“My year of unraveling” is how despairing Christy Morrill described the nightmarish months when he immune system captured his brain.
Something called autoimmune encephalitis attacks the organ that makes us “us” and can appear out of the blue.
Morrill went on a bike ride with friends along the California coast, stopping for lunch, and they didn't notice anything wrong. Morrill didn't know either until his wife asked how it went and he forgot. Morrill will get worse before he gets better. “Frustrated” and “struggling to see the light,” he wrote as delusions and memory holes grew in his mind.
Of all the ways ours the immune system can get out of control and damage the body instead of protecting it, autoimmune encephalitis is one of the most incomprehensible. Apparently healthy people suddenly experience confusion, memory loss, seizures, and even psychosis.
But doctors are getting better at identifying it, thanks to the discovery of a growing list of antibodies responsible that, when found in the blood and cerebrospinal fluid, help make a diagnosis. Every year there's a new culprit antibodies are detected” said Dr. Sam Horng, a neurologist at Mount Sinai Health System in New York who has treated patients with multiple forms of this mysterious disease.
And bye treatment today includes general ways to combat inflammation, with two large clinical trials currently underway aimed at more targeted therapies.
However, it is difficult. Symptoms may be mistaken for psychiatric or other neurological disorders, delaying proper treatment.
“When someone has new changes in their mental state, their condition worsens, and if there is something like a strange quality to it, it kind of increases our suspicions,” Horng said. “It’s important not to miss a treatable disease.”
With early diagnosis and treatment, some patients recover completely. Others, like Morrill, regain normal daily functioning but struggle with some of the inevitable damage—in his case, decades of lost “autobiographical” memories. This 72-year-old literary scholar can still recite long-learned facts and figures, and new memories emerge every day. But even family photographs cannot help him remember key moments in his own life.
“I remember when Ulysses was published in Paris in 1922 in Sylvia Beach's bookstore. Why do I remember this, which I no longer need, and yet cannot remember my son's wedding?” – Morrill is surprised.
Encephalitis means the brain is inflamed, and symptoms can range from mild to life-threatening. Infections are a common cause, usually requiring treatment of the underlying virus or bacteria. But when that's ruled out, an autoimmune cause needs to be considered, Horng says, especially when symptoms occur suddenly.
The umbrella term “autoimmune encephalitis” covers a group of diseases with strange names based on the antibodies that fuel it, such as anti-NMDA receptor encephalitis.
Although these are not new diseases, they gained a name in 2007 when Dr. Josep Dalmau, then at the University of Pennsylvania, discovered the first culprit antibody, sparking a hunt for more.
This anti-NMDA receptor encephalitis usually affects young women and, in one strange factor, it is sometimes caused by an ovarian “dermoid” cyst.
How? According to Horng, this type of cyst has similarities to some brain tissue. The immune system can produce antibodies that recognize certain proteins as a result of growth. If these antibodies enter the brain, they can mistakenly target NMDA receptors on healthy brain cells, causing personality and behavior changes that may include hallucinations.
Different antibodies cause different problems depending on whether they primarily target memory and mood areas of the brain or sensory and motor areas.
Overall, “facets of personality seem disrupted,” Horng said.
The therapy involves filtering harmful antibodies from patients' blood, administering healthy antibodies, and high doses of steroids to relieve inflammation.
These cyst-related antibodies secretly attacked Kiara Alexander of Charlotte, North Carolina, who had never heard of the brain disease. She shrugged off some oddities—a little forgetfulness, passing out for a few minutes—until she ended up in the ambulance with a seizure.
Possibly dehydration, the first hospital concluded. At the second hospital, after the second seizure, the doctor recognized possible signs and ordered a spinal tap, which discovered the culprit antibodies.
When Alexander's treatment began, other symptoms intensified. She has few clear memories of her month-long hospital stay: “They said I just woke up screaming. What I remember is that it was like a nightmare, like the devil was trying to catch me.”
Later, Alexander asked about her 9-year-old daughter and when she could go home, but forgot the answer and asked again.
Alexander considers her lucky that she was quickly diagnosed and had her ovarian cyst removed. But it took more than a year to fully recover and return to full-time work.
In San Carlos, California, in early 2020, it took months to determine the cause of Morrill's sudden memory problem. He remembered facts and spoke eloquently, but was losing memories of personal events—a strange combination that prompted Dr. Michael Cohen, a neurologist at Sutter Health, to send him for more specialized testing.
“It's very unusual, and I mean extremely unusual, to just complain about problems with autobiographical memory,” Cohen said. “We need to think about unusual disorders.”
Meanwhile, Morrill's wife, Karen, thought she had detected mild attacks—and one finally happened in front of another doctor, which helped prompt a spinal tap and a diagnosis of LGI1 antibody encephalitis.
This type most often occurs in men over 50 years of age. These rogue antibodies disrupt neurons signaling to each other, and MRI scans have shown that they target a key memory center.
By then, Morrill, who had led kayak tours in retirement, could no longer get on the water safely. He stopped reading, and as his treatment changed, he began to be plagued by frightening misconceptions.
“I completely lost my mental faculties and fell apart,” Morrill describes it.
He used haiku to make sense of the unknown, and after months of treatment, he finally wondered whether “the drugs running through me” were really “putting out the fire. Rays of hope?
The nonprofit Autoimmune Encephalitis Alliance lists about two dozen antibodies that are known to play a role in these brain diseases.
Clinical trialsoffered at major medical centers across the country, is testing two drugs now used to treat other autoimmune diseases to see if lowering antibody production can improve encephalitis.
Greater awareness of these rare diseases is critical, said Alexander, of North Carolina, who has been searching for other patients. “It’s a terrible feeling, feeling like you’re alone.”
As for Morrill, five years later he is still mourning decades of lost memories: family gatherings, a year of study in Scotland, a trip with his wife.
But he's making new memories with his grandchildren, getting outside again—and leading the AE Alliance support group, using his haiku to illustrate the journey from his “unraveling” to “the present is what I have, sunrises and sunsets” and, finally, “I can hold on to hope.”
“I come back to a time of fun and joy,” Morrill said. “I didn't strive for this. I just wanted to be alive.”
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The Associated Press Health and Science Department receives support from the Howard Hughes Medical Institute's Department of Science Education and the Robert Wood Johnson Foundation. AP is solely responsible for all content.
