Adie Syndrome

Adie Syndrome (Holmes-Adie Syndrome; Markus Syndrome; Saenger
Syndrome; Tonic Pupil; Iridoplegia Interna; Myotonic Pupil; Pseudotonic
Pupillotonia; Weill-Reys Syndrome; Ross Syndrome) 30

General: Cause unknown; more frequent in females; manifested in second and third decades; abnormal sensitivity to 2.5% solution of methacholine; segmental compensatory hyperhidrosis; tonic pupil constricts, whereas normal pupils are unaffected; tonic pupil, hyporeflexia, and segmental hypohidrosis are manifestations of Ross syndrome.

Ocular: Slightly enlarged pupils; delayed or diminished direct and consensual reaction to light; usually unilateral; consensual reflex is abolished on the affected side but normal on the other; amblyopia.

Clinical: Loss of tendon reflexes, particularly ankle and knee jerk (partial or total).

Adie W. Complete and incomplete forms of the benign disorder characterized by tonic pupils and absent tendon reflexes. Br J Ophthalmol 1932; 16:449.

Agbeja AM, Dutton GN. Adie's syndrome as a cause of amblyopia. J Pediatr Ophthalmol Strabismus 1987; 24:176.

Fenichel GM. Tonic pupil syndrome (Adie syndrome). In: Fenichel GM, ed. Clinical pediatric neurology, 2nd ed. Philadelphia: WB Saunders, 1993:336.

Loewenfe1d IE, Thompson HS. The tonic pupil: a reevaluation. Am J Ophthalmol 1967; 63:46.

Magalini SI, Scrascia E. Dictionary of medical syndromes, 2nd ed. Philadelphia: JB Lippincott, 1981.

Reinauer S, et al. Ross syndrome: treatment of segmental compensatory hyperhidrosis by a modified iontophoretic device. J Am Acad Dermatol 1993; 28[2 Pt 2]:308-312.