Adie Syndrome: Symptoms, Causes, Disorders, And Treatments

Adie syndrome or Holmes-Adie syndrome is a form of rare neurological disorder affecting the pupil of a person’s eye.

It is named after the British neurologist William John Adie and Gordon Morgan Holmes. They independently described the same syndrome in 1931.

Adie Syndrome

In most individuals, the pupil is dilated(larger) than normal size and slow to react in response to direct light. Weak or absent reflexes are also associated with the disorder.

Signs And Symptoms Of Adie Syndrome:

Adie syndrome can happen due to conditions such as surgery, trauma, lack of blood flow or infection. Usually, the pupil gets constricts(smaller) in the presence of bright light or when focusing on nearby objects.

The pupil usually opens wider (dilates) in darkness or dim light, when focusing on remote objects, or when a person is excited.

Other symptoms a person with Adie’s syndrome may experience:

  • Facial pain
  • Blurry vision
  • Headache
  • Adie’s Syndrome
  • Emotional fluctuations
  • Adie’s Pupil
  • Holmes-Adie Syndrome
  • Adie’s Tonic Pupil
  • Tonic pupil syndrome

The syndrome is referred to as Adie when both abnormalities of the pupil and loss of deep tendon reflexes are present. However, these symptoms may not occur at the same time.

When only abnormalities affecting the pupil, the disorder may be called as Adie’s pupil, Adie’s tonic pupil or tonic pupil. When an individual’s pupils are of unequal size, then it is known as anisocoria (1).

Adie syndrome Causes

Causes Of Adie Syndrome:

In most cases, the exact cause of Adie syndrome is unknown. There are many possible causes, including a bacterial or viral infection that harms the nerves, but often Adie’s pupil is idiopathic. It means no known cause.

The tonic pupil in Holmes-Adie syndrome is believed to result from inflammation or damage to the ciliary ganglion (a cluster of nerve cells can be found behind the eye) or damage to the post-ganglionic nerves.

The ciliary ganglion is located at the parasympathetic nervous system, an element of the autonomic nervous system (2,3). It controls the pupil’s response to light and other stimulants.

In most instances, ciliary ganglion or post-ganglionic nerves damage is caused by an infection. Injury may result from autoimmune processes, trauma, tumors, and surgery complications.

The deep tendon loss reflexes in Adie syndrome is caused by damage to the dorsal root ganglion. It is a cluster of nerve cells in the root of the spinal nerves.

In rare cases, Holmes-Adie syndrome can be inherited, and it is called as an autosomal dominant characteristic (4).

The risk of transmitting the disease from affected parent to child is 50% for each pregnancy, despite the gender of the child. Adie syndrome occurs in both women and men. It is most often in women between the ages of 25-45.

Adie Syndrome Related Disorders:

Symptoms of the following conditions can be similar to those of Adie’s syndrome. Comparisons may be helpful for a differential diagnosis:

  • Ross syndrome:

In this disorder, the affected people experience an impaired ability to sweat-out normally (anhidrosis). This syndrome is related to Adie pupil and loss of deep tendon reflexes.

The inability to sweat well in some regions of the body may result in the other areas sweating excessively. Individuals may come across heat intolerance due to the sweating abnormalities.

So far, more than 40 cases of Ross syndrome have been registered in the medical literature. The exact cause of this disorder is still unknown (5).

Holmes-Adie syndrome Disorders

  • Third Nerve Lesions:

It may cause a dilated pupil. Tests can decide whether the dilation is due to Holmes-Adie syndrome, or to a lesion of the third nerve (6).

  • Peripheral Neuropathy:

It might cause a un-reactive pupil and certain other disorders. Peripheral neuropathy symptoms are created by disease of a single nerve, or many nerves together. The symptoms can include sensory, motor, reflex or blood vessel function. Lesions might happen in the peripheral nerves or nerve roots (7).

Aside from the diseases mentioned above, several other factors may cause pupil dilation.

Certain drugs can cause an individual’s pupil to dilate. Transdermal scopolamine is a drug meant for motion sickness comes in the form of a patch (8).

If somebody accidentally applies the product on their eye, without washing their hands, it may lead to pupil dilation.

Other substances may cause dilated pupils or a delay in the response of eye muscles to light or darkness.

Adie Syndrome Treatments:

Holmes-Adie syndrome diagnosis can be determined by using dilute pilocarpine. It is a drug in the form of eye drops, to test the pupil’s reaction to light.

The Adie syndrome pupil, which doesn’t constrict in response to light, may happen in response to dilute pilocarpine. Other forms of treatment may include:

  • Glasses: It is used to correct the person’s blurred vision. Therapy using dilute pilocarpine might improve poor depth perception in some individuals.
  • Genetic Counseling: This treatment may benefit family members. It is the process of understanding the medical conditions in the family.

Around 50% of people with Holmes-Adie syndrome recover fully within two years. In some cases, the affected pupil becomes smaller than the normal pupil, whereas, in others, the near pupillary response never recovers fully (9).

Adie syndrome complications

Adie Syndrome Complications:

Adie Syndrome is not a life-threatening disease. This disorder does not ordinarily cause severe disability.

However, Adie’s pupil may lead to premature loss of near-focusing power similar to¬†presbyopia, a disorder that occurs around the age of 40 (10).

Some people may report extreme light sensitivity. Others may face difficulty with night vision or driving at night. Additionally, people with excessive sweating find it difficult to live a healthy lifestyle.

Individuals with Holmes-Adie syndrome may also experience other abilities of autonomic function such as issues with cardiovascular function.

The research on Adie syndrome is still in process. It is one of the rare disorders in medical history. Scientists are trying to identify the cause of the disorder. These experts aim to find better types of treatment for the syndrome.

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