After weeks of waiting and hoping and worrying, the time had finally come for my appointment with the mysterious expert neurologist. My parents and I arrived half an hour early to a spartan waiting room with an almost-eery quiet. A single tub of building blocks and several stuffed panda bears made me wonder if the staff really understood that they had allowed an appointment for an adult.
Forty-five minutes later, this concern was allayed when the doctor emerged to call me back without batting an eye when she saw my nineteen-year-old self. But all at once, I felt my stomach do a somersault as it occurred to me that she could be my last hope. Eight years of misdiagnoses had led here. Could this surprisingly soft-spoken woman finally be the one to help?
One of the top questions I get is: How did I get diagnosed?
You see, with PANS/PANDAS, there’s no definitive test or objective physical sign that can prove you do or don’t have it. This is what’s known as a clinical diagnosis. There are official diagnostic criteria that you have to meet to qualify for the diagnosis of PANS (of which PANDAS is a subset) according to PANDAS Physicians’ Network:
- An abrupt, acute, dramatic onset* of obsessive-compulsive disorder or severely restricted food intake
- Concurrent presence of additional neuropsychiatric symptoms with similarly severe and acute onset from at least 2 of the following categories:
· Emotional Lability and/or Depression
· Irritability, Aggression, and/or Severe Oppositional Behaviors
· Behavioral (Developmental) Regression
· Sudden Deterioration in School Performance
· Motor or Sensory Abnormalities
· Somatic Signs and Symptoms, including Sleep Disturbances, Enuresis, or Urinary Frequency
- Symptoms are not better explained by a known neurologic or medical disorder
- Age requirement – None
*There’s much talk of eliminating the abrupt-onset criterion since many of the experts are successfully treating people who didn’t have a sudden onset but otherwise meet criteria.
However, many doctors don’t realize such guidelines exist, and they misconstrue the lack of objective tests to mean that PANS and PANDAS aren’t real. It can be very hard to even find someone to evaluate you in the first place, and then if you do find someone who is open-minded, they still might only know the most narrow definition of the disorder and not recognize it in you. This is especially true if your symptom onset wasn’t overnight or if your most severe symptom isn’t an obvious case of OCD.
For me, it had become abundantly clear that something horrible was going on in my brain when I developed several neurological and psychiatric problems in the span of a few months. Even the most unhelpful doctors admitted that much, but said they didn’t feel comfortable evaluating me for PANS. And they especially hesitated since I was nineteen, and the “P” for “Pediatric” made the condition less likely in their minds.
The summer of my diagnosis, I couldn’t stay awake for more then ten minutes at a time no matter how much I slept at night. My legs started giving out randomly. Then I woke up one morning with violent involuntary movements all over my body. I forgot words all day long and would resort to pantomiming. A severe and often suicidal depression engulfed me and robbed me of the ability to do much of anything besides sit in a dark room by myself all day. I was dropping weight from my already-slender frame at a dangerous rate due to a sudden, irrational fear that almost any food would make me throw up. Worst of all, my personality changed as if all of the “me” had been sucked from my body.
Unable to accept what was happening to their kid, my parents pleaded with my family doctor to try an experiment:
give me five days of steroids.
At this point, I was so far gone and depressed that I didn’t want the steroids to work. I wanted to give up. I didn’t expect them to have any positive effect, just like everything else I’d tried recently—ranging from antidepressants to anticonvulsants to the exorbitantly-priced narcolepsy medication Xyrem.
Seeing how high the stakes were and that all of the standard medications thrown at me lately had backfired, my doctor agreed after reading some of the literature about steroid bursts for PANDAS.
For the first couple of days, I didn’t notice anything, either positive or negative. My involuntary movements, anxiety, and debilitating depression were as bad as ever.
But on the third day, the moment I woke up, I knew something beyond what I could have imagined had happened: I felt “normal.” I wanted to be alive. I could understand the words in my textbooks. The movements had quieted. I fell only twice compared to the usual hundred times throughout the day. I was able to stay awake. And above all, I just felt like myself for the first time in weeks, if not months.
Unfortunately, when you’ve been sick as long as I had been, it’s not uncommon for oral steroids to be a short-term solution. Many of my symptoms began to come back in the days after the burst, so my appointment with the out-of-state specialist three weeks later was timely.
