Anti-NMDA receptor encephalitis natural history, complications and prognosis

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Anti-NMDA receptor encephalitis Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] AE; Template:D Makkar

Overview

Anti-NMDA encephalitis follows a typical course: initial neuropsychiatric symptoms progress to neurological symptoms.

The acute phase can be severe, potentially lasting weeks to months. Early immunotherapy and tumor removal improve outcomes. Gradual symptom improvement occurs, but recovery time varies. Long-term recovery may take months to years with possible relapses. Mortality is linked to complications such as infection, cardiac arrest, and respiratory distress. The prognosis improves with early treatment, fewer severe symptoms, and tumor excision.

Natural History

Onset: Initial symptoms often involve neuropsychiatric manifestations, followed by neurological symptoms such as seizures, movement disorders, and altered consciousness.

2. Acute phase: Symptoms worsen, leading to severe neurological deficits and potentially life-threatening complications. This phase can last for weeks to months.

3. Treatment: Early initiation of immunotherapy and tumor removal, if present, is crucial for optimal outcomes and improved prognosis.

4. Gradual improvement: With appropriate treatment, most patients experience a gradual improvement in symptoms. Recovery occurs in a stepwise manner, but the time frame can vary.

5. Long-term recovery: Neurological and psychiatric recovery may take several months to years. Close long-term follow-up is essential to monitor for relapses and provide ongoing management.

6. Relapses: Some individuals may experience relapses, particularly during the early stages of recovery, triggered by infections or other factors.

Complications

Among the causes of mortality were infection, sudden cardiac arrest, severe respiratory distress, refractory status epilepticus, tumor growth, and discontinuation of life-sustaining treatment.

Prognosis

First-line immunotherapy led to an improvement in 53% during the first 4 weeks of treatment, with 97% of this "improved" group exhibiting a statistically significant improvement positive result at 24 months. The 47% of patients who did not improve with first-line therapy and who received second-line therapy had better results than those who received only first-line therapy or no further immunotherapy (Barry et al., 2015). In addition to early detection and treatment, other positive prognostic variables include fewer severe symptoms and, if present, excision of the tumor.

References

[1] [2] Template:WH Template:WS

  1. Dalmau J, Gleichman AJ, Hughes EG, Rossi JE, Peng X, Lai M; et al. (2008). "Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies". Lancet Neurol. 7 (12): 1091–8. doi:10.1016/S1474-4422(08)70224-2. PMC 2607118. PMID 18851928.
  2. Titulaer MJ, McCracken L, Gabilondo I, Iizuka T, Kawachi I, Bataller L; et al. (2013). "Late-onset anti-NMDA receptor encephalitis". Neurology. 81 (12): 1058–63. doi:10.1212/WNL.0b013e3182a4a49c. PMC 3795591. PMID 23946310.