Guess I had nothing better to ruminate about…
I’ve spent a maddening amount of time thinking about autoimmune encephalitis (AE). I’m not sure I’ve had another choice.
In the span of the past 2.5 years I got diagnosed; I got treated; I got back to baseline; I relapsed. After making a partial recovery, I relapsed once more.
Nine months out from the last time I felt the semblance of “cognitive normal,” there are now promising signs that count will be interrupted soon due to new help from cyclophosphamide (Cytoxan) and mycophenolic acid (Cellcept). I wish I could say that all I had to do was sit back, relax, and try not to stoke the fires of brain inflammation while my doctors did all the heavy lifting; but most of you already know that scenario is a pipe dream. Navigating a broken medical system with a rare illness is a nightmare. The truth is that I fought tooth and nail every step of the way to get to this point, buoyed by the invaluable support of friends, family, and the likes of you reading this.
The less textbook you are (hello late diagnosed, chronic, undertreated, relapsing, seizure-free, seronegative-ish and dementia-y), the less precedent there is, and the less equipped doctors are to help you on their own. So you’re left to solve your own puzzle lest you find yourself falling through the medical cracks. This realization, learned the hard way, has led me down a myriad of rabbit holes 100% necessary to unearthing every golden Pokémon of information I needed to connect the dots and
catch ’em all! arrive at the recovering state I’m in today. Nothing was spoon-fed.
It was either commit to making this my full-time job—well, as much as a sick, cognitively-impaired patient can do so—or say permanent goodbye to the functional parts of my brain.
Suffice it to say, I’ve been around the block enough to have become somewhat opinionated about the autoimmune encephalitis field: the treatment approaches, the research, the knowledge-to-action gaps. Some disagree with these opinions; others have used them as food for thought that helps them take meaningful next steps towards recovery. I’ve had the latter happen enough times for me to feel it’s worth it to start taking more of these opinions beyond the realm of private conversation—even if new information discovered later necessitates the need for me to eat my own words. We’re talking about an evolving field, after all.
What follows below (and in a future blog series?) is commentary on what I’ve noticed occurring in the AE world as a patient with an encephalitis-wearied set of eyes. ***Please take what I say with a grain of salt, do your own research, and talk to your own medical providers. I’m not an authority; I’m just a fellow AE survivor partially reflecting insights collected from others, and partially adding in a dash of my own spice.***
This is not geared towards those already under the care of a competent medical team, who are firmly planted on the road to recovery. In that case, I would probably suggest skipping past discussions like this as you don’t need more things on your plate to worry about. AE recovery even in the best of circumstances is an overwhelming undertaking. On the other hand, this IS geared towards those who are struggling, stuck, or otherwise dissatisfied with the handling of their AE-related medical care—the type of reader that this blog tends to attract.
The greater goal here is to help more AE patients maximize their potential for recovery. By voicing perspectives you won’t find in the echo chambers of formal healthcare, I hope to empower you towards becoming a more discerning and engaged participant in your conversations with medical providers.
Where is the autoimmune encephalitis field in 2022?
I am highly ambivalent about the state of the autoimmune encephalitis field in 2022. Sort of like the contrast between how I felt on World Encephalitis Day last year, and how I feel today.
Clearly, we have a long ways to go when AE support groups contain significant numbers of patients struggling through every step of the journey, from diagnosis through to long-term recovery. But despite the doom and gloom, I would say things are pretty hopeful for the average newbie placed on this journey.
There are more and more stories out there of people receiving early diagnosis and treatment, who ultimately come back from the depths of sickness to enjoy a reasonably good quality of life. Especially if you live in a large city, with enough searching you can generally find a neurologist, psychiatrist, or rheumatologist who has encountered AE and knows their way around the basic principles of treatment. Or at the least, any decent doctor with enough determination and resources should be able to locate the information they need to catch and treat AE.
Treat well? That’s another matter.
Terrible cases of medical errors, negligence, and gaslighting? Still happen.
But the equivalent of basic “doctor directions” are available now to literally anyone with an internet connection, with a growing volume of exciting journal articles published each year. The majority newly diagnosed and treated today are expected to make a good recovery.
