I’ve got Adie’s tonic pupil, also known as Adie’s syndrome, Holmes-Adie syndrome, or Adie’s pupil … again. A hallmark characteristic of Adie’s is a pupil that is dilated larger than normal. The good news is that Adie’s, in the grand scheme, is not a big deal. Going through it can be a bit scary though, and consulting the internets about one pupil being bigger than the other with blurry vision yields things like aneurism and stroke. No mention of Adie’s. So I’m writing this post to add a patient’s perspective on Adie’s in hopes that those that need to find it do.

The story begins…

It’s circa 2005, I’m in my mid-20s living in a cute little two story ginger bread looking house in Saint Louis. I’m washing my face before bed and as I look in the mirror to make sure I got all the mascara off. I see my left pupil is much larger than my right and I begin to low key freak out. I don’t recall any vision changes at the time, but I remember being completely unnerved at the sight of my mismatched eyes and being anxious to get in to see an ophthalmologist.

I spent nearly a full day at the office of a neuro-opthalmologist affiliated with Washington University. I wish I remembered his name. After a myriad of questions (have you used dramamine patch recently? fun fact: women used to put dramamine in their eyes to make them dilate and look sexxxy… one eye dilated is kinda creepy), and lots of tests, I have a diagnosis: Adie’s Tonic Pupil. I also learned that folks with Adie’s may have some weird symptoms nowhere near their eyes like sluggish or asymmetric deep tendon reflexes when tendon’s are tapped with a reflex hammer, and abnormal excessive sweating (hyperhidrosis)… awesome.

So what exactly is Adie’s Tonic Pupil?!

Adie’s Tonic Pupil is what happens when there is damage to the parasympathetic nerves that control the pupil’s size. The parasympathetic nervous system is one of two main divisions of the autonomic nervous system, which is responsible for regulating involuntary bodily functions like digestion, heart rate, respiratory rate, and apparently pupil constriction. The other division of the autonomic nervous system is the sympathetic nervous system.

Parasympathetic nerves innervate the muscles of the iris and cause the pupil to constrict (miosis) in response to light or during near vision tasks. Adie’s is generally considered a disorder of the parasympathetic nervous system, particularly affecting the parasympathetic innervation of the iris muscles.

The primary nerve involved in Adie’s is the postganglionic parasympathetic fibers of the ciliary ganglion. These fibers originate from the Edinger-Westphal nucleus in the midbrain, travel via the oculomotor nerve (cranial nerve III), and synapse at the ciliary ganglion. From there, they innervate the sphincter pupillae muscle of the iris, which is responsible for pupil constriction (miosis). I won’t pretend to know what all that means, but here’s a picture that @aaronrutman posted on X (fka Twitter) with some of the key words highlighted:

As a patient, what I heard was that the nerves that control my pupil died, and that doesn’t sound good. The doc did tell me, or at least my takeaway was, that the muscles in the eye will eventually compensate for the loss of nerves, but TBD when that might happen. Some people it takes months, some years, some never.

In the interim…

I tried hopelessly to fill a low dose pilocarpine eye drop prescription. Pilocarpine is a medication that stimulates the muscles of the iris to constrict. I briefly toyed around with colored contacts because I was a bit sensitive about the aesthetics of just one large pupil, and super curious to see what I’d look like with blue or brown eyes. Most importantly, I drove into parking garages very slowly during daylight hours. The most annoying symptom I had back in 2005 was the turtle speed it took for my eyes to adjust to changes in light. Driving from the sunlit street into the dark parking garage at my office building would briefly blind me.

Things got better…

Eventually my left eye muscles did compensate for the loss of nerve function, and my eyes returned to normal for the most part. Whenever I would get tired or have a few glasses of wine, my pupils would become noticeably mismatched, aka anisocoria.

I moved to San Francisco in 2011, I got a new ophthalmologist who ran me through the usual eye tests and also ordered brain scans to check for multiple sclerosis (MS). Both MS and Adie’s involve neurological dysfunction, and, from what I understand, can be associated with each other. Multiple sclerosis is a neurological autoimmune condition that affects the central nervous system, and there have been some cases where individuals with MS develop Adie’s. In my case, the scans confirmed that I did not have MS.

… and then…

It’s 2024 and over the last several weeks I noticed that my vision is blurry more often. I ignored the symptom — I’m tired, I’m stressed, I read a lot for work, I’m in my 40s… blurry vision happens. Then I noticed that my right pupil was enlarged… and off-center. Even though I’d been diagnosed Adie’s in my left eye nearly two decades ago, I still had a google and stressed over the thought of a brain bleed or worse.

I saw my ophthalmologist who confirmed that I’ve got Adie’s in my right eye now, nearly 20 years after I first got it in my left eye.

The symptoms

I have more symptoms now than I remember having the first time around. Honestly, I don’t know how many of the symptoms I can blame on Adie’s, but I’ll list them out nonetheless:

• affected pupil is often much larger than the other, and off center
• it is frustratingly hard to read at times, and I sometimes cover the affected eye to help focus
• low grade headaches that come and go
• Alice and Wonderland warped effect having two eyes trying to focus and ajust to light differently
• bit nauseated, feeling like my equilibrium is being messed with
• anxious
• emotional, or more emotionally sensitive than is typical for me
• tired… just want to rest (maybe on a walk with a podcast or in bed with Netflix)
• periodic sinus pressure (I’ve been plagued with sinus issues for 20+ years, not sure if there’s any relation)

The prognosis

The prognosis is still the same — Adie’s is generally considered to be a benign condition in terms of overall health. Hopefully, over time the muscles in the affected eye will compensate for the loss of nerve function.

Some good news for those with Adie’s — these days it is easier to get pilocarpine eye drops! The FDA recently approved pilocarpine for treatment of presbyopia, which is super common. Presbyopia literally translates from Greek to “old eyes” and refers to the fuzzy near vision that causes people to squint at the dinner menu in their 40s and 50s.

I’ve been using a drop of pilocarpine in my affected eye for a week now and it does help me to see better, but it also has annoying side effects — some burning at onset, tension headache around eye, hard to see in dim light, general discomfort. Overall the side effects seem relatively mild, and I’m hopeful that they subside over time. I also don’t use the drops every day… I feel like using them every day might slow my eye muscles from stepping in to compensate for lack of nerve function. That’s just my non-medical logic, and I don’t love the pilocarpine side effects.

What causes Adie’s and how rare is it?

The exact cause of Adie’s is not fully understood in all cases, including mine. Some causes cited for the development of Adie’s include: viral infection that leads to inflammation or damage to the nerves; autoimmune factors, specifically the immune system targets the nerves that control the pupil size; trauma or injury that damages the nerves that control the eye; other neurological conditions, like MS; and idiopathic aka IDK.

Estimates of prevalence vary, but the National Center for Biotechnology Information (NCBI), each year roughly 4-5 people out of 100,000 may develop Adie’s syndrome. Young adults between the ages of 25 to 45 are most commonly affected, with women roughly twice as likely as males to get Adie’s.

And that’s my Adie’s story to date! Thanks for reading. If you’re recently diagnosed with Adie’s, welcome to the club.

xoxo

-ash