Though its first description was published in Annals of Neurology in 2005, we started to pay atention to NMDA receptor autoimmune encephalitis in 2007, when several more cases and the pathogenic antibodies were described. It was difficult to believe that it could be possible to discover a new disease well into the twenty first century. It was not that a diagostic marker, a rare onset of a known disease or a particular clinical feature were described. It was the description of a disease, like in the Charcot days, but adding the diagnostic test, the pathogenic mechanism and the (almost) miraculous treatment.
This happened just three years ago… After three years of intense publishing, meetings and deserved attention to Dr Dalmau (and co-workers) it turned out that this disease was not that uncommon. Hundreds of cases arose when nobody had been aware of these patients. Most of them may still have an “idiopathic” adjective at their diagnosis field in the discharge report or may have died while their doctors still wonder what could that be.
Some may think that chance always plays a role when such discoveries are made, but in this case an entire life devoted to the study of immunlogic mechanisms of paraneoplastic diseases was the key to success. It’s not just a discovery, it’s a great amount of lab and clinic work. It could have been just another interesting case report but it was the description, not only of a syndrome, but of the basic immunopathology of it as well. More, it was the stablisment of a consistent model of approach to the study of neuro-immune diseases, starting with patients sera to follow with murine models and not the other way. A model that has in anti-NMDAR encephalitis its best example but that has contributed to the description of other auto-antibodies causing treatable encephalitis, such as GABAb, or the re-explanation of the true ethiologic factors that cause diseases previously attributed to antibodies against potassium channels, such as those caused by LGI1 and CASPR2 antiboides.
Now, having shaken the Neuroimmunology stablishment by getting far more attention in immunological issues than MS neuroimmunologists get, it seems, as it is reflected in the last Lancet Neurology paper, that they are trying to explain the physiopathology of the bizarre collection of symptoms the anti-NMDAR encephalitis shows. This will be a different adventure we also will eagerly follow not only because of its interest but because it’ll be another step towards the end of magical explanations of human behaviour. The end of exorcist girls… and the arousal of neurological awareness about a (most of the times) treatable disease that can be, otherwise, devastating.