Adie's Pupil

Definition

Adie's pupil is a neurological condition of unknown origin with an unusual, asymmetric presentation known as anisocoria, an inequality in the size of the pupils of the eyes. It is believed to be a result of damage to the nerve innervating a muscle of the eye known as the ciliary body. Alternately, the problem may be located at the ciliary ganglion, a kind of nerve junction structure from which the nerve to the ciliary body runs. Accommodation, or the adjustment of the eye for distance, is affected, as well as pupillary dilation and contraction, the ability of the eye's iris to open or close in response to ambient light. Adie's pupil primarily affects women from 20 to 40 years of age. It is considered to be a benign condition with no known cure. When Deep Tendon Reflexes (DTRs) of the legs—knee and ankle jerks—are also affected, accompanied by symptoms including localized, discreet areas of the skin that do not sweat, postural hypotension (low blood pressure related to sudden standing or rising), and unsteady heart rhythms, the condition is referred to as Adie's syndrome.

Description

The eyes are a complex anatomical and neurological unit. The outer surface of each eye is protected by a cornea—a normally clear cover that initiates the bending of light rays into the eye. Beneath the cornea lies the colorful iris, a membrane containing two muscles capable of contracting and dilating like the diaphragm of a camera as it floats in the "aqueous humor." Beneath the iris, the lens, under the influence of the ciliary body, further bends and directs the incoming light back to the retina, where it is received and transferred through the optic nerve at the back of the eye to the visual center of the brain (the visual cortex) at the back of the head. From the visual cortex, instruction based on whether the object of vision is near or far, and whether the surrounding light is bright or dim, goes back to the muscles of the eye—the ciliary body—through the ciliary ganglion, and results in a re-shaping of the lens (accommodation) and an opening or closing of the pupil (pupillary reaction), as needed, in order to focus more sharply. Under normal circumstances, brightness and accommodation for near vision will result in contracture of the ciliary body and the pupil, whereas darkness and accommodation for distance normally results in a relaxation of the ciliary body and dilation of the pupil. For a person with Adie's pupil, however, nerve signals arriving at the ciliary body of one eye are weaker than to the other eye, believed to be a result of damage to or degeneration of the ciliary ganglion or the ciliary body. The affected eye muscle is unable to contract, dilate, or focus with the same strength and speed as the unaffected eye. Relative to the opposite, unaffected eye, in normal daylight the pupil of the affected eye will be larger whereas, in a quickly darkened room, the affected eye will be smaller. Furthermore, the nerve from the ciliary ganglion to the ciliary body has 30 fibers dedicated to changing the shape of the lens and only one fiber dedicated to dilating the iris. As a result, a person with Adie's pupil is even less able to dilate their pupil than to focus. Some research suggests that as the person ages, the ability to dilate gradually lessens to the point that the eye may have a smaller (constricted) pupil almost all the time.

The list of other names by which Adie's Pupil is known includes: Adie's Tonic Pupil; Tonic Pupil Syndrome; Holmes-Adie Syndrome and Adie-Holmes Syndrome; Psuedotabes, Papillotonic Psuedotabes, and Psuedotabes pupillotonica; Kehrer-Adie Syndrome; Markus' Syndrome; Weill's Syndrome, Weill-Reys Syndrome, and Weill-Reys-Adie Syndrome; Psuedo-Argyll Robertson Pupil, Psuedo-Argyll Robertson Syndrome, and Nonluetic Argyll-Robertson Pupil; Myotonic Pupil and Myotonic Pupillary Reaction; Saenger's Syndrome; and, Constitutional areflexy-tridoplegia interna. These many names derive from the lengthy history of study of this condition, beginning in 1813, when James Ware, a London ophthalmologist, or medical doctor specializing in the eyes, described some of these symptoms. Dr. Adie added to an already long list of doctors who had studied it, when in 1931 he described the condition as self-generating. It was first referred to as Adie's syndrome in 1934 by the French neurologist, Jean-Alexandre Barré.