I can recall the words autoimmune encephalitis entering my awareness about 5 years ago, while I had already been floundering for years without a concrete diagnosis. At that time, nothing I could find about AE made me think this was likely to be my diagnosis any more than the next label someone might throw my way. Nowadays, the illness is widely discussed as a heterogenous condition. Awareness and knowledge of AE and other related afflictions is rapidly expanding.
The more we discover, the more the artificial divide between neurology and psychiatry crumbles, and the more these specialties look towards the immune system and infections for clues towards unravelling the mysteries of brain illnesses. Research is trending towards all of this being more interconnected than what conventional medicine has traditionally accepted, validating what certain pockets of patients, caregivers, and scientists have already suspected for decades. (In separate but related news, there was a landmark study published last month for the first time providing compelling evidence of causality between Epstein-Barr virus and the pathogenesis of multiple sclerosis.)
So, through that lens, I would say that the AE field has come a long way in recent years. It doesn’t seem that way when you’ve been a patient beleaguered by this illness day in and day out, for years; but taking a step back to look at the broader picture, it’s clear that we are steadily making advancements that are resulting in improved outcomes for the average person impacted by AE.
It’s just not happening quick enough for some of us, right now: those further out into the illness, rudderless, refractory to first- or second-line treatments, whose case features deviate from widely reported norms.
Which…. seems to be quite a few of us.
But how much do we really know?
Several support groups and patient organizations list names of doctors with experience in treating AE patients. Some are big-name experts who have studied the illness for over a decade. To the unacquainted, you would naturally trust that you’re in good hands if you seek out the care of someone listed.
But in reality, it seems the jury is still out on how many people that strategy works for.
Don’t get me wrong—I think they’re a good place to start your search, and that it’s worthwhile to seek out a known “expert” rather than someone who hasn’t established a name in this field. This is especially true for textbook AE patients with a monophasic course, because there are conventional steps taken in this clinical scenario that are widely discussed in the literature. While there’s still some variation, you tend to see doctors choosing a handful of similar routes that lead to a similar outcome.
Beyond the acute phase—or in severe, refractory cases—is where consistency in approaches, opinions, treatments, and outcomes starts to collapse…. Sometimes, rather spectacularly. As a patient, being in this stage of the AE journey can feel like being in the wild west where safety OR disaster could be around the next corner. You’re either alone without a map; or with a sketchy map so fragmented you’ve instinctively grown a distrust for it. Because the last time you encountered disaster, it was concealed and uncharted.
The troubling trend that I and fellow patients have encountered is that some experts will forcefully give you that fragmented map—without proper warning that it is, indeed, fragmented. Instead, it tends to be presented as unquestionably accurate and representative of concrete fact.
When AE was brought to the fore in the late 2000s, there was such limited information and so many more unknowns that treating doctors—at least the ones convinced that AE was the correct diagnosis—had little choice but to humbly admit to their limitations, and execute clinical care under the guidance of one of a handful of pioneers in the field. These days, there’s enough diagnosed cases and AE research literature now for doctors with certain specialty backgrounds to feel confident about their understanding of the illness, and to develop strong convictions about exactly how it should be diagnosed and treated. I mean, no big surprise there—I guess I just described the job of the average doctor as they progress along in their career.
But the part that’s frequently disregarded is how we arrived at our current understanding of AE in the first place.
We didn’t do tidy experiments every step of the way, coming up with airtight data to prove every piece of information a doctor is going to tell a patient. Realistically that’s not feasible. Instead, we did some basic science to establish a patchy foundation for our understanding of AE (not at all to discredit the immense brainpower and ingenuity behind what happens in research centres around the globe); the rest is educated guesses, borrowing principles from what is seen in other diseases. Treatment decisions from early cases were retrospectively reviewed, clinicians repeated decisions that led to outcomes they were comfortable with, and now we are left with standard practices deemed “tried and true” which lay the groundwork for the reality patients are faced with today.
With the squabbles so many of us have with government-funded healthcare systems and insurance providers, it’s little secret that first- and second-Iine AE treatments lack the robust data behind them you might expect. We just know from experience they get the majority of AE patients back on their feet. That should start to change in the future: the very first prospective, international, randomized controlled trial for NMDAr Encephalitis just began recruitment. Just now, just this year, in 2022. (You can read more about the ExTINGUISH Trial and its investigations into inebilizumab (Uplizna), spearheaded by Dr. Stacey Clardy (University of Utah + Salt Lake City VA), Dr. Maarten Titulaer (Erasmus University Rotterdam), and Dr Gregory Day (Mayo Clinic Jacksonville), from the AE Alliance and Anti NMDA Receptor Encephalitis Foundation.)
