratoma removal	—	—	—	Tumor removal MANDATORY when found — improves outcome significantly (Dalmau; ~50% young women); without removal, immunotherapy alone less effective
prednisone	60 mg PO daily × 2-4 wk then taper over 4-6 mo	PO	daily slow taper	Maintenance taper after IV pulse; slower than MS-flare to prevent relapse; bridge to steroid-sparing

Plan: Anti-NMDAR encephalitis escalating immunotherapy + tumor removal + supportive (Graus 2016 PMID 26906964; Titulaer 2013 PMID 23290630; Titulaer 2013 PMID 23290630)

3. When to call your provider

Contact your care team if any of the following happen:

Breakthrough relapse on rituximab → consider switch or escalate to tocilizumab / bortezomib
New psychiatric / seizure / dyskinesia → ED + steroid pulse
IgG <500 + recurrent infection → IVIG / pause rituximab
Pregnancy confirmed → MFM + neuro coordination
Severe depression / suicidality → urgent psych

4. When to seek emergency care

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

Young female (15-45 y) with subacute psychiatric prodrome → seizures → movement disorders → autonomic + AMS — classic anti-NMDAR pentad; pelvic US for ovarian teratoma ~50% (Graus 2016 PMID 26906964)
Anti-NMDAR with ovarian teratoma found on pelvic US/MRI — TUMOR REMOVAL MANDATORY (improves outcome significantly; ~50% of young women); + first-line immunotherapy (Dalmau)
Pediatric anti-NMDAR — post-HSV triggered more common than adult; less ovarian teratoma association; pediatric dosing methylpred 30 mg/kg/d (max 1 g); IVIG preferred adjunct (PMID 34301820)
Biphasic neurological relapse 1-6 wk after HSV encephalitis recovery — anti-NMDAR autoimmune encephalitis; up to 27% of HSV survivors; psychiatric prodrome + new seizures + dyskinesia (Armangué 2018 PMID 30049614)
ICU phenotype — orofacial dyskinesia + autonomic storms (BP/HR/temperature lability + hypoventilation) + status — dexmedetomidine for agitation, clonazepam + valproate for dyskinesia (Dalmau)(life-threatening)
Pregnancy + anti-NMDAR encephalitis — IVIG safer than PLEX; cyclophosphamide CONTRAINDICATED in pregnancy; rituximab Cat C; MFM + neuro coordination
No improvement at 4 wk on first-line steroid + IVIG + PLEX + tumor removal — escalate to second-line rituximab 1 g × 2 + cyclophosphamide 750 mg/m² monthly × 6 mo (Titulaer 2013 PMID 23290630)

5. Follow-up

Cognitive rehab (memory + executive + attention deficits common post-recovery — 75% return to baseline at 2 y); neuropsychiatry (depression / behavioural change frequent); speech / occupational / physical therapy; AED management if seizure history; pregnancy planning + postpartum surveillance; psych follow-up for late-onset psychosis or relapse; PHQ-9 + cognitive battery at 3 + 6 + 12 + 24 mo

6. Sources

Guideline: Graus 2016 Lancet Neurol autoimmune-encephalitis clinical diagnostic criteria (PMID 26906964) + Titulaer 2013 Lancet Neurol NMDAR treatment & prognostic cohort n=577 (PMID 23290630) + Dalmau 2008 Lancet Neurol original NMDAR case series n=100 (PMID 18851928) + Abboud 2021 JNNP autoimmune-encephalitis management consensus (PMID 33649022 / 33649021) + Armangué 2018 Lancet Neurol post-HSV autoimmune encephalitis (PMID 30049614) + Nosadini 2021 paediatric NMDARE international consensus (PMID 34301820). [depth-pass-2 2026-05-18: prior 6/6 anchor PMIDs were PubMed-MCP-confirmed mis-attributions — corrected; see neuro.encephalitis-anti-nmdar.v1._research-bundle.md]