In her New York Times bestseller Brain on Fire: My Month of Madness, journalist Susannah Cahalan describes dreadful psychological experiences associated with her eventual diagnosis of autoimmune encephalitis. Compared with other forms of autoimmune disease and other types of encephalitis, knowledge about this particular diagnostic entity seems to be unfamiliar to most physicians; the medical literature discussing it has been sparse. A Google search in early 2010 yielded nothing. Mayo Clinic published the results of its 7-year study of 72 patients with “suspected autoimmune dementia” along with an accompanying editorial in October of that year—only a few months after I had recovered from the condition. Only in 2016 have the first broadly accepted diagnostic criteria been published in The Lancet Neurology.
My story began in the wee hours of Saturday, September 13, 2008, when Hurricane Ike struck Galveston Island. My husband Jack and I returned to the island the following Tuesday to find ourselves homeless. Most of the island’s residents were in shock, and the situation only grew worse as we began dealing with the aftermath.
Being so overwhelmed, we joked among ourselves about not being able to find or remember anything, which was of course extremely discombobulating. We wondered if we were experiencing some kind of post-traumatic stress syndrome . One afternoon in March 2009, as Jack and I stood in the driveway talking with our contractor, I started to speak, but when I got halfway through the sentence, I didn’t have the vaguest idea where I was heading with it. I began to notice that I was having difficulty stringing sentences together in conversations; soon, my contractor was providing words that had become inaccessible to me. As a writer and editor, I found this disturbing but at first attributed it to general confusion caused by our stressful situation.
One evening shortly thereafter, I had returned to our temporary beachfront apartment and was checking my e-mail messages. I opened a message, and although I thought I understood its content, I could not manage to read through the message. I decided that reading it aloud, one word at a time, might force me to work my way through the entire message. When I began reading it aloud, what I heard coming out of my mouth was total gobbledygook. I was utterly stunned as I realized that I had absolutely no control over my speech. Thoroughly frightened, I immediately turned off the computer and waited anxiously for my husband’s return. That terrifying experience was never repeated, but it was enough to get me into a neurologist’s office early in April 2009.
Office Visit with Neurologist, mid-April 2009
In my visit to the neurologist, he ran me through the usual battery of mental status and functioning tests. I remember it taking me a painfully long time to count down from 100 by 7s. Neuropsychiatric testing showed evidence of generalized cognitive slowing but without any focal deficits. Eventually, I was told that I had experienced some kind of “neurological event” in my left frontal brain. The neurologist prescribed a drug called Keppra, which he said “might” help. By the end of June, most of my symptoms had disappeared, and soon I was able to resume my medical editing work and finish repairing our house from the storm damage.
Early in 2010, Jack and I were planning a large party to celebrate our 50th wedding anniversary. During the last week in February, just one week before the festivities, I noticed some of the same symptoms I had experienced the year before: particularly, difficulty gathering my thoughts together for oral expression. Also, I was somewhat unsteady on my feet and my appetite had diminished. I immediately sent an e-mail informing the neurologist of these symptoms:
Here we go again. The third week of February 2010, I found myself having difficulty figuring out where I was going with a sentence or being unable to think of a word to complete a thought—just like last year. And again, my handwriting is laborious and has become smaller. I feel a little shaky and frail, and I don’t have my usual feeling of being in control.
We decided to investigate what was going on as soon as the weekend celebration concluded. Over the next couple of weeks, I attended to a previously scheduled colonoscopy and other matters that needed attention before my condition totally overtook my life—and, I should add, to a great extent that of my husband.
First UTMB Hospitalization, March 16-18, 2010
On March 16, I was admitted to the University of Texas Medical Branch (UTMB) Hospital with the Chief Complaint of “Worsening brain function.” By this time, I was complaining of generalized cognitive slowness and speech difficulties along with intermittent memory problems. I was unable to operate my cell phone and was encountering difficulty turning on my personal computer, setting our house thermostat, and regulating the temperature of my bath water. Cognitive testing revealed a dramatic decline in processing speed and a fairly dramatic decline in all domains.
An MRI of the brain yielded unremarkable results. An electroencephalogram on March 17 showing focal slowing over the left temporal and parietal regions of the brain was interpreted as abnormal. A follow-up EEG on March 24 was again abnormal and described as “slightly worse than previous EEG.”
