Background Anti-NMDA receptor (NMDAR) encephalitis is a recently characterised autoimmune disorder mainly affecting young women. Although the clinical features of the acute disease are well characterised, cognitive long-term outcome has not been examined in detail.
Methods The authors investigated cognitive performance in nine patients with proven anti-NMDAR encephalitis after recovery from the acute disease period (median 43 months after disease onset, range 23 to 69). Patients underwent a comprehensive neuropsychological assessment, including memory tasks that have previously been shown to be sensitive for hippocampal dysfunction.
Results Substantial persistent cognitive impairments were observed in eight out of nine patients that mainly consisted of deficits in executive functions and memory. The severity of these deficits varied inter-individually. Patients with early immunotherapy performed significantly better. The most severe deficits were observed with inefficient or delayed initial treatment.
Conclusion Our results suggest that cognitive deficits constitute a major long-term morbidity of anti-NMDAR encephalitis. These deficits relate to the distribution of NMDARs in the human brain and their functional role in normal cognition. Good cognitive long-term outcome may depend on early and aggressive treatment.
- limbic system
- cognitive neuropsychology
- intensive care
- clinical neurology
- Whipple's disease
- paraneoplastic syndrome
- eye movements
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Funding This study was supported by the Deutsche Forschungsgemeinschaft (DFG PL 248/4-1).
Competing interests KPW is a full-time employee of and holds stock in Euroimmun. JD receives royalties from the editorial board of Up-To-Date and from Athena Diagnostics for a patent for the use of Ma2 as autoantibody test. JD has received a research grant from Euroimmun, and his contribution to the current work was supported in part by grants from the National Institutes of Health and National Cancer Institute RO 1CA89054, 1RC1NS068204-01, and a McKnight Neuroscience of Brain Disorders award.
Ethics approval The Charité University Hospital ethics committee.
Provenance and peer review Not commissioned; externally peer reviewed.