What is Adie syndrome?

Adie syndrome is a rare neurological disorder that impacts the autonomic nervous system, causing one pupil of the eyes to be larger than the other.

It is further characterized by one eye possessing a larger-than-normal pupil that will constrict slowly in bright light, called a tonic pupil, and the absence of deep tendon reflexes, most often associated with the Achilles tendon. 

What are the symptoms of Adie syndrome?

Changes in light usually make pupils smaller or larger. The changes happen fast, so our eyes can adapt to view through various lighting conditions. 

In Adie syndrome, the pupil stays typically dilated even if the light is very bright.

Common symptoms in the eye with Adie syndrome include:

• Blurry vision
• Dim vision
• Difficulty with depth perception
• Photophobia or sensitivity to light or having 'glare from light.

The symptoms of Adie syndrome may affect only one eye. They may eventually affect both eyes, though.

Additional symptoms

In addition to the ocular symptoms, Adie syndrome presents with slow or absent deep tendon reflexes. Deep tendon reflexes are involuntary and automatic muscle reactions, such as knee jerks and Achilles tendon reflexes.

Other symptoms the patient may exhibit include the following:

• Headaches
• Pain in the facial region
• Mood swing

A person's sweating may vary, from excessive to reduced at times.

Ross's Syndrome: Though technically decreased sweating, absent reflexes, and poor pupil responses are described as the characteristics of Ross's syndrome, some clinicians prefer to use this term to describe a variant of Adie syndrome.

Though symptoms of Adie syndrome are rarely severe or disabling, they can disrupt one's daily life considerably and may require treatment.

What causes Adie syndrome?

The causes of Adie syndrome often are unknown, though a few cases have genetic root causes and may be hereditary from one of its parents or family members.

These could develop from the following:

• Tumors
• Infections
• Trauma injuries
• Autoimmune problems
• Complications were caused at the time of surgery.

Deep tendon reflexes, including the Achilles and patellar reflexes, can also be lost from nerve damage. The exact causes of damage to the dorsal root ganglion at the root of spinal nerves might cause a lack of response in those areas.

How do you diagnose someone with Adie syndrome?

Adie's pupil is somewhat self-explanatory by the lack of pupillary response. However, even for a self-explanatory diagnosis, the eye doctor, known as an ophthalmologist, will wish to know the aetiology if possible and describe the findings in order to treat the patient properly.

The first question an ophthalmologist will ask is your medical history. Have you been having any infections or injuries recently when you noticed your pupils are different.

Now, an eye test encompassing the following would be conducted by the ophthalmologist:

• Pupil response testing: This straightforward test shows how your pupil responds to bright and low light.
• Slit-lamp exam: This device enlarges and lights up your pupils.
• Diagnostic eye drops: These may cause your pupils to become dilated but might fail if you have Adie's pupil.

How is Adie syndrome treated?

• Because the symptoms of a person with Adie syndrome can vary, the treatment depends on the symptoms.
• Physicians prescribe eye drops containing pilocarpine, a drug that may help constrict a pupil that has become too large.

• Reduction in the size of the affected pupil will also help reduce the glare of light and depth perception.
• Some may require prescription eyeglasses or a correction of their prescription to adapt to and compensate for vision loss.
• People with profuse sweating can also undergo thoracic sympathectomy if the medicine fails to help.
• A few signs, such as reflexes not working or being extremely slow, may last forever.

Can Adie syndrome be prevented?

There is no known cure for Adie syndrome; however, its symptoms can be relieved with some treatment options.

• Bifocal lenses or reading glasses to aid in focusing difficulties.
• Sunglasses to minimize light sensitivity.
• Prescription eye drops containing pilocarpine or brimonidine cause pupils to constrict, facilitating night driving and night vision.

What is the prognosis for Adie syndrome?

As the pupillary disorder in Adie is so poorly understood, the prognosis may be uncertain.

With that caveat aside, a good proportion of individuals will recover fully in months to years. In some, the affected pupil will remain smaller than the usual pupil and will not get larger than the normal pupil. In others, the pupil response may never recover completely.

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