As best I recall, I was very weak, unsteady, passive, and rather apathetic. My penmanship became so cramped and laborious that eventually I could not even write my name. Of course, I had stopped driving in the middle of March. Even I knew that I would have had no business trying to drive a car, but going anywhere had become such an effort, I really didn’t care. I was content to rest a lot and mostly do nothing. I have always enjoyed books but had stopped reading earlier in the year, which in retrospect should have been a sign that something was wrong.
Our son returned from Philadelphia later in the month, contacted my clients, attended to financial matters that I usually managed, and lent support to my long-suffering husband. They did not feel that I should be left alone, which I thought was unwarranted, but I realized on some level that because I could neither dial a telephone nor use a computer nor drive a car, if something untoward were to happen, I would have been in quite a pickle. Apparently, I was even unable to count from 1 to 10. Jack arranged for a leave of absence so that he could stay home and take care of me.
In a March 22nd e-mail to friends and family, our son described my status with more objectivity than I could:
“. . . over the past year and a half she has experienced a few episodes of memory loss that fortunately seemed to go away over time. In the week prior to the party, some of her symptoms reappeared. . . . A few days after the party, her memory loss greatly accelerated to the extent that she was unable to perform some basic functions, such as turning on a computer, identifying a computer mouse, using a phone, and articulating thoughts. She previously had not had such memory difficulties. The problems persisted for several days, and last week she was admitted to the hospital and spent several days undergoing tests to identify a cause and treatment. Unfortunately, the tests have been inconclusive, and the best description that we have been given is that she has decreased brain function. What we do know is that she does not have a degenerative brain disorder. Alzheimer’s has been ruled out. However, the doctors believe she has some issue very deep inside her brain. More tests are forthcoming.
Currently, my mother labors with her speech. Often she knows what she wants to say, but cannot make the words come out. Her ability to communicate is challenged. She also has trouble with numbers, such as counting, dialing, and writing. . . . She is in a constant state of tiredness and moves very slowly. Although we are trying to reteach her basic functions, it is not clear if she is learning. Many of you know my mom as a good conversationalist and never at a loss for words or an opinion. She no longer exhibits that loquaciousness,
My mother is very cognizant of her impairments. This makes her frustrated, irritable, and depressed. We are told that her self-awareness is a good thing, although at the moment it is hard to believe.
I understand that I was very stubborn and unreasonable during this time. One weekend evening, because our freezer was overflowing with meals that many of our good friends had brought us, I decided that we should invite guests over for dinner. Jack tried to talk me out of this, but I assured him that it would be a piece of cake. All we had to do was place the food in the microwave and zap it, so I invited a couple of friends to come over and share our bounty. Shortly after they arrived, one of Jack’s trainees called and asked if she could come over to visit with me. So soon we had five people to feed. I went into the kitchen, placed the food in the microwave, stood in front of the microwave, and literally could not figure out how to press the START button. I had to call my friend into the kitchen to do it for me. Of course, that gave my loving husband a great opportunity to say “I told you so.”
Follow-up Office Visit, March 29, 2010
I returned for a follow-up office visit with the neurologist on March 29. The Medical History noted that “Recent hospitalization documented significant global worsening of cognitive function and severe problems with use of complicated devices (electronic telephones/computers).” Further, it mentioned that “the lab work performed thus far does not identify the etiology of this patient’s process.”
Fatigue plus anxiety and depressed mood were noted. The Review of Systems stated “She feels confused but denies delusions, homicidal ideations, and suicidal ideations.” Mental status was evaluated as normal. The assessment was encephalopathy, with the most likely etiologies considered to be metabolic and inflammatory. The plan called for immediate hospital admission so that a lumbar puncture could be performed.
Second UTMB Hospitalization, March 29-31
From the neurology clinic office visit, I was sent directly to be admitted to the hospital. I felt sort of like a game piece in Monopoly, progressing from square to square. Throughout this entire ordeal, I was astonished at my uncharacteristic acceptance of the situation and my passivity.
The lumbar puncture was performed, and the results for the most part were unremarkable. The Final Diagnosis was “Neuro-cognitive function deficit of unknown reason.”
The Quandry Continues
So there we were, not much closer to a confirmed diagnosis than we had been almost a month earlier. Although the laboratory findings did not confirm it, the neurologist told us that he suspected a condition called autoimmune encephalitis. We could find almost nothing in the medical literature about this condition. When my husband’s colleagues asked him what was wrong with me and he told them, they replied consistently either “I’ve never heard of it” or “I’ve heard of it but don’t know what it is.” Jack spent every night frantically calling medical colleagues throughout the country, but no one had anything to offer. Meanwhile I became less and less able to process the actions that I knew how to perform, and he was trying frantically to figure out what he was going to do with this useless woman.
