Adie Syndrome: Signs, Symptoms, Causes and Treatment
Synopsis: Adie syndrome, sometimes called the Holmes-Adie syndrome, is a neurologic disorder in where the ability of the pupil to constrict is impaired, usually in one eye.1
Author: Thomas C. Weiss Contact: Disabled World
Published: 2015-07-13 Updated: 2017-06-04
Adie syndrome is a form of rare neurological disorder affecting the pupil of a person's eye. In most people, the pupil is dilated and slow to react to light on nearby objects. In others who are affected, the pupil might be constricted instead of dilated. Poor or absent reflexes are also associated with the disorder. Adie syndrome is not progressive, nor is it life-threatening, or disabling. Other names for Adie syndrome include:
Adie syndrome, also called Holmes-Adie syndrome or Adie's tonic pupil, is a neurological disorder characterized by a tonically dilated pupil that reacts slowly to light but shows a more definite response to accommodation. Adie syndrome presents with three main symptoms, namely at least one abnormally dilated pupil (mydriasis) which does not constrict in response to light, loss of deep tendon reflexes, and abnormalities of sweating. Other signs may include hyperopia due to accommodative paresis, photophobia and difficulty reading.
- Adie's Pupil
- Adie's Syndrome
- Adie's Tonic Pupil
- Tonic Pupil Syndrome
- Holmes-Adie Syndrome
- Papillotonic Psuedotabes
Signs and Symptoms of Adie Syndrome
Adie syndrome is a rare neurological disorder that usually affects the pupil of one eye or occasionally both eyes. Usually, the pupil constricts in the presence of bright light, or when focusing on nearby objects. The pupil usually dilates in light that is dim or in darkness, when focusing on far away objects, or when a person becomes excited. In Adie syndrome, these usual reactions to darkness and light do not happen.
In the majority of people with Adie syndrome, the affected pupil is larger than usual all the time and does not constrict very much or at all in response to light stimulation. The person's pupil constricts slowly when focusing on objects close to view. In some people with the syndrome; however, the opposite is true - the pupil stays smaller than usual all the time. Most people with Adie syndrome have poor or absent reflexes as well. Other symptoms a person with this syndrome may experience include:
- Facial pain
- Blurry vision
- Emotional fluctuations
The disorder is not progressive and does not usually cause severe disability. In Adie syndrome, muscles that cause the pupil of the person's eye to contract or dilate remain tensed, creating the symptoms people experience. Lesions to a certain part of the third nerve cells might cause the lack of reflexes. Scientists have identified lesions in certain nerves in some people, although they do not know what causes them to happen.
Causes of Adie Syndrome
While the exact cause of Adie syndrome remains unknown, it might be inherited as an autosomal dominant trait. Human traits, to include the classic genetic diseases, are the product of the interactions of two genes; one received from the mother and the other from the person's father. In dominant disorders, a single copy of the disease gene will be expressed, 'dominating,' the other usual gene and resulting in the appearance of the disease. The risk of transmitting the disorder from affected parent to child is 50% for each pregnancy, despite the gender of the resulting child. Adie syndrome happens in both women and men, yet most often in women between the ages of 25-45.
Disorders Related to Adie Syndrome
Symptoms of the disorders below may be similar to those of Adie Syndrome. Comparisons might be useful for a differential diagnosis.
- Third Nerve Lesions: Third nerve lesions may cause a dilated pupil; tests can determine whether the dilation is due to Adie syndrome, or to a lesion of the third nerve unrelated to Adie syndrome.
- Peripheral Neuropathy: Peripheral neuropathy might cause an un-reactive pupil; so can certain other disorders. The symptoms of peripheral neuropathy are produced by disease of a single nerve, or many nerves at the same time. The symptoms can involve motor, sensory, reflex or blood vessel function. Lesions, usually degenerative, might happen in the peripheral nerves or nerve roots.
Aside from diseases, a number of other factors may cause pupil dilation.
Certain drugs can cause a person's pupil to dilate. For example; transdermal scopolamine is a drug for motion sickness that comes in the form of a patch. If a person accidentally gets the product on their eye - by not washing their hands when they are finished with the kit, it may cause pupil dilation. Other drugs may cause dilated pupils, or a delay in the response of eye muscles to darkness or light.
Treating Adie Syndrome
A diagnosis of Adie syndrome can be achieved by using dilute pilocarpine - a drug in the form of eye drops, to test the pupil's reaction to light. The Adie syndrome pupil, which does not constrict in response to light, will constrict in response to dilute pilocarpine. Other forms of treatment may include:
- Genetic Counseling: Genetic counseling may benefit parents and family members. Other treatment is symptomatic and supportive.
- Glasses: Glasses might be prescribed to correct the person's blurred vision. Therapy using dilute pilocarpine may improve poor depth perception in some people.
Research of Adie syndrome continues to be pursued.
Scientists are attempting to identify the underlying cause of the disorder. The goal of these scientists is to find better types of treatment for the syndrome.
Adie syndrome most commonly affects younger women (2.6:1 female preponderance) and is unilateral in 80% of cases. Average age of onset is 32 years.
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