My parents and I stepped into a small office with a big desk and crowded bookcases full of books on all manner of neurological diseases. No sooner had I plopped into one of the chairs that the doctor was already eyeing what I thought, by then, were subtle involuntary movements. She spent two hours interviewing me and my parents, but I’m convinced she knew it was PANDAS within minutes, or maybe even seconds. (Though certainly not everyone is as clear-cut of a case as I was.)
In the end, my diagnosis was made based on the symptoms, the response to steroids, the course of my illness, and the fact that it had all gone downhill after a confirmed case of Epstein-Barr Virus (mononucleosis).
Before my appointment, my family doctor had also ordered a test called the Cunningham Panel, which measures antibodies that are found in people with PANS/PANDAS. Obviously, it came out positive, so this was farther evidence of an autoimmune process at work, even though the standard autoimmune tests like ANA and sedimentation rate were normal.
My brain MRI had also been normal, but animal models suggest that the brain inflammation in PANS/PANDAS may happen at a microscopic level. Thus, the tests encephalitis doctors may run can come out negative and a person still has PANS/PANDAS. On the other hand, a positive Cunningham Panel (with which doctors may be less familiar) is still considered significant.
Finally, what obliterated all doubt that my condition was anything other than immune-mediated was my dramatic response to steroids. Over the following years, I had doctors who didn’t witness it claim it must have been placebo effect, as if somehow all cases of mental illness must have nothing to do with the rest of the body. But everyone who was there—including my psychiatrist—were confident the response was not psychological. Why? Because I didn’t expect or want it to work and it took a few days for it to take effect.
(Plus, there was a similar response to steroids years earlier, before I had even heard about PANS. I wondered for years why my depression, panic attacks, and cognitive issues had almost magically disappeared for a week or two after getting my wisdom teeth out. It turns out they had given me steroids, which I never would have guessed could help any of my longstanding issues. It would be impossible to attribute the improvement to placebo effect, so I’m sure the same thing happened again in 2014.)
Advice for anyone seeking a neuroimmune diagnosis:
In the end, it took me eight years and fifteen doctors before I was diagnosed with PANS. Granted, there was far less awareness in the 2000s than there is now, but my advice would be to be persistent. If your gut instinct is telling you that there’s more to your situation than straight-up psychiatric illness, keep digging. Just because one doctor dismisses your concerns doesn’t mean that the next one will, too.
Moreover, don’t assume you’re going to get the best care in an evaluation for PANS at the nearest research hospital (unless that hospital happens to be one of the handful with legitimate PANS clinics). Though there are undoubtably many fine doctors at these institutions, I’ve had the worst experiences at research hospitals. I believe this is because teaching hospitals tend to stick to the book, so to speak. If something hasn’t been completely proven, or wasn’t in the textbooks when the doctors in charge were in school, then they don’t want to consider it. There also can be egos involved.
In my experience, it seems that doctors who practice on their own tend to feel less obligated to stick to the status quo because there are no higher-ups looking over their shoulder. Obviously, no one is going to do something that they deem unethical or completely outside their scope of practice, but I’ve observed that private-practice doctors tend to be more open to exploring things with which they’re less familiar.
So bring all the research you can to your appointment
PANDAS Physician’s Network has a research library that you can send to your doctor, or you can print out some of those papers and bring them yourself. Highlight key points to save them time. Most doctors aren’t going to consider something like a neuroimmune condition without reading the research and the reasoning of why you suspect it. ASPIRE is another great resource with a concise list of suggested labs, lots of great videos explaining the condition, and another page where you can look for doctors near you.
Along these lines, it can also help if you make it clear that you don’t expect your family doctor to prescribe anything like IVIG or Rituxan. This is out of their scope of practice, but if they know you intend to see a specialist if need be, they might be more open to at least helping you run some basic tests and treating any infections you discover. For many people, antibiotics, oral steroids, or even addressing a food/environmental intolerance can be enough. Not everyone ends up needing years of treatment like I did.
Six years on, I’m in remission from PANS.
Last week, I spent my six-year diagnosis anniversary working intensely on a project for the job I have in my field. Anything related to PANS barely crossed my mind, as it doesn’t directly affect me the vast majority of the time. Yes, I have other health issues, but I barely have OCD, only rarely have a slight involuntary movement, and finally have stable moods. I worried so often along the way to recovery that I’d never be where I am today, but you just have to hang in there and keep trying and keep digging for answers.