Will Graham and NMDA Receptor Antibody Encephalitis

In Hannibal, Will Graham was affected by NMDA Receptor or Antibody Encephalitis, also known as Anti-NMDAR Encephalitis.

And, just as in the majority of real-life cases, he displayed disturbing psychiatric behaviour, including hallucinations and disorientation.





Here experts Dr Sarosh R Irani and Professor Angela Vincent, of John Radcliffe Hospital, Oxford, give a breakdown of the condition.

What is NMDA-Receptor Antibody Encephalitis?

The major role of our immune system is to recognise and get rid of infection. But sometimes some components of the immune system, called antibodies, may instead react with proteins in our own body causing an autoimmune disease. When this reaction is against proteins in the brain it is called Autoimmune Encephalitis.

Many cases of Autoimmune Encephalitis associated with antibodies against the N-methyl D-aspartate (NMDA) receptor have recently been reported.

NMDA receptor is a protein in the brain that helps control the electrical activity of nerves and therefore antibodies against these receptors are likely to have an important role in directly causing the disease.

Diagnosis

The symptoms and signs seen in patients with NMDA Receptor Antibody associated Encephalitis can be distinctive and are prompting many clinicians to request the NMDA receptor antibody test to diagnose this condition. The disease mainly affects young people, with around 30% of cases under 18 years of age. Women are affected more often than men.

Symptoms

At onset, the most distinctive features include prominent psychiatric symptoms, seizures, confusion and memory loss. Patients will sometimes show bizarre and often rather disturbing behaviours. Typically 10 to 20 days later, patients develop a movement disorder, variations in blood pressure, heart rate and temperature and may become less conscious. The movement disorder often consists of continuous writhing and twitching of face and limbs but can also be a generalised slowing-down of movement. More recently, patients with only a few of these features have been recognized to have NMDA-receptor antibodies.

Treatment

Treatment consists of immune therapies and removal of a tumour, if present. The immune therapies use medicines to dampen down the immune system. These include steroids, immunoglobulins (a blood product given into the vein in a drip) and plasma exchange (when some of a person’s blood is taken out from a vein, washed and then put back into the vein in a drip). In addition, some patients are treated with other drugs which dampen down the immune system. All drugs have known side-effects but their benefits are generally felt to outweigh possible side-effects.

Prompt therapies offer a good chance of substantial recovery in the majority of patients. As they improve there is often a reduction in the amount of NMDA receptor antibody in the person’s blood when the test is repeated. Some patients are now being treated after recognition of the clinical symptoms and signs, while the antibody result is awaited, to try to improve recovery. However, recovery is usually slow and many patients spend a few months in hospital, including time on the intensive care unit undergoing ventilation. Those who return to work typically only do so after a year or two.

In summary, NMDA Receptor Antibody Encephalitis is a recently identified autoimmune disease that causes psychiatric features, confusion, memory loss and seizures followed by a movement disorder, loss of consciousness and changes in blood pressure, heart rate and temperature. The disease can respond well to various therapies that dampen down the immune system and the removal of an underlying tumour if one is found, but improvement is often slow.

Susannah Cahalan

New York Journalist Susannah Cahalan’s account of her battle with Anti-NMDA Encephalitis resulted in the New York Times bestselling book, Brain on Fire: My Month of Madness.

Susannah has since become an Ambassador with The Encephalitis Society and our chief supporter in the United States of America.

Click on the video below to hear Susannah speak about her experiences - and to see similarities between the clock drawn by Will Graham and Susannah.





For more about Susannah, visit www.susannahcahalan.com

*Image of Will Graham may be subject to copyright