Therefore, accommodation difficulties are not obvious or less obvious. Because there are so many nerve cells serving accommodation usually a sufficient amount survive. In Adie syndrome, both these nerve cells are damaged. However, most of the cells of the ciliary ganglion (97%) serve accommodation and supply the ciliary muscle which adjusts the crystalline lens of the eye to near vision. These nerves communicate with the iris sphincter muscle, the muscle that controls how much light enters the pupil (causing the pupil to either contract or grow larger). These nerves carry signals that help to control the pupil’s response to stimuli such as growing smaller or larger in response to light, dark or other stimuli. The ciliary ganglion supplies nerves (innervates) to the eye. The parasympathetic nervous system relaxes the body and inhibits or slows down high energy functions. The ciliary ganglion is part of the parasympathetic nervous system, which is itself part of the autonomic nervous system. It is believed that most cases result from inflammation or damage to the ciliary ganglion, a cluster of nerve cells found in the eye socket (orbit) just behind the eyes, or damage to the post-ganglionic nerves. In most instances, the exact cause of Adie syndrome is unknown (idiopathic). There have been some reports in the medical literature that individuals with Adie syndrome may experience other abilities of autonomic function such as issues with cardiovascular function.

Adie syndrome usually affects the pupil of one eye, although the other eye may eventually become affected as well. The disorder does not ordinarily cause severe disability. Headache, facial pain, or emotional fluctuations may occur in some patients. A physician may tap this area with a small rubber hammer, which should cause the lower leg to kick out in individuals with Adie syndrome this reflex response is poor or does not occur. For example, the patellar reflex occurs in the area just below the kneecap (patella). Deep tendon reflexes are involuntary muscle contractions that occur to a sudden stimulus. Individuals with Adie syndrome have absent or poor (sluggish) deep tendon reflexes as well. Sometimes blurry vision or sensitivity to bright lights (photophobia) can occur. Some individuals may not have symptoms associated with the affected pupil. When no longer focusing on the close at hand object, the affected pupil may remain smaller than normal or grow larger (re-dilate) at an abnormally slow rate, sometimes taking as long as several minutes to return to its original (i.e. Eventually, after months or years, the pupil, which was initially larger than the unaffected pupil, will become smaller than the unaffected pupil. This reminds of the movement of an earthworm.

If the pupil is observed with a loupe or microscope a typical behavior can be observed both spontaneously or during near response: Some sectors of the sphincter muscles constrict and others don’t. The pupil will constrict slowly when focusing (=accommodating) on objects close at hand (normally referred to as near response). In many cases it is not ideally round but slightly ovally distorted.

In most patients with Adie syndrome the affected pupil is larger than normal (dilated) all the time and does not constrict very much or not at all in response to direct light. The pupil normally opens wider (dilates) in dim light or darkness, when focusing on far away objects, or when a person is excited. Normally the pupil gets smaller (constricts) in the presence of light or when focusing on nearby objects. When a person’s pupils are of unequal size, the term anisocoria may be used. When only abnormalities affecting the pupil are present, the disorder may be referred to as Adie’s pupil, Adie’s tonic pupil or, most commonly, tonic pupil.

However, these findings may not develop at the same time. The term Adie syndrome is used when both abnormalities of the pupil and loss of deep tendon reflexes are present. Adie syndrome involves a usually non progressive and limited damage to the autonomic nervous system, which is the portion of the nervous system that controls or regulates certain involuntary body functions including the reaction of the pupils to stimuli. In rare cases localized disturbance of sweat secretion is associated with Adie syndrome (Ross syndrome). In most individuals, the cause is unknown (idiopathic), but Adie syndrome can occur as due to other conditions such as trauma, surgery, lack of blood flow (ischemia) or infection. Absent or poor tendon reflexes are also associated with this disorder. In most patients the pupil is larger than normal (dilated) and slow to react in response to direct light. 5 Myths About Orphan Drugs and the Orphan Drug ActĪdie syndrome, or Holmes-Adie syndrome, is a rare neurological disorder affecting the pupil of the eye.Information on Clinical Trials and Research Studies.

Unequal pupil size stroke