Abstract
In recent years, the discovery of antibodies to specific neuronal antigens that then go on to cause encephalitis has gone a long way to change the investigation and management of a potential encephalitic process. These have now become known under the umbrella term of the \'autoimmune encephalitides\'. In this article we look at anti- N-methyl-D-aspartate receptor encephalitis, a condition most often found in young females and has an association with a number of malignancies, most commonly ovarian teratomas. Most patients will have a viral prodrome, followed by psychiatric, seizure, dysautonomic and dyskinetic features, but can present at any point along this pathway. Treatment involves prompt tumour identification and removal where appropriate and initiation of immunosuppressive therapy, usually commencing with corticosteroids. A substantial proportion of patients will make a full recovery, but many will need medical, psychiatric and social care following completion of the acute phase of the illness.
Highlights
In the last few years there has been the identification of a number of new neuronal antibody targets that are proven to be the underlying cause of what are under the umbrella term of the autoimmune encephalitides
With increasing physician awareness more and more information regarding the epidemiology of anti-NMDAR encephalitis is known and the exact incidence cannot yet be fully determined
Anti-NMDAR encephalitis has been reported to have a predilection in Asian and Pacific Islanders [3] and the finding of associated ovarian teratoma is more common in Black females [2]
Summary
In the last few years there has been the identification of a number of new neuronal antibody targets that are proven to be the underlying cause of what are under the umbrella term of the autoimmune encephalitides. Hughes et al [12] cultured hippocampal neurones in cerebrospinal fluid (CSF) containing anti NR1 receptor antibodies This led to a decrease in the number and density of NMDA receptor clusters. Unlike other forms of limbic encephalitis, there was no lymphocytic infiltration [13] These findings are interesting, given that in the laboratory no structural damage was observed to the neurones, but the presence of gliosis would imply that there must be an inflammatory aspect at some point during this process leading to scarring and may well explain the prolonged or incomplete recovery that many patients go on to have. Low levels of complement in the CSF implies this pathway is not involved in the disease [15] and a lack of disruption in the blood brain barrier in pathologically examined specimens [14] means it is not completely clear what the mechanism is that starts the autoimmune process
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