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Late-onset anti-NMDA receptor encephalitis

Maarten J Titulaer1, Lindsey McCracken, Iñigo Gabilondo

  • 1From the Department of Neurology (M.J.T., I.G., M.R.R., F.G., J.D.), Hospital Clinic, Universitat de Barcelona/Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Spain; Department of Neurology and Neurosciences (M.J.T., L.M., M.R.R., R.B.-G., J.D.), Perelman School of Medicine, Hospital of the University of Pennsylvania, Philadelphia; Department of Neurology (T.I.), Kitasato University School of Medicine, Sagamihara; Department of Neurology (I.K.), Brain Research Institute, Niigata University, Japan; Department of Neurology (L.B.), Hospital Universitario La Fe, Valencia; Biostatistics and Data Management Platform (A.T.), IDIBAPS, Hospital Clinic; and Institució Catalana de Recerca i Estudis Avançats (ICREA) (J.D.), Barcelona, Spain.

Neurology
|August 16, 2013
PubMed
Abstract

Insights

Older adults with anti-NMDA receptor encephalitis experience delayed diagnosis and poorer outcomes compared to younger individuals. Early immunotherapy is crucial for improving recovery in this patient group.

Area of Science:

  • Neurology
  • Immunology
  • Clinical Medicine

Background:

  • Anti-NMDA receptor (NMDAR) encephalitis is a severe autoimmune neurological disorder.
  • Clinical features and outcomes in older adults (≥45 years) are not well-characterized.

Purpose of the Study:

  • To delineate the clinical characteristics and prognosis of anti-NMDAR encephalitis in patients aged 45 and older.
  • To compare outcomes between older and younger adult patients.

Main Methods:

  • Observational cohort study.
  • Analysis of 661 patients with anti-NMDAR encephalitis, with a focus on 31 patients aged ≥45 years.
  • Comparison of demographic, clinical, treatment, and outcome data between age groups.

Main Results:

  • Older patients (≥45 years) were more frequently male and had a lower incidence of tumors (often carcinomas, not teratomas) compared to younger adults.
  • A significant delay in diagnosis (median 8 vs. 4 weeks) and treatment (median 7 vs. 4 weeks) was observed in older patients.
  • Older patients had less favorable outcomes at 2 years (60% vs. 80% with modified Rankin Scale score 0-2), though 60% achieved full or substantial recovery by 24 months.
  • Multivariable analysis identified younger age, early treatment, and absence of intensive care as predictors of good outcome.
  • Rituximab and cyclophosphamide showed efficacy in patients refractory to first-line therapies.

Conclusions:

  • Anti-NMDAR encephalitis presents differently in older adults, with delayed diagnosis and treatment contributing to poorer outcomes.
  • Tumor frequency is lower in older patients, with a different tumor type profile.
  • Prompt and aggressive immunotherapy is recommended to improve clinical outcomes in this demographic.

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