Different experts, different outcomes
Evidently, the depth of our present AE knowledge is only the tip of the iceberg.
Some experts are wonderful humans who are transparent about admitting to the limitations of their/the field’s collective knowledge; others, not so much. The latter thrusts that fragmented map into your hands, looking at you with suspicion if you report something in the landscape they don’t see charted. Instead of “that’s curious; tell me more,” you’re met with “that doesn’t fit encephalitis; talk to your shrink.”
I know I’m not alone in being told troubling things by “AE experts,” some of them I’m sad to say are in leadership roles within the community. Here’s one of the more innocent-sounding ones: “We know brain inflammation can’t last more than 2 years. After that, it’s burnt out; any residual symptoms are sequelae.”
I spent a couple weeks believing this, before I stopped ignoring my gut and went on to find a different doctor who immediately recognized I was in a relapse and offered me further treatment—nevermind the fact that I had spent the first 5 years of my illness with untreated, active brain inflammation. It would have been disastrous if I took that expert’s words at face value; by the time I was set up to start receiving plasmapheresis under the next doctor, I was exhibiting signs of further decline and autonomic instability.
But how many people without the luxury of time, energy, emotional support, cognitive ability, and monetary resources, who cannot simply afford to run around on a wild goosechase from doctor to doctor, are left with permanently disabling consequences because of a single doctor letting their assumptions get in the way of making more appropriate decisions?
Sometimes I see people receive convincing but ultimately illogical medical advice, who trust it because it was said by an AE expert who’s supposed to be amongst the top in the world. I’ve been there. Sometimes the impact is fairly inconsequential; but other times there would be a significantly different outcome if this person were evaluated by a different doctor. I’m not here to judge which case is which.
But the truth no one wants to hear is that some patients are going to sustain life-altering results from seeing one doctor over another, expert or otherwise. Really, I suspect that occurs less frequently in the overall AE patient population than what I’m biased to notice. But I’ve experienced firsthand and know from fellow patients that doctors have varying interpretations of the AE literature, and that there are some major differences in how individual providers translate medical knowledge to application. Even within the same institution.
Side point: sometimes, it’s that local no-name neurologist (or psychiatrist!) that gets you the furthest. The curious, open-minded one who readily admits to the perimeters of their knowledge, but still chooses to work together with you to chart the contours of your unique neuropsychiatric map. I would hold on to those ones, as they’re a rare breed whose help you may need in the future. Don’t put your eggs in one basket if you can help it. As I’ve recently encountered again, doctors can move around, change their office policies, or go on maternity leave—seemingly on a dime. Then continuity of care becomes dicey, given aforementioned differences in approach between providers.
You have answers, we have… questions?
You can find a lot of videos about AE (see: YouTube) from various medical personnel including neurologists, rheumatologists, psychiatrists, neuropsychiatrists, and rehabilitation therapists. There’s a lot of information out there. In addition, there are various conferences, webinars, virtual rounds, and meetings you’ll find—often free to the public—throughout a given year.
See enough of them, however, and you’ll notice the conversation gets a little stale. The majority of content continuing to make it to these venues is the same recycled, surface level information that informed patients/caregivers have already heard before. There’s a place for that, sure; but I think there’s a paucity of public-facing, thought-provoking discussions that could actually advance the field further.
Or maybe I’m just bitter I don’t have a window into those conversations, with seemingly little cross-talk occurring between patients/caregivers and scientists 🤔.
Moving forward, I hope to see more experts moving beyond the textbook to discuss other topics such as atypical cases, novel treatment approaches, cutting-edge research, and controversial ideas. Maybe we’ll get some of that down the pipeline, with some promising work coming from groups around the globe such as the German Network for Research on Autoimmune Encephalitis and the Australian Autoimmune Encephalitis Consortium Project.
To round out today’s post, I’m leaving you with some questions I’ve been pondering. Spend time with them and you’ll increasingly start to identify—if you haven’t already—what assumptions your doctors are operating under, how messy the encephalitis field is, and how much more we have left to discover.
