Adie’s Syndrome

Abstract

Adie’s syndrome is characterized by two features: a tonic pupil and absent tendon reflexes. This condition has been recognized by neurologists for many years, but there has never been an adequate explanation of the etiology and pathogenesis. In 1902, Saenger¹ and Strasburger² simultaneously described the condition. Since this description, the syndrome has been given various names, such as the tonic pupillary reaction, pupillotonia, myotonic convergence reaction, and tonic convergence of pupils apparently inactive to light. In 1931 Adie³ gave an adequate and concise description of the condition. From a study of twenty-two cases, he found that these cases seemed to fall into two definite groups: (1) The complete form with a typical tonic pupil and absence of tendon reflexes, and (2) the incomplete forms which he further subdivided into (a) the tonic pupil alone, (b) atypical phases of the tonic pupil alone (“iridoplegia,” “internal ophthalmoplegia”), (c) atypical phases of the tonic pupil with absent reflexes, and (d) absent reflexes alone.

In Adie’s syndrome the dilatation of the pupil is unilateral and it does not respond to light when the usual methods are used for eliciting the light reflex. After the patient has been placed in a darkened room for a long period of time, the pupils become equal in size. If a bright light is flashed into the affected pupil following this procedure, it responds slowly. As soon as the light is removed, the pupil returns to its original size. The most important feature of the tonic pupil is that. . .