This disease is a sly one . . . Let me take you through the short version of my long and frustrating journey to a proper diagnosis.
When I first developed psychiatric symptoms in the fall of 2020, I sought the help of a psychiatrist. Among other symptoms, I was experiencing brain fog, low-energy depression, and extreme mood swings that affected my day-to-day function. Following their advice, I switched antidepressants, which I had already been on for two years, and introduced a mood-stabilizer (which doubles as a seizure medication) called Lamotrigine. We played with the dosage of these medications until my symptoms were more or less managed, but I still didn't feel like my old self. Our best guess was that I had developed Bipolar II disorder, which is characterized by depressive and hypomanic episodes. My presentation was atypical, however, with swings occurring almost daily rather than monthly or seasonally. As time went by and I developed neurological and physical symptoms, this diagnosis was ruled out.
When my first seizure-like episode occurred in the fall of 2021, I was taken to an emergency room. One minute I was limp and unresponsive, the next I was crying and talking like a child. The ER staff did an electrocardiogram (EKG) and a head CT, both of which came back normal. They hadn’t found any answers, but once they heard that I was potentially bipolar, I was no longer taken as seriously. No further testing was done, and I was forced to wait until the episode resolved itself about two hours later.
Weeks later, an outpatient electroencephalogram (EEG) came back normal. Epilepsy was ruled out. A neurologist referred me to a GI doctor because my episode had started with abdominal pain, but stool samples and an abdominal X-ray both came back normal. They diagnosed me with suspected celiac disease. I declined an endoscopy for confirmation because I had lived gluten-free for many years on account of pain and bloating. In the end, nothing new had been found.
Months later, I developed white nipple discharge in both breasts. My gynecologist said this can be related to hypothyroidism, which I have a history of, so we tested my thyroid stimulating hormone (TSH). It was at the low end of normal at 0.47. Since my thyroid seemed healthy and my breast exam was negative, the galactorrhea was deemed a rare side effect of one of my medications.
Four months after that, I had my second seizure-like episode and found myself in the emergency room again. I was given two heavy sedatives (admittedly too much) for a suspected seizure while my blood work was taken. My TSH was 140 (extremely high) and my thyroid hormone (T4) was 0.6 (low). After the doctors had a look at my medical records though (suspected bipolar, remember?), no further testing was done. I was discharged practically unconscious and woke up in my bed six hours later, unsure of how I had gotten there. My discharge paperwork said my diagnoses were — I quote — "Mental health problem and underactive thyroid." 125 mcg of Synthroid were ordered to my pharmacy. While we waited on this, I started taking 150 mcg (doubling my current 75 mcg pills) at the suggestion of my dad, a family physician.
Sometime between my OBGYN visit and this trip to the ER, my thyroid health had drastically declined.
Two days later, I had another seizure-like episode while surrounded by fellow nurses and first responders who got me on the ground and called for an ambulance. I told them I wanted to avoid the previous ER where I had been treated poorly, so I was taken to a different hospital and admitted for further testing. Labs may differ from hospital to hospital, but at this time my TSH was 75.28 and my T4 was 0.94. Evidently, the two days of 150 mcg had helped. This hospital had no inpatient endocrinologist, so I was referred to a nurse practitioner (NP) who would see me a week later. While admitted, a neurologist came to see me and said these episodes looked like simple partial seizures. This time, I was put on a continuous EEG overnight to further evaluate the potential of epilepsy.
I had multiple episodes while hooked up to the monitor. During the episodes, I exhibited involuntary facial movements which resembled tardive dyskinesia. I also developed an almost constant head tremor and struggled to talk without focusing on one object. When I did get a word out, it was slurred.
Though this was all recorded, no abnormal brain activity was found. Furthermore, Keppra (a common medication given for epilepsy) was not effective. An MRI, taken both with and without contrast, and CT scans both came back normal, showing no masses, blood clots, or brain bleeds. From these results, the neurologist concluded that my symptoms were not neurological in origin and suggested they might be stress related. He even proposed that the stress from the past four years of nursing school had caught up with me — after I had graduated, passed my NCLEX, and had a job waiting for me. My family and I did not agree with this statement. He called my symptoms psychogenic non-epileptic seizures (PNES) and suggested following up with my psychiatrist and a neurologist.
Before discharge, I decided to go for a walk in the hall. For the first time, I had trouble putting one leg in front of the other. The jerks affected my entire body.
I found out later that a different neurologist discussed my case in the hall with my dad. He said he had heard of a disease called Hashimoto's Encephalitis, but that it was rare and he doubted it was the case here.
A week later, the outpatient endocrinologist NP immediately diagnosed me with a myxedema crisis (extreme hypothyroidism). She said that if I had been given any more sedatives that day in the ER, I ran the risk of going into a coma, which could have become fatal as organ functions were impaired. She was also certain that my presentation indicated Hashimoto's thyroiditis, an autoimmune disease where the body attacks its own thyroid. She tested for thyroid antibodies in my blood and found my thyroid peroxidase antibodies (TPO AB) were >900. Standard range is <8. My thyroglobulin antibodies were 16.1. Standard range is <4.
