Adie syndrome is a rare neurological issue that affects the pupil of the eye and the autonomic nervous system. The affected pupil may be more dilated than normal and might be slower to respond to light.
Adie syndrome also produces other symptoms, such as vision changes and a loss of deep reflexes. In many cases, the exact cause is unknown, though inflammation or nerve damage may play a role. Treatment may involve using specialized glasses to treat any vision impairments, as well as eye drops to help the affected pupil.
People may sometimes refer to Adie syndrome as Holmes-Adie syndrome (HAS) or tonic pupil syndrome. Keep reading to learn more about Adie syndrome, including some possible causes and the symptoms and treatment options.
In most cases, Adie syndrome is idiopathic, which means that doctors do not know the exact cause. Symptoms may have their own mixed causes.
A leading theory is that inflammation or nerve damage in the ciliary ganglion, which sits just behind the eye, causes symptoms in the eye, including the improperly dilated pupil.
This inflammation or damage to the nerves may arise from issues such as:
- autoimmune conditions
- complications from surgery
- traumatic injury
The loss of deep tendon reflexes, such as the Achilles or patellar reflex, may also occur due to similar damage in the nerves. Damage to the dorsal root ganglion at the root of spinal nerves may have similar causes and could lead to a loss of reflex in these areas.
In rare cases, causes of Adie syndrome may have a genetic aspect, as well. In these instances, it is possible to inherit the syndrome from a parent.
Typically, Adie syndrome may result in a change in the dilation of one of the pupils.
In general, the pupils get smaller or larger in response to changes in light. These changes happen very quickly to help humans see in different light conditions. If a person has Adie syndrome, the affected pupil does not do this correctly. The pupil may stay dilated, meaning that it is open wider than necessary in the presence of light.
The eye may be less responsive, even when doctors shine a bright light near the eye to test for a reaction.
The affected eye may not necessarily cause symptoms. However, some people may experience impaired vision. The vision may be blurry, for instance, or the person may have trouble with depth perception.
In other cases, symptoms such as light sensitivity or the presence of “light glares” within the field of vision may also occur.
The symptoms of Adie syndrome tend to affect just one of the eyes. However, they may eventually begin to affect both eyes.
In addition to symptoms in the eye, a person with Adie syndrome will also have sluggish or absent deep tendon reflexes. Deep tendon reflexes are automatic, involuntary responses of the muscles in response to stimuli. Examples include reflexes in the knee and Achilles tendon.
Some people may experience additional symptoms, including headaches, pain in the face, and mood swings.
In some cases, a person may experience changes in sweating, which can be either excessive or reduced. While the combination of decreased sweating, absent reflexes, and poor pupil responses is technically known as Ross’s syndrome, some doctors may diagnose this as a variant of Adie syndrome.
The symptoms of Adie syndrome are typically not severe or debilitating, but the condition can disrupt daily life and usually requires treatment. Anyone experiencing these symptoms should see a doctor or ophthalmologist for a diagnosis.
An ophthalmologist can help diagnose the symptoms of Adie syndrome. However, correctly diagnosing rare diseases, such as Adie syndrome, can take time.
A diagnosis of Adie syndrome generally involves evaluating the person’s medical history and genetics. Doctors will use a physical exam to look for the typical symptoms of Adie syndrome, such as slow reflex responses or symptoms in the eye.
In some cases, doctors may recommend genetic testing to look for known associated traits.
The type of treatment is likely to depend on the symptoms that a person with Adie syndrome is experiencing.
Doctors may recommend eye drops containing a medication such as pilocarpine (Salagen), which can help constrict a pupil that has become overly dilated. This constriction may help with symptoms such as light glare and depth perception.
Other people may need to use prescription glasses or get a change in their prescription to help adjust their vision and compensate for any vision loss.
Those with excessive sweating may need a procedure called a thoracic sympathectomy to treat the issue if they do not respond to medication.
Other symptoms, such as absent or slow reflexes, may be permanent.
Few complications arise from Adie syndrome outside of the symptoms themselves.
Research suggests that 80% of cases are unilateral, meaning that they only affect one eye. Therefore, only about 20% of cases involve both eyes to some degree. The involvement may also progress, meaning that it may start in one eye and slowly start to affect the other eye, as well.
The eyesight of a person with Adie syndrome may change, and many people will have to wear corrective lenses to compensate for changes in vision.
The symptoms of Adie syndrome can be bothersome and disrupt daily life, but they are typically manageable.
Aside from diagnosing and treating any possible underlying conditions, the outlook for people with Adie syndrome is generally good.
The syndrome is not progressive and does not pose any major threat. It is not life threatening and does not typically cause disability.
Some symptoms may be progressive. For instance, the loss of tendon reflexes tends to progress, and this is permanent.
While the pupil of the affected eye is generally larger when the person is younger, the affected pupil may shrink as the person ages. Older people with Adie syndrome may have a much smaller pupil in their affected eye.
Adie syndrome is a rare neurological condition that causes symptoms in the eyes and the autonomic nervous system. The affected eye may be more dilated or open than normal, and it will be very slow to respond to light.
The person may also have very sluggish or absent deep tendon reflexes.
Although the symptoms of Adie syndrome can be annoying, they are typically manageable. Eye drops and eyeglasses can help manage symptoms in the eye. Other symptoms, such as the loss of deep tendon reflexes, are often permanent.
Anyone experiencing noticeable symptoms of the condition, such as one pupil that is larger than the other or changes in vision, should see a doctor for a diagnosis.