JS was in her early twenties and unexpectedly pregnant. Her mother relates that one day in her fourteenth week, for no reason she began to be upset and panicky. This worsened and two days later she started continually drinking, forgetting what she had done, and repeating words and phrases over and over. She started talking to pictures of her dead grandparents.

The following day she was confused and ran outside into heavy traffic. She was admitted to the psychiatric unit with suspected schizophrenia and started on high doses of antipsychotic medication. She did not seem to respond. She remained confused and disconnected and over the next three weeks developed jerky, repetitive movements and stood rigidly at times. The psychiatrist was concerned she had a seizure and a neurology opinion was sought.

When we saw her she was babbling the same word “mamama” repeatedly and loudly. She was agitated and difficult to examine. She appeared psychotic but something about her movements suggested that JS could be suffering from a primary neurological condition.

An electro-encephalogram supported our suspicions: J probably had a recently-defined illness called anti-NMDA (N-methyl D-aspartate) receptor encephalitis.

She was started on “immunotherapy” with high dose steroids and underwent plasma exchange in the intensive care unit under sedation. Slowly she began to turn around and improve, and her agitation and movements settled and she is recovering. Blood tests confirmed the diagnosis. The baby was thankfully not affected.

She started talking to pictures of her dead grandparents

Anti-NMDA encephalitis is one of a group of antibody-mediated brain conditions that are blurring the division between neurology and psychiatry. Anti-NMDA, -VGKC and -GAD are acronyms for the most common of these. They present in a variety of ways but can mimic acute psychiatric states, or rapid onset confusion or dementia. They are not easy to diagnose clinically but are being increasingly frequently diagnosed through a blood antibody test. The diagnosis has to be suspected to order the test however, and there are some patients without detectable antibodies who still respond to immunotherapy. This is probably because there are antibodies that cause this illness that have not yet been identified. If there is sufficient suspicion, a trial of immunotherapy treatment may be warranted.

Although previously thought to be rare, these conditions are being increasingly seen and are presenting a challenge for psychiatric units who are now doing more of these antibody tests, and for neurology units who are having to have special facilities and training to treat agitated and sometimes uncooperative patients. Patients may have to be heavily sedated or put on a respirator in an intensive care unit to enable treatment.

NMDA encephalitis is very rare but particularly occurs in women in their early 20s. Antibodies may come from an ovarian tumour in some cases so this always must be screened for. Older patients are also being increasingly diagnosed with antibody encephalitis. Prior to 2007 when NMDA encephalitis was first diagnosed these patients were probably diagnosed as psychotic. It is very gratifying to see patients recover from what is a frightening experience for both them and their parents.

Patrick Pullicino is professor of clinical neurosciences at the University of Kent.

(Note: JS are not the patient’s real initials)

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