Holmes-Adie syndrome, or Adie syndrome, is a neurological disorder that affects about 2 in every 1,000 people.1 It was named for the neurologists who discovered it — William John Adie and Sir Gordon Morgan Holmes.
Adie syndrome affects the autonomic nervous system, which controls automatic bodily functions like the pupillary response. Adie syndrome may also cause a decrease or loss of deep tendon reflexes, especially in the Achilles tendon.
It’s more common in females than in males and often affects young adults between the ages of 25 and 45. Adie syndrome may also cause a decrease or loss of deep tendon reflexes, especially in the Achilles tendon.
Other names for Adie syndrome include:
- Adie’s syndrome
- Tonic pupil syndrome
- Adie tonic pupil
- Adie’s tonic pupil
Adie syndrome isn’t serious or life-threatening. However, the loss of deep tendon reflexes may be irreversible.
What is Adie’s Pupil?
A tonic pupil, or Adie pupil, is the main characteristic of Adie syndrome. The condition is often called Adie’s pupil when only the pupil is affected.
The affected pupil may be abnormally large (dilated) and slow to constrict in bright light. In most cases of Adie syndrome, only one eye is affected (unilateral). It’s a common cause of unequal pupil sizes (anisocoria).
Adie syndrome is mostly idiopathic, meaning it occurs without known cause. But it may involve damage to bundles of nerve cells, including:
- Parasympathetic ciliary ganglion. Located just behind the eye, the ciliary ganglion supplies the pupillary sphincter muscle, which constricts the pupil, and the ciliary muscle, which changes the shape of the lens.
- Dorsal root ganglion. It is part of the autonomic nervous system located at the root of the spinal nerves.
The nerve damage is likely due to inflammation, which may stem from infections, injuries, or ocular surgery.
How is Adie’s Pupil Different from Other Pupils?
An Adie tonic pupil’s distinct characteristics differentiate it from normal pupils and other pupil abnormalities.
However, these characteristics aren’t exclusive to an Adie tonic pupil. It’s essential to get an accurate clinical diagnosis from a medical professional.
Adie’s Pupil vs. Argyll Robertson Pupil
Argyll Robertson pupils are abnormally small in both eyes (bilateral tonic pupils). This condition is specific to late-stage syphilis. The pupils get smaller (constrict) when focusing on a near object, but not in response to bright light.
In Adie syndrome, the tonic pupil usually affects only one eye. The pupil in the affected eye may constrict in response to light, but more slowly than it should.
Adie’s Pupil vs. Hutchinson’s Pupil
A Hutchinson pupil is a fixed and dilated pupil in one eye. It may look like Adie syndrome due to the unequal pupil size difference.
A Hutchinson pupil differs in that it occurs when the oculomotor nerve gets compressed. This usually results from a mass inside the brain.
Adie’s Pupil vs. Normal Pupil
An Adie tonic pupil differs from a normal pupil in many ways:
- Pupil size. The tonic pupil in the affected eye is noticeably larger than the pupil of the normal eye.
- Response to light. A normal pupil dilates in low light and rapidly constricts in bright light. Adie syndrome causes a poorly reactive pupil that takes a long time to constrict in bright conditions.
- Response to near objects. While an Adie tonic pupil is sluggish to react to light, the pupil constricts more than a normal pupil when focusing on near objects.
How is Adie’s Pupil Diagnosed?
An ophthalmologist can diagnose the symptoms of Adie syndrome with a comprehensive exam. They’ll ask about any symptoms you have and look for a pupil that appears larger than it would in a normal eye.
To come to a diagnosis, your doctor may perform the following tests:
- Slit lamp examination. This involves examining the inside of your eye with a special lighted microscope.
- Diluted pilocarpine. Applying these medicated eye drops to your eyes will cause the affected pupil to constrict more than the normal pupil.
People with Adie syndrome may need to see multiple medical professionals to evaluate non-eye symptoms like impaired sweating and reduced tendon reflexes.
Does Adie’s Pupil Affect Vision?
Adie syndrome doesn’t significantly impair your vision. However, people with Adie syndrome may experience:
- Differently sized pupils
- Light sensitivity (photophobia)
- Blurry vision, both near and far
- Trouble with depth perception
- Problems with light glare
- Difficulty adapting to dark conditions
Tell your eye doctor if you have any of these vision symptoms. They can recommend the best treatment for you.
Summary
Holmes Adie syndrome, also known as Adie syndrome, Adie’s syndrome, and tonic pupil syndrome, is a neurological disorder that affects the eye pupil. It involves damage to parts of the autonomic nervous system that control pupillary constriction.
The most obvious symptom of Adie syndrome is an abnormally large (dilated) pupil. It most often affects one eye but can occur in both. The affected pupil is slow to constrict in bright light. Other common symptoms include reduced deep tendon reflexes and excessive sweating.
Adie syndrome isn’t serious or life-threatening. However, it’s important to see a doctor for a diagnosis. Symptoms of Adie syndrome may resemble those of other pupil abnormalities that are more serious and require treatment.
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