Autoimmune Encephalitis interventions

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Julinka Auta Fernandes

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Overview

Autoimmune encephalitis may require therapeutic plasma exchange and procedures to manage respiratory failure, severe autonomic dysfunction, nutritional difficulties, or refractory catatonia. Interventions are selected according to disease severity and complications and should accompany appropriate immunotherapy and treatment of an associated neoplasm.[1]

Therapeutic Plasma Exchange

Indications

Autoimmune encephalitis may be treated with therapeutic plasma exchange (TPE), also commonly abbreviated PLEX. The procedure removes circulating antibodies and other inflammatory mediators. The 2024 Canadian consensus recommends high-dose corticosteroids combined with either plasma exchange or intravenous immunoglobulin (IVIG) for severe adult disease. Plasma exchange is a first-line option rather than exclusively a rescue treatment.[2]

Treatment Course

Autoimmune encephalitis treatment protocols vary. The Canadian consensus describes five exchanges on alternate days over approximately ten days in adults. The apheresis team individualizes vascular access, exchange volume, replacement fluid, and treatment frequency.[2]

Autoimmune encephalitis treatment sequencing requires coordination because plasma exchange can remove recently administered immunoglobulin or therapeutic antibodies. Schedule IVIG and antibody-based medicines with the treating neurology and apheresis teams.[1]

Plasma removed during therapeutic plasma exchange in a patient with autoimmune encephalitis. Photograph by NotSB10, via Wikimedia Commons, licensed under Creative Commons Attribution–ShareAlike 4.0.[3]

Risks and Monitoring

Autoimmune encephalitis patients undergoing plasma exchange require assessment of hemodynamic stability, bleeding risk, vascular access, and ability to tolerate the procedure. Important complications include:

  • Hypotension and fluid shifts.
  • Electrolyte disturbances.
  • Bleeding or coagulation abnormalities.
  • Catheter-associated infection or thrombosis.
  • Difficulty completing treatment in patients with severe agitation or autonomic instability.[1]


Respiratory Interventions

Autoimmune encephalitis can cause central hypoventilation, reduced consciousness, and impaired airway protection. Endotracheal intubation and mechanical ventilation may be necessary when breathing or airway protection is inadequate. Severe dysautonomia or refractory status epilepticus may also require intensive-care management.[1]

Autoimmune encephalitis with prolonged ventilatory dependence may require consideration of tracheostomy. The decision depends on the anticipated course, airway needs, and overall condition; no fixed autoimmune encephalitis-specific timing applies to every patient.[4]

Cardiac Monitoring and Pacing

Autoimmune encephalitis-associated dysautonomia may cause marked heart-rate fluctuations, severe bradycardia, or asystole. Patients with clinically significant rhythm disturbances require continuous cardiac monitoring and urgent cardiology and intensive-care assessment.[4]

Autoimmune encephalitis with recurrent, life-threatening bradyarrhythmias may require temporary or, in selected cases, permanent pacing. Pacing is not routine treatment for uncomplicated sinus tachycardia or an isolated abnormal electrocardiogram. The potential reversibility of autonomic dysfunction should inform device selection and follow-up.[4]

Nutritional Support

Autoimmune encephalitis may impair swallowing, awareness, or the ability to maintain adequate intake. Swallowing assessment, aspiration precautions, and dietetic review guide the need for enteral feeding. Nasogastric feeding or gastrostomy may be considered according to expected duration and clinical circumstances.[4]

Electroconvulsive Therapy

Autoimmune encephalitis complicated by severe, treatment-resistant catatonia may warrant consideration of electroconvulsive therapy (ECT). The Canadian consensus describes its use in selected refractory cases; evidence is predominantly observational rather than derived from randomized trials.[2]

Autoimmune encephalitis treatment with ECT requires coordinated assessment by psychiatry, neurology, and anesthesia, particularly when seizures, autonomic instability, or respiratory compromise are present. ECT addresses selected neuropsychiatric complications and does not replace disease-directed immunotherapy.[2]

Interventions in Children

Autoimmune encephalitis in children requires pediatric procedural expertise and age-appropriate monitoring. For N-methyl-D-aspartate receptor (NMDAR) antibody encephalitis, international consensus includes plasma exchange among first-line treatments, particularly in severe disease. Vascular access, hemodynamic tolerance, agitation, and procedural burden influence its feasibility.[5]

See pediatric considerations.

Associated Tumor Management

Autoimmune encephalitis associated with a relevant neoplasm requires prompt specialist evaluation for tumor treatment, including resection when appropriate. Tumor-directed treatment should be coordinated with immunotherapy rather than deferred until neurological recovery.[2]l


For tumor-directed surgery, see surgery. For investigation of an underlying tumor, see screening for an associated neoplasm.

Follow-Up

Autoimmune encephalitis interventions require repeated assessment as neurological function improves. Review continuing needs for vascular access, ventilatory support, feeding tubes, and cardiac devices, while monitoring for procedural complications. Rehabilitation and multidisciplinary discharge planning remain essential.[4]

For additional management, see medical therapy and symptomatic and supportive therapy.

References

  1. ↑ 1.0 1.1 1.2 1.3 Abboud H, Probasco JC, Irani S, et al. Autoimmune encephalitis: proposed best practice recommendations for diagnosis and acute management. J Neurol Neurosurg Psychiatry. 2021;92(7):757–768. doi:10.1136/jnnp-2020-325300. PMID:33649022.
  2. ↑ 2.0 2.1 2.2 2.3 2.4 Hahn C, Budhram A, Alikhani K, et al. Canadian Consensus Guidelines for the Diagnosis and Treatment of Autoimmune Encephalitis in Adults. Can J Neurol Sci. 2024;51(6):734–754. doi:10.1017/cjn.2024.16. PMID:38312020.
  3. ↑ NotSB10. Plasma from patient with autoimmune encephalitis removed by PLEX. Photograph taken May 28, 2021. Wikimedia Commons. Own work; Creative Commons Attribution–ShareAlike 4.0.
  4. ↑ 4.0 4.1 4.2 4.3 4.4 Abboud H, Probasco J, Irani SR, et al. Autoimmune encephalitis: proposed recommendations for symptomatic and long-term management. J Neurol Neurosurg Psychiatry. 2021;92(8):897–907. doi:10.1136/jnnp-2020-325302. PMID:33649021.
  5. ↑ Nosadini M, Thomas T, Eyre M, et al. International Consensus Recommendations for the Treatment of Pediatric NMDAR Antibody Encephalitis. Neurol Neuroimmunol Neuroinflamm. 2021;8(5):e1052. doi:10.1212/NXI.0000000000001052. PMID:34301820.