Hashimoto's thyroiditis calls for very precise maintenance with synthetic thyroid hormone replacement, but the NP thought that my Synthroid's potency may have decreased due to heat inactivation of the pills and taking my medication too close to breakfast. She also concluded that taking greater than 100 mcg at my weight would put me at risk for factitious hyperthyroidism, which comes with its own problems, and dropped my dose back down to 75 mcg, taken properly. She said she had seen similar neurologic symptoms accompany myxedema before and suspected all my related symptoms would go away if I followed the strict medication protocols (though she recommended seeing a neurologist nonetheless). A thyroid ultrasound was also ordered as my thyroid had visibly enlarged, developing into a goiter. This confirmed thyroiditis (inflammation of the thyroid), but no nodules or tumors were found.
A neurologist NP examined me a week later. At this point, I was still able to clench my hands, track fingers waved in front of my eyes, and walk heel to toe in a straight line . . . but none of these would last. My main neurological symptoms were non-epileptic seizures, which included asymmetrical facial movements, and a consistent head tremor. I sometimes had trouble talking and walking, but this was intermittent at the time and the NP did not witness it. She concluded I had late-onset tic disorder or Tourette's Syndrome — even though I was twenty-two years old and had no related history. My family and I did not agree with this diagnosis, either. Since there was nothing obvious in my brain scans, she recommended following up with my endocrinologist. She did get blood work done to check my Lamotrigine levels, which were in range, but when we asked for a lumbar puncture for further testing it was denied.
I was taken to the emergency room once again after experiencing an episode that lasted much longer than normal. My dad requested a phone call with the ER doctor. He wanted me to be admitted or get a neurological consult with a spinal tap and autoimmune blood panel. The doctor refused. I was discharged during the episode with my mouth stuck to the side, slurred speech, and a complete inability to walk. My boyfriend had to request a wheelchair for me.
Bipolar. Celiac disease. Stress. Tic disorder. All of these diagnoses had amounted to nothing. I felt absolutely crazy. I tried to shake myself out of it and convince myself nothing was wrong, but as I pushed myself forward, I only got worse . . .
It’s important to mention here that I come from a medical family. My dad is a family physician; my sister is a nurse practitioner; my other sister and her fiancé are dermatologists. My dad had discussed Hashimoto's Encephalitis, briefly mentioned to him in the hospital, with my siblings. The first time I heard the term was when my brother started a group message ("Shae Support Group") and sent a link to a research article on Autoimmune Encephalitis. "I think this should be our number one diagnosis right now. I would really look into specialists around the country who see this . . . Hang in there Shae. We're gonna figure this out." He sent a link to the Autoimmune Encephalitis Alliance’s web page, where I found a neuroimmunologist specialist near me. My dad made a phone call explaining my situation and made an appointment for me six weeks out. They were surprised to hear that no one had done a lumbar puncture/spinal tap or autoimmune blood panel yet.
While searching around for more information on this new diagnosis, I came across a nonprofit organization called Hashimoto's Encephalopathy/SREAT & Seronegative Autoimmune Encephalitis Alliance (HESA). Their website was a great resource for research articles, potential symptoms, obstacles to diagnosis, treatment options, lists of recommended physicians, and the basics of the disease. The physician we made an appointment with was on one of their lists. I also found their Facebook page, where I was able to directly communicate with people who were experiencing what I was experiencing, and their YouTube channel. As I heard their stories, my heart dropped; I remember thinking, "That's me." Though the stories were heartbreaking, I learned there were treatment options out there and therefore hope.
My dad suggested I see an endocrinologist MD in the meantime. This doctor raised my Synthroid back to 100 mcg, saying that 75 mcg was not enough to address my thyroid levels. We mentioned Hashimoto's Encephalitis and asked what he knew about it. He said this was a controversial diagnosis and that some didn't even believe it was real. However, he was open-minded enough to consider the research and willing to work with an encephalitis specialist to diagnose or rule out HE. First he tested me for Lupus, which came back negative. He then sent my blood to the Mayo Clinic to run an ENS2 panel (Encephalopathy, Autoimmune/Paraneoplastic Evaluation, Serum). No specific antibodies attacking the brain were found in my blood.
The long-awaited appointment with the neuroimmunologist finally arrived. After six weeks of waiting, my neuro function had declined significantly and I was having extreme difficulty talking and walking. The neuroimmunologist described my language as a combination of Chinese and German — a phenomenon formally known as foreign accent syndrome. He also witnessed a seizure-like episode during my exam. I showed brisk reflexes and a positive Hoffman's sign, which suggested the presence of an upper motor neuron lesion and corticospinal tract dysfunction caused by inflammation in the cervical spine. He confirmed that Hashimoto's Encephalitis is controversial because it is not well understood; however, he had done extensive research on the subject and had even diagnosed a few patients with it in the past. His plan was to finish the full workup to rule out any other causes of encephalitis like other disease processes, infection, or specific antibodies directly attacking the brain. Once all other diagnoses were ruled out, he would attempt a steroid trial.
