Adie Syndrome - Symptoms, Diagnosis, Complications and Treatment

What Is Adie Syndrome?

Adie syndrome is a neurological disorder that affects the eye pupil and the autonomic nervous system resulting in poor light. This disorder is otherwise known as Adie tonic pupil or Holmes-Adie syndrome. This syndrome is named after the British neurologist William John Adie and Sir Gordon Morgan Homes, an Irish neurologist. It is relatively a rare condition affecting around five people in every 1,00,000 people and is most commonly seen in young adults between 25 and 45 years of age. Adie syndrome is more likely to affect females than males. The condition is referred to as Adie syndrome when both the pupil and the deep tendon reflexes are affected. When only one pupil is affected, then the condition is termed Adie’s pupil.

How Does Adie Syndrome Develop?

Adie syndrome is idiopathic, meaning the exact cause is not known. A theory stated that inflammation or nerve damage in the ciliary ganglion could cause these symptoms in the eye. The ciliary ganglion supplies nerves to the eye. These nerves carry signals that help control the pupil’s response to the stimuli, like dilating or becoming smaller in response to light, dark, or other stimuli. The inflammation or nerve damage occurs due to the following reasons:

• Infections.

• Traumatic injury.

• Tumors.

• Autoimmune conditions.

• Complications from the surgery.

Adie syndrome is non-progressive and is caused due to damage to the autonomic nervous system, the portion of the nervous system that controls or regulates some involuntary movements, such as the reaction of the pupils to the stimuli. In very rare cases, Adie syndrome is caused due to a genetic condition where the children inherit the condition from their parents.

What Are the General Presenting Signs and Symptoms of Adie Syndrome?

Normally the pupils constrict or get smaller in the presence of light or when focusing near objects, and the pupil dilates in dim light or darkness or when focusing on objects that are placed far away. In rare cases, the iris can lose most or all of its color. But in people with Adie syndrome, several different symptoms are developed of which primary symptoms include:

• The pupil of the affected eye seems larger or more dilated than the normal eye.

• The affected eye reacts abnormally to the light.

• Decreased or loss of deep tendon reflexes.

• Abnormalities of sweating.

• Difficulty in reading.

• Hyperopia (farsightedness or blurry vision when seeing nearby objects).

• Anisometropia (a condition when both eyes have unequal refractive power).

• Cardiovascular abnormalities like orthostatic hypotension.

• Adie syndrome can also be associated with chronic coughing.

In the beginning stages, the pupil reacts slowly while performing close tasks like reading. This is because the eye begins to lose its close-ranging focusing power. After the extended near focusing, the involved pupil becomes tonic and remains constricted even after discontinuing the accommodative effort. Adie syndrome generally affects the pupil of one eye, but eventually, the other eye also gets affected. The other symptoms include:

• Headache.

• Facial pain.

• Emotional fluctuations.

How Is Adie Syndrome Diagnosed?

Adie syndrome is diagnosed by a thorough clinical examination and a thorough medical history. The eye is examined entirely using a water-downed Pilocarpine to test the pupil’s reaction. The drug Pilocarpine is given in the form of eye drops which causes the pupils to constrict. In people with Adie syndrome, the affected pupils which do not constrict in response to the light will constrict in response to this Pilocarpine, to which a normal pupil usually will not constrict.

Some people notice tonic pupil constriction when the conjunctiva is touched or irritated. The doctor will also examine the size of the affected and unaffected eyes in dim or dark and light. The diagnostic tests are described below:

• Slit-Lamp Examination: In this examination, the device magnifies and illuminates the pupils. The syndrome shows the symptoms when closely watched.

• Pupil Response Testing: In this examination, the doctor determines how well the pupil responds to dim or bright light. The testing also examines how the pupil accommodates and focuses on an object.

• Sweat Testing: The starch iodine test and the spoon test are used in the detection of anhidrosis (inability to sweat normally) and to exclude other conditions like Ross or Harlequin syndromes (on sweating the skin color changes).

Can Adie Syndrome Be Reversed?

Typically Adie syndrome does not go away. The size of the pupil can become smaller over time but does not regain the ability to respond normally to light.

How Is Adie Syndrome Treated?

The type of treatment depends on the symptoms and severity of the condition a person with Adie syndrome is experiencing. The condition is generally treated with eye drops containing Pilocarpine which helps constrict a pupil that has been overly dilated. When the pupil is constricted, then symptoms like light glare and depth perception are resolved. Certain people may be suggested to use prescription glasses to adjust their vision and to compensate for vision loss. Thoracic sympathectomy is the definitive treatment for sweating abnormalities. It is surgery that is performed to treat excessive sweating. In this surgery, the nerves that cause the body to sweat too much are cut. A few symptoms like absent or slow reflexes are usually permanent.

What Is the Differential Diagnosis of Adie Syndrome?

• Third cranial nerve palsy.

• Argyll Robertson pupil (small and irregular pupils that do not react to light but constrict during focusing).

• Horner syndrome (the symptoms include a drooping eyelid, constricted pupil, and loss of sweating).

• Parinaud syndrome (is characterized by vertical gaze palsy and light near dissociation of pupils).

• Myasthenia gravis (fluctuating ptosis and diplopia).

What Are the Complications of Adie Syndrome?

Adie syndrome is a benign condition and is not a life-threatening condition. It causes premature loss of near-focusing power. Certain people may also have extreme light sensitivity. They may also have difficulty with night driving or night vision. Excessive sweating can also affect the quality of life.

What Is the Prognosis of Adie Syndrome?

Adie syndrome can cause some chronic symptoms. However, it does not cause severe health problems and affects the life expectancy of the affected individuals. In some people, the prognosis of Adie syndrome is good with treatment and in some, it causes permanent symptoms.

Conclusion:

Generally, the symptoms of Adie syndrome can be worrisome and can disrupt day-to-day life but can be managed by taking appropriate treatment. A worrisome symptom may affect the person's life and affect their daily tasks. Adie syndrome is not a progressive disease and is not life-threatening or does not pose any major threat to the person's life. In most cases, the prognosis of Adie syndrome is typically good.