A U.S. film about a rare but serious disease that impairs the body’s autoimmune system is being shown in Japan, boosting hope among patients and their families that it will help raise awareness of the little-known illness.
“Brain on Fire,” released in Japan on Dec. 16, is based on a 2012 memoir of the same name by journalist Susannah Cahalan, who in 2009 came down with the disease, called anti-NMDA receptor encephalitis.
The book vividly depicts how Cahalan, then a 24-year-old budding reporter at the New York Post, started experiencing fatigue, numbness, insomnia and memory loss. Her condition worsened in the following weeks as she started exhibiting violent and psychotic behavior and suffered from seizures.
The mysterious disease went on to rob her of her ability to speak and walk, eventually sending her into an unresponsive stupor.
All of this took place within about a month. This type of dramatic deterioration in health long had no diagnostic name and no cure. It was only in the mid-2000s that researchers found that the symptoms are caused by antibodies produced by the body’s immune system attacking NMDA receptors in the brain, impairing the hippocampus, which controls memory, as well as the frontal lobes, which control high-level brain functions and personality.
Because Cahalan’s onset of the illness came only two years after the disease was identified and reported in a scientific journal for the first time, and because its incidence is so low — some experts say it afflicts only 1 in 3 million people — few people, even medical professionals, were aware of it back then. It thus took her weeks and a battery of tests before she was given the correct diagnosis and started receiving treatment. In fact, she was nearly sent to a psychiatric ward after being mistaken for having bipolar disorder or schizophrenia.
Cahalan, who was in Japan earlier this month to promote the film, said her aim in writing the book was to empower patients suffering from any disease.
“I think one of the most important lessons is that you have to be your own advocate, and if you can’t be your own advocate, there needs to be a support structure in place,” Cahalan, now fully recovered, told The Japan Times. “And I think people need to be comfortable questioning the authority of a doctor. … I hope that the story will give support to people who are in the midst of something like this and give people (strength) to question if they feel their doctor is not listening to them or they are not being really heard.”
Misae Kataoka, 61, who watched the film and met Cahalan at a screening event in Tokyo, said she was deeply inspired by the story. Kataoka’s 38-year-old daughter, Kozue Ito, has been battling the same disease for more than four years.
According to Kataoka, her daughter’s illness started in May 2013 with seemingly innocuous symptoms, such as headaches and high fever. Then one night in August that year, while she was at home in Saitama Prefecture watching TV with her husband, she suddenly blurted out that she “would be killed” by a character in a popular TV series, and started wandering about the room while staring at the ceiling. Her husband called an ambulance and she was taken to a university hospital in the city of Saitama, but she fell into a coma two days later.
Like in Cahalan’s case, the hospital had never seen a patient with anti-NMDA receptor encephalitis, so the diagnosis was late to come. By the time treatment started, which included infusions of steroids and intravenous immunoglobulin (IVIG) — a blood product made from the serum of blood donors — and plasma exchange, three months had elapsed.
Dr. Satoshi Kamei, professor and chairman of the division of neurology at Nihon University School of Medicine in Tokyo, who reported the first cases of this then-nameless encephalitis to an academic conference in Japan in 2004, said the disease typically starts with flu-like symptoms, which then progress to excitement, delusion and hallucination. He said patients then fall into unresponsiveness, where their eyes are open but they do not respond to stimulus or pain. It is usually at this time when many develop difficulty breathing and are placed on an artificial respirator in addition to having rigid, involuntary body movements and seizures, he said.
Though research into the disease has made advances in recent years, exactly what triggers it remains a mystery. According to an international study of 565 patients presented to a 2012 meeting of the American Academy of Neurology, 81 percent of the patients were female, and a majority got sick when they were in their teens or 20s.
The same research showed that 39 percent had teratomas in their ovaries, in which case a removal of the tumor is known to significantly improve the condition. But the remaining patients had no teratomas.
Dr. Mikio Hirayama, a counselor at Kasugai General Health Care Center in Aichi Prefecture — who as a neurologist has seen two women with the disease in 2007 and 2008 — said early diagnosis is key, as it severely influences the recovery process.
“If you are diagnosed early it really gets better,” he said. “Now I think most hospital doctors know about the disease, so I think there is less chance of a misdiagnosis.”
He added, however, that more awareness is needed, especially among psychiatrists in private practice, some of whom might not be familiar with the disease and could be confused by patients showing psychotic symptoms.
Cahalan said she was lucky to have been correctly diagnosed and treated. But she says she also wants to emphasize the long and winding path to recovery that many patients and their families face after diagnosis.
“The part that the movie doesn’t explore is the recovery part, which of course I understand isn’t the exciting Hollywood part,” she said. “But recovery is a long experience. For some people, they never fully recover, and in my case it took me about a year to recover to where I am now. … I hope to impart to these people who are going through this disease or any disease that takes recovery that you have just to be patient, and you have to almost see this horrible moment as an opportunity to reassess your life.”
Kataoka, for her part, says she wants to join forces with other families of people with the disease, to share knowledge, offer support and campaign for better therapy options. In June, Kataoka set up the nation’s first patient support group, which more than 40 families have joined.
Her daughter’s recovery has been mind-bogglingly slow for Kataoka, too, but she has not given up. Thanks to her tireless care, Ito is getting better.
In August, after days of high fever, Ito developed sepsis, or a complication of an infection, and nearly died. Doctors found that she had a huge pocket of water in her kidney, measuring about 30 cm in width, Kataoka said.
Ito went through a five-hour surgery to have the build-up, called renal abscess, removed. Then on Aug. 25, some three weeks after the surgery, Ito suddenly regained consciousness — for the first time in four years.
“I’ve visited her at the hospital every day, and her eyes would be open at times, but they never followed people, even when nurses came into the room,” Kataoka said. “But on that day, I noticed her eyes were following nurses, and she smiled at them. When I asked her if she could identify me, she nodded.”
For more information about the film “Brain on Fire,” titled “Kanojo ga Mezameru Sonohi Made” in Japanese, visit kanojo-mezame.jp