Leah Ehle’s autoimmune encephalitis survival story

The Ehle Family

My name is Leah Ehle. I am married and have two Pugs.  I live in Connecticut and work as a Nurse Practitioner in the field of gastroenterology.  I have worked in the medical field for over 10 years, first in oncology and then after my masters I went into gastroenterology. I have always prided myself on my memory, both when I was in school and when working.   Outside of work, I enjoy hiking and rock climbing and have always been an active person.

June 2017: I had never had a serious illness in the past, but everything changed for me this last summer.  The start of the issues began in early June when my husband and I were adding a new puppy to our family.  Millie, our new pug puppy, came to us just after she turned 8 weeks old and joined our older pug Oswald, who had already been a part of our family for 11 years. After Millie’s arrival, I started to feel off at times and had several odd episodes.  I became very anxious and restless, but I blamed this on the stress of having a very energetic young puppy.  Shortly after, I started to gag every time I brushed my teeth.  This worsened to the point where I vomited on my bathroom floor while trying to put the toothbrush in my mouth. I knew this was odd but I just blamed it on stress. I just continued to become more anxious after this event and just did not feel like myself.

July 4th: I worked on July 4th despite not feeling like myself, but continued to rationalize that it was all stress related and fought through the day.  I could barely eat at lunch, but forced myself to do it anyway.  That night my husband wanted to take me out for sushi, because I liked the restaurant and it was all that we expected to be open at that time of day on July 4th.  I did not feel like eating, but I went anyway because we both thought it would be better than me stewing at home and maybe I would enjoy myself.   Unfortunately, I once again had to force myself to eat, with each bite getting harder and harder to stomach. I began to feel sick at the restaurant as we were finished our meal and ran to the restroom to vomit.  It was so strange because I didn’t feel nauseated but I couldn’t stop vomiting.  In addition, I felt very confused.  These symptoms continued through the night until about 3am when I finally stopped vomiting and fell asleep.

July 5th: I was scheduled to work on July 5th and, even after being sick all night, I got up when my alarm went off.   I was getting ready to take the dogs out and called my husband, as he was already at work.  I was confused on the phone and I was not sure what day it was and I didn’t know I had worked the day before.   Basically, I couldn’t remember what I should be doing. We both thought this was odd, but blamed it on dehydration and I decided to go to work anyway.  I really realized just how confused I was, when I arrived at the hospital for work.  I attempted to interact with several people, but I couldn’t understand what they were saying to me or even begin to answer their questions. I quickly realized that I couldn’t stay at work since I couldn’t communicate. I went home and began to get sick again throughout the day. I finally stopped vomiting in the evening and felt a little less confused, but not totally back to normal.

Mid-July: I went back to work the next day, but the confusion never really went away. For the next week, I continued to feel slightly confused and very anxious. It was very hard to put a finger on what was going on, but I just continued to try to get through the days.  I woke up with pressure above my left eye and felt increasingly confused at the beginning of the next week. I was even having trouble remembering names that were very familiar to me.   I would lose track of my thoughts and would have to stop talking while I was seeing patients or talking to other providers. I would also say things that I realized didn’t make sense.   I had similar challenges when recording the details of my interactions with patients in their charts.  I would sit at the keyboard for long periods not being able to form sentences.

I also started to have difficulty understanding what people were saying to me. I began to hear music that wasn’t playing and had other auditory hallucinations frequently. I would even think the people speaking on the radio were saying exactly what I was thinking.  I was aware that all these experiences were not happening as I was experiencing them and that caused increasing fear and anxiety.  Several times I burst out laughing when I was by myself for no sensible reason.

I was also having similar comprehension issue when I was reading. I would think the text said exactly what I was thinking and then would re-read it and realize it had nothing to do with what I was thinking. This continued to worsen as the week went on. I got to the point where I couldn’t remember the names of people I have worked with for several years, my dogs’ names, and even my husband’s name. I was trying to tell my family and my husband something was wrong, but it was difficult for them to understand what was happening.