Causes & symptoms

Some external causes such as trauma or surgery to the eye, and some internal causes such as an unknown virus, an inflammatory condition, or a familial tendency have been suggested. In the case of trauma or surgery, it is believed that the damaged or cut nerve fails to regrow with all fibers running in the same direction for proper reattachment to the ciliary body. The propensity to affect women between 20 and 40 years of age more than men of all ages promoted one doctor to speculate that it may be, or related to, an autoimmune disorder, especially when the individual lives a stressful lifestyle and other related family members have suffered neurological diseases or disorders. Caffeine, a high sugar diet, or an imbalance in essential fatty acids that are important in maintaining nerve health may also be factors, but no studies have been identified supporting this hypothesis. Occasionally similar symptoms are seen in syphilitic persons; however, one source notes that as early as 1914, syphilis was ruled out as the cause of this condition. This unusual pupillary reflex, also known as "tonic pupil," because it is slow to change, may also be associated with diabetes, alcoholism, a herpes viral infection, or a cranial arterial condition known as giant cell arteritis. Symptoms, in addition to the slow-to-respond, differently sized pupil of the affected eye, may include headache, as a result of the strain on the vision, a sensitivity to bright lights, and loss of some reflexes, especially the knee-jerk.

Diagnosis

Diagnosis is made on the basis of a thorough history and physical examination, followed by tests to rule out other suspected causes according to the persons history. An examination of the eye using a "slit lamp"—an intensely bright lamp shielded by a shade with a slit it it—reveals undulating, irregular, worm-like movements with a segmented or ratcheted appearance in the iris of the affected eye, uncoupled to movements in the iris of the unaffected eye. A positive (hypersensitive) reaction to pilocarpine drops, an alkaloid substance from the jaborandi tree that causes the otherwise slow-to-constrict pupil to constrict intensely, is considered diagnostically additive.

Treatment

No specific treatments were noted; however, treatments that strengthen or protect the nervous system might be helpful, treatments such as the B complex of vitamins, antioxidants including Vitamins C and E, alpha lipoic acid, and Superoxidedismutase (S.O.D.), and dietary modifications of fat that have been found helpful in multiple sclerosis, another condition due to damage to nerve pathways affecting nerve transmission, including intake of essential fatty acids and reduced intake of trans-fatty acids and hydrogenated fats. Dietary reductions in caffeine and sugar, including sugar substitutes like aspartame, may be helpful. Lifestyle practices that reduce stress and tone the parasympathetic nervous system, such as yoga or massage, may be helpful.

Allopathic treatment

No specific treatments were noted, however cosmetic use of dilute pilocarpine drops to constrict the tonic pupil was suggested.

Prognosis

Partial recovery of function is more likely if the initiating damage was to the ciliary body rather than to the ciliary ganglion. As a result of the number of fibers dedicated to each from the ciliary ganglion, recovery of innervation to the lens controlling function is more likely than recovery of the pupillary reactive function. One source noted also that reinnervation may be slow, segmental and irregular, similar to the ocular symptoms themselves.

Prevention

No preventative measures were noted.

BOOKS

Bates, Barbara. A Pocket Guide to Physical Examination and History Taking. Philadelphia: Lippincott, 1991.

Mareib, Elaine N. Essentials of Human Anatomy and Physiology, 3rd ed. Benjamin/Cummings, 1991. pp. 219

Pavan-Langston, Deborah. Manual of Ocular Diagnosis & Therapy, 4th ed. Little, Brown & Co., 1995, pp. 336.

Simon, Roger P., Michael J. Arnihoff, and David A. Greenberg. Clinical Neurology, 4th ed. Stamford: Appleton & Lange, 1999, pp. 145.

Tasman, M.D., William. Duane's Clinical Ophthalmology. 2.15. Philadelphia: Lippincott, 1992. pp. 12.

Vander, M.D., Arthur J., James H. Sherman, Ph.D., and Dorothy S. Luciano, Ph.D. Human Physiology, Mechanics of Body Function, 5th ed. McGraw Hill, 1990, pp. 310.

Vaughn, Daniel, Taylor Asbury, and Paul Riordan-Eva. General Ophthalmology, 15th ed. Stamford: Appleton & Lange, 1999, pp. 267.

OTHER

Enersen, Ole Daniel. Adie's syndrome. 1994-2001. [cited May 12, 2004]. <http://www.whonamedit.com/synd.cfm/1837.html>.

Genetic Information and Patient Services, Inc. (GAPS) "The Gaps Index." Adie Syndrome. [cited May 12, 2004]. <http://www.icomm.ca/geneinfo/adie.htm>.

World & Medicine—Ophthalmology. Encyclopaedia of Ophthalmology—Greatest Links' Collection. "Adie's syndrome." In Principles of Neurology, 6th ed., p.279. [cited May 12, 2004]. <http://wmed.narod.ru/w_ophth/diseases/o_motil/om_06.htm>.

Katherine E. Nelson, N.D.