My former neighbor’s daughter, whom I’ll call “Ricki,” had it all—a great job, great little Manhattan apartment, great life. Only in her early 20s, she already had accomplished much in the fashion world.
But over the course of barely more than a month, all that blew up as Ricki developed increasingly weird and worrisome behaviors.
First, there were memory problems. She couldn’t recall simple things, such as where she normally kept her car keys or the names of her coworkers. The following week, her speech slowed, her body felt numb and her movements became awkward. Soon after, Ricki began hallucinating and a psychiatrist put her on medication.
A week later, she had a seizure and was hospitalized. A huge battery of tests, including a spinal tap and brain MRIs, revealed nothing except a little inflammation…doctors were stumped.
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Then Ricki began having violent spells, leaping at nurses and orderlies. Said to be psychotic, she was transferred to a psychiatric hospital. And there she might have stayed for the rest of her life—if not for a stroke of luck.
Ricki’s father, a retired physician, did not believe that the daughter who had always been so sweet, smart and sane could have become, in a matter of weeks, seriously and irrevocably mentally ill. So he diligently researched her symptoms and discussed her condition with his doctor friends. Fortunately, one of the father’s colleagues suggested consulting Souhel Najjar, MD, a neurologist at NYU Langone Medical Center in New York City.
Dr. Najjar gave the young woman a thorough examination, including a special blood test that revealed antibodies targeting certain receptors in the brain—and within days, he had made the diagnosis. Ricki was suffering from a rare autoimmune disorder called anti-NMDA receptor encephalitis.
DEADLY “DEMONIC” DISEASE
Encephalitis, or inflammation of the brain, usually is the result of a viral infection, but it also can be caused by bacteria, fungi or parasites—or, as doctors now realize, by an autoimmune disorder.
In the case of anti-NMDA receptor encephalitis, severe brain inflammation occurs when the immune system attacks special proteins on the surface of nerve cells in the brain called NMDA receptors. These receptors control various cognitive functions, mood, behavior and personality traits—so when they are compromised, the brain malfunctions.
The resulting symptoms include mood and personality changes, violent outbursts, paranoia, psychosis, memory loss, speech problems, numbness, seizures, involuntary movements, increased heart rate, irregular heart rhythm, slowed breathing and/or decreased levels of consciousness. Patients sometimes sink into an unresponsive catatonic state that may last for weeks. Some experts even suspect that anti-NMDA receptor encephalitis is the true cause underlying many cases of “demonic possession” described in the Bible!
This disease is largely unknown, having first been reported in the medical literature only six years ago. It typically strikes in early adulthood, but it has been diagnosed in children as young as one and in seniors as old as 85. It can strike both genders, though more than 75% of those affected are female.
I contacted Dr. Najjar to learn more about this disturbing and devastating disease. He told me that, though considered rare, anti-NMDA receptor encephalitis may be more common than is currently recognized, with many patients being misdiagnosed with severe psychiatric disorders. “These patients act aggressive, belligerent, violent, psychotic and paranoid. Yet now that we know what the real problem is, we have to wonder how many people have been locked up in psychiatric units for years after they were misdiagnosed,” Dr. Najjar said.
Sadly, some patients with anti-NMDA receptor encephalitis die from medical and neurological complications related to irregular heart rhythm, very low blood pressure, slowed breathing, prolonged convulsions and/or persistent coma.
The earlier that anti-NMDA receptor encephalitis is diagnosed and treated, the better the outcome tends to be. Treatment includes immunotherapy—a heavy-duty dose of steroids and other drugs aimed at suppressing the body’s attack on itself. Typically, Dr. Najjar said, the treatment is effective, and most severe symptoms improve within three to four week after treatment begins. However, patients often need physical, occupational and/or cognitive therapies to regain their ability to walk, talk and function normally, and full recovery may take up to two years.
What causes this disease? That’s not entirely clear. As with other autoimmune disorders, such as lupus or rheumatoid arthritis, a genetic predisposition may contribute to an individual’s risk, Dr. Najjar said.
A certain type of ovarian tumor called a teratoma appears to play a key role, particularly in females of childbearing age. Bizarrely, teratomas often contain skin, hair, tooth and/or brainlike cells. A recent study from the University of Pennsylvania found that up to 55% of female patients under 18 years of age who had anti-NMDA receptor encephalitis also had teratomas in their ovaries. The body’s immune system may be provoked into producing antibodies that attack the abnormal brainlike cells in the tumor, Dr. Najjar explained. Since the antibodies don’t distinguish between the brainlike cells in the tumor and cells in the brain itself, the patient’s brain becomes a battlefield. If a teratoma is found in a patient’s ovary, the ovary needs to be surgically removed.
What’s puzzling is that anti-NMDA receptor encephalitis also can develop in patients who do not have teratomas. Such cases may be the result of some kind of virus that damages the blood/brain barrier, allowing antibodies to enter the brain, Dr. Najjar said. These patients are at greater risk for recurrence, even many years after the initial episode. For instance, the University of Pennsylvania researchers reported that, of the 20% of anti-NMDA receptor encephalitis patients who experienced a relapse, most had not had teratomas.
Lucky lady: Ricki is recovering nicely, thanks to timely intervention. But here’s what all of us should remember from her story…
If a loved one suddenly starts exhibiting psychiatric symptoms that are way out of character, Dr. Najjar said, it is vital to consult a neurologist and ask whether anti-NMDA receptor encephalitis could be the culprit.
Source: Souhel Najjar, MD, associate professor of neurology, director, electroencephalogram laboratory, NYU Langone Medical Center, New York City, and director, Neuroscience Center, Staten Island University Hospital, New York. His role in diagnosing and saving a patient stricken with anti-NMDA receptor autoimmune encephalitis is described in the new book by Susannah Cahalan, Brain on Fire: My Month of Madness (Free Press).