First ER Visit: I became so worried that on Thursday night after work I went to the small-town ER where I was working that day. I knew this wasn’t the best place to go, but I could get a CT scan of my head and labs done. The CT scan did not show anything odd and the lab work was normal except my white blood cell count, which was a little high. The providers felt the most likely driver of my symptoms was anxiety and said if my confusion were to worsen that I should get an MRI of my brain. They recommended that I treat my anxiety. I was a little relieved, but still knew that things weren’t right.

The next day I went to work again, and while nothing had really improved I wasn’t worsening either.  That night I went to a wake, at first I felt ok, but the more I was talking to people the more I had trouble remembering certain words and keeping my conversations normal. I said some weird things that night that my co-workers are still laughing about. I continued to feel increasingly worse through the day.

July 22nd: My husband and I decided to do a strenuous hike to try to get my mind off of the problems I was having.  I was really exerting myself on the hike and I stopped hallucinating and I was actually able to think clearly. It was amazing to feel like myself again for that small time. I stopped exerting myself while going back down the trail and the hallucinations returned and the ability to think clearly receded.  I was crushed.  I got to the point that night where I was so afraid and anxious that I sought a prescription for Zoloft.   I was able to get the anxiety medicine and took it on Saturday night hoping it would help.

July 23rd: I planned to go on another hike on Sunday hoping this would let me think clearly again. Unfortunately, when out on the hike on Sunday I did not have the same reaction as the day before. I continued to feel more confused, anxious, and had more hallucinations as the day went on. I called my parents crying, because I couldn’t figure out what was happening. I couldn’t even make myself go in the grocery store and waited on my husband to complete the shopping, because of my mental state.  We stopped at Subway to get some food and the worker asked me what I wanted on my sandwich and I couldn’t answer.  My husband had to finish my order for me.  I kept telling my husband that I couldn’t go to work the next day like this.  He kept trying to encourage me, thinking this was anxiety related, and that it would be ok.  He even bought me a small notepad to record my interactions.  I knew there was no way I could keep track of what patients were saying to me or remember what to ask.

Second ER Visit: I continued to worsen through that evening. I began to have rigors, feeling cold and physically shaking, around 5 or 6pm. I told my husband that I needed to go back to the ER.  He thought first we should take my temperature to make sure I didn’t have a fever. We decided to go to the drug store to get a thermometer, as we could not find ours.

I began to have a seizure, while on the way, and my husband finally agreed that I needed to go to the ER.  The left side of my face began to droop and I was laboring to breathe.  We arrived at the large city hospital (Hartford Hospital), where I also work, after some very quick driving.   I was no longer having a seizure, when we arrived at the ER, but I couldn’t talk and had to have my husband give my medical history when we went through intake.

I had an EKG while in the ER.  They also did some labs and I was found to have a low sodium level.  They began to push fluids, which did seem to stabilize me a little bit as I began to regain my ability to speak.  The ER physicians could tell my cognitive function was not normal, particularly since I knew many of these people since they were my colleagues.  There was a brief disagreement between the ER physicians, who wanted to do a spinal tap to check for an infection while the neurologists wanted to do an MRI first.  Ultimately the neurologists won the argument and made the move to admit me to the hospital.

Hospital Admission: I went for an MRI of my brain late that night (around 3 AM) and was awoken at 5 AM with the news that I had temporal lobe encephalitis.  The hospital set out several goals for my treatment on Monday.  I would need a lumbar puncture (spinal tap), EEG (measures brain activity), and a CT scan of my chest, abdomen and pelvis to rule out a cancer causing this brain swelling.  I give tremendous credit to the hospital (nurses, techs and doctors) for getting so much testing done in less than 24 hours.

This time remains a blur to me, because I was still barely able to talk or comprehend what people were saying to me.  My tests were all coming back negative for an infection, which was good because it ruled infection out as the driver of the brain swelling.  However, the neurologist was now unsure if it was a brain tumor or autoimmune disease causing the swelling, which was very scary to me.

