Certainly this time all my fame as a pioneer was not enough to introduce treatment and save the life of a very young child with NMDA receptor encephalitis. It was soon after the New Year in 2010. I was returning from Alacaniz, close to Teruel, in one of the rare times when I have been able to take a couple of weeks of holidays and embark on an adventure trip. This time it was to Portugal and Spain to finalize the second edition of my book Fora da Casinha, uma análise histórica da loucura, which would come out later that year. Alcaniz, by the way, is a medieval castle where the Catholic religion may have been invented.
It is unlikely the family and others involved would have allowed autoimmune therapy in the case of this child, who would have been the youngest patient in the world, probably, at the time. Any how, it was all a bit too much. this child had been brought from the amazon region to a large pediatric hospital in southern Brasil and then refered to me because of this very strange neurological disease which had lasted 5 weeks, consisting of incredibly intense involuntary choreic movements, a psychiatric comatose state, and an infectious state. Slowly, very slowly, over a couple of weeks, we established that the infectious state was due to the excess movement. This was carried out through video-EEG, blood, imaging and CSF studies. When she was kept in a very cold air-conditioned quiet darkened room, the body temperature came down and movements almost stopped. By this time parents and a few other relations had almost gone mad with this situation; and there were other complications. We, my clinic, where the girl was admitted, were not paid, for example. So we discharged the patient to home care, and heard that eventually she died in another town. Later the father died too.
At the time, I studied furiously, looking into all kinds of encephalitis that could lead to this mix of symptoms I had never seen before, and have seen only once since. Unfortunately, it was only a couple of weeks after the girl left when I received the material of the American Academy of Neurology meeting and first heard of Dr Dalmau’s work on NMDA receptor encephalitis. Only a few years later, there is a very long paper by him of another AAN lecture, this time a Cotzias Lecture: NMDA receptor encepahilitis and other antibody-mediated disorders of the synapse. Neurology 2016, 87: 2471-2482.
In this paper, with the help of figures and the freedom of enough text and space, he ventures into a completely new paradigm, that diseases as schizophrenia may be related to the pathophysiology described in these cases. Furthermore, because patients with NMDA receptor encephalitis have a profound amnesia for the period of their disease, and because of other related research findings, memory mechanisms are also involved in these autoimmune pathophysiological mechanisms being now investigated from a completely new viewpoint.
Dr Paulo Bittencourt
