“My year of collapse” is how a despondent Christy Morrell described the nightmarish months when… Immune system You hijacked his brain
So-called autoimmune encephalitis attacks the organ that makes us “us,” and can appear suddenly.
Morrell went on a bike ride with friends along the California coast, stopping for lunch, and they didn’t notice anything wrong. Morell didn’t do it until his wife asked him how it went – and he forgot. Morel will get worse before he gets better. “Troubled” and “fighting to see the light,” he wrote as delusions developed and gaps in his memory grew.
Of all our ways The immune system can go into overdrive Damaging the body rather than protecting it, autoimmune encephalitis is one of the most difficult diseases to understand. Apparently healthy people suddenly develop confusion, memory loss, seizures, and even psychosis.
But doctors are getting better at identifying it, thanks to discoveries of a growing list of rogue antibodies responsible, which, if found in the blood and spinal fluid, aid in diagnosis. Every year a new comet Antibodies are detectedsaid Dr. Sam Hornig, a neurologist at Mount Sinai Health System in New York who cares for patients with multiple forms of this mysterious disease.
And while Treatment today It includes general approaches to combating inflammation, and there are two major clinical trials underway aimed at more targeted treatment.
It’s still difficult. Symptoms can be confused with other psychiatric or neurological disorders, delaying appropriate treatment.
“When someone has new changes in their mental state, it gets worse, and if there is some kind of strange quality to that, that’s something that would raise our suspicions,” Hornig said. “It is important not to miss a treatable condition.”
With early diagnosis and care, some patients recover completely. Others, like Morell, regain normal daily functioning, but suffer some permanent damage — in his case, the loss of decades of “autobiographical” memories. This 72-year-old literature major can still spout the facts and figures he learned long ago, and make new memories every day. But even family photos can’t help him remember pivotal moments in his life.
“I remember that ‘Ulysses’ was published in Paris in 1922 in the Sylvia Beach Library. Why do I remember that, which is of no use to me anymore, and yet I cannot remember my son’s wedding?” Morel wonders.
Encephalitis means that the brain is inflamed and symptoms can vary from mild to life-threatening. Infection is a common cause, and usually requires treatment of the underlying virus or bacteria. But when that’s ruled out, an autoimmune cause should be taken into consideration, Hornig said, especially when symptoms appear suddenly.
The umbrella term autoimmune encephalitis covers a group of diseases with strange-sounding names based on the antibodies that fuel them, such as NMDA receptor encephalitis.
Although they are not new diseases, the disease got a name in 2007 when Dr. Josep Dalmau, then at the University of Pennsylvania, discovered the first causative antibody, which led to a search for more.
Anti-NMDA receptor encephalitis tends to affect younger women, is one of the odd factors, and is sometimes caused by a “dermoid” cyst on the ovary.
how? Hornig explained that this type of cyst resembles some brain tissue. The immune system can develop antibodies that recognize certain proteins from the growth. If these antibodies enter the brain, they can mistakenly target NMDA receptors found on healthy brain cells, leading to changes in personality and behavior that can include hallucinations.
Different antibodies create different problems depending on whether they mostly affect the memory and mood areas of the brain, or the sensory and motor areas.
“The personality aspects seem to be weak,” Hornig said.
Treatments include filtering harmful antibodies from patients’ blood, injecting healthy antibodies, and taking high doses of steroids to calm inflammation.
Those antibodies attached to the cyst surreptitiously attacked Chiara Alexander in Charlotte, North Carolina, who had never heard of the brain disease. She ignored a few oddities – a little forgetfulness, zoning out for a few minutes – until she found herself in an ambulance due to an epileptic seizure.
Probably dehydration, the first hospital concluded. At a second hospital, after a second seizure, the doctor recognized the possible signs and ordered a spinal tap to detect the causative antibodies.
As Alexander’s treatment began, other symptoms increased. She has little clear memory of her month-long hospital stay: “They said I would wake up screaming. From what I can remember, it was like a nightmare, like the devil was trying to get me.”
Later, Alexandra asked about her 9-year-old daughter and when she could come home, only to forget the answer and ask again.
Alexander feels lucky that her condition was diagnosed quickly, and her ovarian cyst was removed. But it took more than a year to fully recover and return to work full time.
In San Carlos, California, in early 2020, it took months to determine the cause of Morell’s sudden memory problem. He remembered facts and spoke eloquently, but he was losing the ability to recall personal events, a strange combination that prompted Dr. Michael Cohen, a neurologist at Sutter Health, to send him for more specialized tests.
“It’s very unusual, I mean very unusual, to just complain about an autobiographical memory problem,” Cohen said. “One has to think about unusual disturbances.”
Meanwhile, Muriel’s wife, Karen, thought she had detected minor seizures — and one finally occurred in front of another doctor, which helped prompt a spinal tap and diagnosis of LGI1 antibody encephalitis.
It is the most common type in men over the age of 50. These rogue antibodies disrupt how neurons signal to each other, and MRI scans showed they targeted a key memory center.
By then, Morrell, who spent his retirement guiding kayak tours, was no longer able to get into the water safely. He stopped reading, and as his treatments changed, he became agitated by frightening delusions.
“I lost all my mental energy and collapsed,” Morrell describes.
He used haiku poetry to understand the incomprehensible, and after months of therapy he finally wondered if the “medicines flowing through me” were really “putting out the fire. Are they rays of hope?”
The nonprofit Autoimmune Encephalitis Alliance lists about two dozen antibodies — and counting — known to play a role in these brain diseases so far.
Clinical trialswhich is offered at major medical centers across the country, is testing two drugs now used for other autoimmune diseases to see if reducing antibody production can alleviate brain inflammation.
Raising awareness of these rare diseases is critical, said North Carolina’s Alexander, who sought out fellow patients. “It’s a terrible feeling, to feel like you’re alone.”
As for Morell, five years later he still mourns decades of lost memories: family gatherings, the year he spent studying in Scotland, traveling with his wife.
But he’s making new memories with his grandchildren, and getting back outside — and leading the AE Alliance support group, using his haiku to illustrate the journey from “fall apart” to “the present is what I’ve got, sunrise and sunset” to “I can keep hope.”
“I’m returning to a real time of fun and joy,” Morrell said. “I wasn’t shooting for the sake of it. I just wanted to be alive.”
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