Neuro-Cognitive Testing: His initial plan was to start me on steroids while we waited for tests that could answer about cancer or autoimmune disease, but he wanted neuro-cognitive testing done first.  The goal of the testing was to determine a baseline for my brain function. I think the neuro-cognitive testing was the hardest part emotionally of this whole experience. I couldn’t remember words, I couldn’t do the simplest of tasks and it was extremely fatiguing. I could barely remember who the President was and I couldn’t remember the Vice President. The simplest things to remember had just evaporated from my mind. That experience made me realize just poorly my brain was functioning and it shook me.   I guess up to this point I had rationalized everything that happened and this forced me to look in the mirror.

Initial Treatment:  I started my first large dose of IV steroids (1gm of solumedrol to be exact) at about 12 PM that day.  It was amazing the difference the steroids made.   I was a different person only about 2-3 hours after the receiving the IV steroids. I was starting to think normally, remember names and be able to converse with people. My mother arrived at the hospital around this time.   She lives in Indiana and flew out to Connecticut as soon as she could when she found out I was sick. She wasn’t expecting the person she saw. I was able to talk to her and have a relatively normal conversation, considering the situation.

My mental status was so much better and my sodium improved significantly by the next day. It was determined that I could go home and finish my IV steroids at home. I received that treatment for a total of 5 days for what was assumed to be autoimmune encephalitis.  There was still the worry that I could have a tumor being masked by inflammation, but we had to wait for an official diagnosis.

Late July and August: This began the long journey of living with the aftermath of this episode. I continued to be slightly confused after finishing the IV steroids and to have occasional hallucinations.   I started high dose oral steroids following the IV steroids.   These steroids made me very emotional and the dose was so big that it significantly affected my heart rate and my ability to sleep.  I was in constant communication with my neurologist who was working hard with the Mayo Clinic to determine if I had an autoimmune disease.   The confusion and hallucinations finally subsided about a week and a half after treatment began. During this time I was very fortunate to have my mother staying with me, I wasn’t well enough to be alone yet and wasn’t able to drive. This allowed my husband to return to work and not worry about me being alone.

Diagnosis: I received the diagnosis after 16 days of worrying whether or not this episode was from a brain tumor or autoimmune disease.  It was determined that I had autoimmune encephalitis secondary to the LGI1 antibody.  This diagnosis was found by Mayo using the cerebral spinal fluid (CSF) that was obtained from the spinal tap that I had while in the hospital.

It felt amazing to have a diagnosis.  I was relieved that this condition was rarely related to cancer.  However, the journal articles I read about this condition were not particularly comforting either.  They described an associated degree of ongoing cognitive dysfunction after having temporal lobe encephalitis from this particular antibody.  This was stressful for me because of the pride I have always put in my memory.

Ongoing: I was put on cell-cept along with continued steroid treatment, which is currently being tapered.   My neurologist has consulted with several experts in the field on treatment.   I am about 4 months out from being admitted to the hospital. I feel like I’m improving all the time and I am now back to work. I still live with constant fear though that this disease is going to return and possibly damage my brain. I get anxious whenever there is a weird noise that the hallucinations have returned. I am currently in therapy to learn how to cope with this extremely scary experience and how to live with the aftermath.

I’m extremely grateful for the rapid diagnosis and treatment.  The providers, nurses and techs took me seriously and it was their fortitude that enabled the diagnosis.  Even only a decade ago, I may have been put on psychiatric treatments rather than having the correct condition treated. I am very grateful for the effort being put into increasing the understanding of this horrifying disease.  We need to continue to study how to best treat it and prevent relapse. I hope my story can help others and provide more data to better understand this disease.

Thanks: I wanted to thank you to the many people who helped get me through this difficult and trying period of my life. I wanted to thank my mother who stayed with me for 6 weeks.  I couldn’t have gotten through this without her. I am very appreciative for my father managing things at home alone and sacrificing his own time with my mother so she could be with me. I wanted to thank my husband for the patience and love he gave me through this time period. He sat through and continues to sit through many tears, irrational fears and thoughts. He has shown patience that I don’t think many people are capable of having. I wanted to thank my friends, family, and co-workers for the many cards, gifts, flowers, encouraging words I received during this time. I also wanted to thank my co-workers for covering for me at work, they never made me feel rushed to return or that I caused them any hardship. I couldn’t have done this without the amazing support system that I am so lucky to have.

Post written by Leah Ehle with correspondence and editing by Lynn Chapman.