I might be naively optimistic, but it’s my hope that I can look back at this post on World Encephalitis Day 2023, and see that the field has made progress towards answering some of them:
- Do the most widely-accepted diagnostic criteria (Graus et al. 2016) capture all patients who have active brain inflammation? Are there patients who would significantly benefit from immunotherapy who do not meet the criteria for AE as defined in this seminal work?
- How sensitive are our current diagnostic tools— standard CSF testing EEGs, MRIs, PET scans—for capturing brain inflammation? Do these tests miss chronic, low grade inflammation?
- Is it possible that symptoms considered by the average specialist to be “sequelae” could actually be chronic inflammation that would warrant additional immunosuppression?
- It is widely accepted that long-term recovery occurs gradually over a period of 2 years (or longer). Where did that timeframe come from? Is that timeframe simply an observation of what occurs given our current treatment protocols, rather than an ideal we should be targeting? Could this timeline be compressed, resulting in faster recoveries, if more aggressive treatment approaches were adopted?
- If so many survivors have ongoing levels of significant dysfunction, might there be a problem in the initial treatment approach that the field has currently embraced? Are we undertreating the average patient, stifling them from achieving a more complete recovery?
- When considering treatment escalation, how are specialists calibrating risk vs. benefit? Might we be overweighting the mitigation of physician liability over what is truly best for the average patient and their quality of life?
- How are we assessing what is best for the patient? Should patient/caregiver values be incorporated into decision-making? How much choice should patients/caregivers be given when treatment decisions are being made?
- What are the pros/cons of “undertreatment”? What about “overtreatment”?
- Has the average specialist carefully considered what patient outcome they’re aiming for, tailoring their treatment decisions accordingly? Or are they administering a routine protocol that matches their subjective comfort level, leaving the patient/caregiver to accept whatever low OR high level of residual issues remain?
- The current body of research literature is largely a characterization of what occurs in textbook AE patients. Should non-textbook patients e.g. ones diagnosed years late be judged against this body of literature? Should we assume they require the same intensity and duration of treatments? Do these patients have worse outcomes, on average, due to treating them the same way we treat textbook patients who received quicker access to treatment?
Annnnnd that’s it for me this World Encephalitis Day 2022! If you have a comment or want more posts like this, let me know via Facebook, Instagram, Twitter. Knowing my writing helped someone gives me motivation to actually sit down and translate my thoughts into words (which is a strugglebus these days).
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You may be interested in taking a look at the following next:
The AE Awareness Month Podcast Series
What happens when your autoimmune encephalitis treatments aren’t working?
The Real-World Challenges of Surviving an Autoimmune Encephalitis Diagnosis
The 7 Ways To Piss Off Your Neurologist, As Told By An Encephalitis Survivor
Excellent questions! I have many of the same and haven’t gotten answers to them. Thank you so much for sharing your experience, it is so important to so many. It is a frustrating journey for sure, even when you get a diagnosis. I had viral encephalitis in 2017 and recovered quite well for 2 years, then onslaught of 101 new neuro symptoms. I’m in canada, no neurologists would see me. Eventually i parked myself in the ER with stroke symptoms and refused to leave. It still took a few neurologists to sort things out and many refused imaging! Finally a new diagnosis to add to the list, which is syringohydromyelia. A syrinx in thoracic spine, which develops secondary to CSF flow restriction (infection, injury, congenital issues). I believe mine secondary to the encephalitis, but to find a specialist who knows much about both is not possible in our system. My dr’s just say ‘i don’t know’, and of course there is no treatment. I have connected with some great practitioners to add to my team and they have helped me recover. Naturopathic Doctor, counsellor, craniosacral therapist, acupuncturist. I’m doing quite well now except for little flares every 3ish weeks. If you come across any information on syrinx after encephalitis, please let me know! I have found very little. Big love, healing and strength from me!!
Hi Jese! Guh, it’s terrible to know that you’ve been going through all that and yet unfortunately it’s also not surprising. Especially in Canada… I know it’s so so hard to get second opinions from specialists, not to mention in a timely manner. “Syringohydromyelia” is a new one to me but it’s now on my radar and if I notice anything on it I’ll be in touch! Good to hear you kept pushing until you got great practitioners on your team and that you’re doing better now, but also hoping you reach full recovery ahead sending hugs and healing your way!