Next, the neurologist wanted to order positron emission tomography (a PET scan). Recovering from the hurricane, UTMB did not have that capability then, so the neurologist referred me to a colleague at Methodist Hospital in Houston, where that imaging could be performed. My UTMB neurologist knew that the Houston neurologist had had experience treating patients with autoimmune encephalitis. In fact, I learned later that he had treated 15 such patients over the course of his practice.
Methodist Hospital admission, April 16-21
I was admitted to Methodist Hospital on April 16, underwent the PET scan, which also was not confirmatory, and treatment was begun the next day. For five days, megadoses (250 mg twice a day) of a steroid called prednisolone were administered intravenously.
Once home again, I found the 21 days of tapering off the prednisolone exceedingly uncomfortable. I had never taken so much medicine and felt very strange. I continued to feel useless, frail, tired, and weird. Slight improvement had occurred; however, when I went to the polls to vote early in May, I had to sign my name with an “X.”
Therapy at Transitional Learning Center
My discharge summary from Methodist Hospital had called for further therapy. Following screening at home on April 30, I was accepted for treatment at the Transitional Learning Center (TLC) in Galveston, a facility that specializes in providing post-acute care for patients with acquired brain injuries. I began the program on May 5.
The initial speech and language pathology evaluation found decreased cognitive-linguistic skills, characterized by decreased orientation, memory, thought organization, problem solving, and abstract reasoning. Pragmatic skills were judged appropriate. I needed consistent cues or memory aids to recall complex and novel information, and minimal cues to plan and follow through on complex future events. I was unable to recall a 3-digit number. Thought organization was characterized by slightly impaired divergent and convergent categorization, slightly impaired visual sequencing, and delayed responses. Verbal problem solving was characterized by appropriate cause and effect, effective solutions, and delayed responses. Abstract reasoning was characterized by good understanding of figurative language but impaired logical reasoning.
Over the next 2 ½ months, I reported twice weekly to TLC. I had only a few sessions of physical and occupational therapy as the major emphasis of my therapy proved to be in the areas of cognitive thought and deductive reasoning. The speech therapist worked with me on these processes, and I was given exercises to perform as homework between sessions. One of my favorites was to work out the dinner table placement of guests whose relationships to one another were described in the assignment. The improvement that occurred was very exciting. One day, I couldn’t figure out how to turn on my computer; several days later, I could miraculously do it. Little steps of progress like that amazed me. I marveled at the brain’s ability to repair itself. I was discharged by TLC on July 22, 2010, and shortly thereafter by my Methodist Hospital neurologist.
Autoimmune Encephalitis Today
At present, autoimmune encephalitis is not considered quite as rare as it was even a few years ago. I have to wonder if this is because many of its victims simply may have gone undiagnosed because so little has been known about it. My diagnosis was based on clinical symptoms rather than actual laboratory confirmation. In her book, Cahalan states that the disease mostly afflicts young people. My age was 74 years when I suffered it, and while my experience had some similarities to hers, particularly in the frustrating attempts to find a diagnosis, the autoimmune encephalitis presented quite differently in my case. I had no seizures, hallucinations, delusions, or psychoses.
As I write this narrative now, my age is 81 years. I continue my professional writing and editing as well as involvement as a faculty wife, a grandmother, a community board member, a college chorale member, a passionate opera lover, an avid reader, and an enthusiastic traveler. I am thankful to have recovered from my bout with autoimmune encephalitis and grateful for the care I received.
The Autoimmune Encephalitis Alliance is working to raise awareness of this difficult-to-diagnose condition. I hope these efforts will stimulate more research into this debilitating disease and greater knowledge of it among the medical profession and the public at large.
Cahalan S. Brain on Fire: My Month of Madness. New York: Simon & Schuster; 2013.
Flanagan EP, McKeon A, Lennon VA, Boeve BF, Trenerry MR, Meng Tan K, et al.
Autoimmune dementia: clinical course and predictors of immunotherapy response.
Mayo Clin Proc. 2010;85(10):881-897.
Editorial. Mayo Clin Proc. October 2010;85 (10):878-880.
Graus F, Titulaer MJ, Balu R, Benseler S, Blen CG, Cellucci T, et al. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol. 2016;15(4):391-404.