Adie Syndrome


Adie pupil, Adie syndrome, Holmes-Adie syndrome, pupillotonia

How common is Adie syndrome?

In 80% of the cases the disease occurs unilaterally, in the further course of the disease it can develop on both sides. The very rare disease is observed significantly more frequently in women than in men and occurs annually in about 4.7 cases per 100,000 inhabitants. By definition, the unilateral onset is typical for an Adie syndrome.

A large, oval and usually irregularly shaped pupil is observed. There is segmental paralysis of spontaneous iris movements and a delayed reaction to near vision (delayed pupil dilation). The sharpness of focus in patients with Adie’s syndrome often complain of acute sensitivity to light and deterioration of vision in patients with Adie’s syndrome often complain of acute sensitivity to light and deterioration of vision in patients with Adie’s syndrome.

In addition, the reflexes of the legs may be completely absent, which is attributed to reduced monosynaptic wiring. The pupil is medium to wide in Adie syndrome and reacts to long exposure with slow, tonic constriction. Often a good response to post-fixation is observed, but in acute cases the close-up can also be slow, so that visual acuity improves with increasing close-up.

The lack of ability to narrow the pupil results in a loss of depth of field. When looking into the distance, the time required for the pupil to dilate again is classically longer in Adie syndrome. Pharmacodynamically, the Adie syndrome becomes apparent when a local application of 0.1% pilocarpine drops is made.

As a result, the affected pupil narrows significantly, but the pupil of the other eye hardly reacts at all. The pilocarpine test is diagnostically helpful especially in acute pupillotonia, where the light and close-up reaction is missing, because it clearly shows the presence of Adie syndrome. As an alternative to Adie syndrome, damage to the short ciliary nerves due to trauma or surgery may also be present, which must be clarified anamnestically.

The cause of most cases of Adie syndrome is unknown. An occurrence in temporal connection with systemic diseases such as varicella zoster virus diseases, cranial arterial inflammation, syphilis and Lyme disease has been observed. Adie syndrome has also been observed during the presence of lung cancer (bronchial carcinoma) and Hodgkin’s lymphomas.

Important is a neurological and ophthalmological (ophthalmologic) clarification and pharmacodynamic examination. In addition, the patient should be informed and, if necessary, carry an emergency identification card with him/her, which records the pupil differences for later medical examinations and can possibly prevent a misinterpretation (perioperative, craniocerebral trauma). Adie syndrome is a harmless disorder of the pupillary reflex that does not require treatment.

It is currently suspected to be a damage of the motor Ia-fibres, but the cause of the Adie-Syndrome is currently not exactly clear. An inflammatory process in the brain is conceivable, which is being discussed as the cause of the pupillotonia. A herpes simplex disease is also conceivable as a cause.