We were so happy to be taken seriously and given the thorough workup we wanted!
My blood work showed elevated IgM, which is associated with certain autoimmune diseases, and an elevated Sedimentation Rate, indicative of high levels of inflammation. My lumbar puncture was negative for infection or specific antibodies attacking the brain, meaning I was negative for seropositive encephalitis. With this information, other causes of the suspected brain inflammation had been ruled out.
After the full workup was done, we knew these things to be true: my thyroid antibodies were high, my body was in a heightened state of inflammation, and my neurological function was steadily declining. To confirm the suspected Hashimoto's Encephalitis, all we needed to do was see if I was steroid responsive. Simple enough, right?
The first-line treatment for Hashimoto's Encephalitis is a high dose of anti-inflammatory corticosteroids. The idea is to decrease brain inflammation as quickly as possible. If the patient responds to steroids with a relief of neurological symptoms, this confirms there was active inflammation in the brain. Oddly enough, the treatment protocol for the disease is also the way to diagnose it.
I was prescribed one gram (1,000 mg) of Methylprednisolone, also known as Solu-Medrol, each day for five days. This was to be administered via an Easypump. After a peripheral IV was placed at an infusion clinic, I was sent home with the medication.
"This dose of steroids will either kill you or save you," my dad said. I only made it four out of the five days of treatment before I was admitted to the ICU.
My body began to shut down. My blood pressure hovered around 80/50, my pulse at 50. All my neurological symptoms were heightened to an unbearable degree. For three days, I was bedridden and unable to say a single word. Not one single word.
They increased my Lamotrigine by three times the normal dose to control my non-epileptic seizures. The doctors ran tests that had already been done at previous hospitals, checking to make sure nothing had been missed. Another lumbar puncture and MRI were performed — both negative. I was placed on an EEG again for multiple days. This time they found brain irritability, or small, sharp spikes, on the reading. In spite of this, the neurologist said my symptoms were not neurological in origin and suggested I be evaluated by a psychiatrist for conversion disorder, suggesting my symptoms could be attributed to past trauma.
EEG reports, later released: "This is an abnormal video EEG study. Continuous EEG recordings showed slow wave activity in the temporal electrodes in both hemispheres, at times appearing more prominent on the left, consistent with focal cerebral dysfunction in the temporal regions, which may be more prominent on the left. There were rare sharply contoured slow waves seen in the left temporal electrodes, raising the possibility of potential epileptogenicity in the left temporal region." "Continuous EEG recordings showed focal slow-wave activity and epileptiform discharges independently in the temporal electrodes in both hemispheres, consistent with potential epileptogenicity and focal cerebral dysfunction in the temporal regions."
It seemed that I was not responding to the steroids, and we began to fear we were back at square one. Unconvinced that my disease progression indicated a psychological issue, we were getting nervous that I would never receive adequate treatment.
Everything changed on the evening of the third day of admission, or the seventh day from the start of the steroid treatment. Not only was I finally able to form words, but I heard my voice without a foreign accent. My blood pressure shot up to 157/94 and my pulse to 120.
I cried. We all cried. The steroids had kicked in. I was steroid-responsive.
I saw the inpatient psychiatrist the next day. She said that my psychological and neurological symptoms seemed to be caused by an identifiable source: my thyroid. Because I had responded to the steroids, she did not think this was conversion disorder or that I needed cognitive behavioral therapy. This was so validating.
Two days later, I walked out of the hospital with a cane and a new perspective on life. I was seizure-free for four weeks.
A year and many misdiagnoses later, I was officially diagnosed with steroid-responsive encephalitis associated with autoimmune thyroiditis (SREAT). The short definition of this condition is inflammation of the brain caused by the body mistakenly attacking itself — in this case, inflammation of the thyroid and brain. It goes by many names, including nonvasculitic autoimmune inflammatory meningoencephalitis (NAIM) and seronegative autoimmune encephalitis, which emphasizes a lack of detectable antibodies in the spinal fluid or blood. Most commonly, you will hear me refer to it as Hashimoto's Encephalitis.
Though it was a long and painful journey just to find a label, I consider myself one of the lucky ones. Many people go years without a diagnosis and develop life-long conditions like short-term amnesia due to lack of treatment. Others find themselves stuck in psych wards or rotating in and out of emergency rooms, their treatments failing and no light visible at the end of the tunnel. If sharing my story helps just one person get diagnosed sooner, it will have been worth the pain of reliving it.
Stay tuned for further posts about my treatment and ongoing care.