Adie’s Syndrome

Tonic Pupil

Objectives

●Define ●Epidemiology ●Etiology ●Symptoms●Testing●Treatment

Eye Anatomy Overview

Features

●One, or both, pupils become tonic●Decreased deep tendon reflexes●Otherwise asymptomatic●Found in young women●Exact cause is unknownoMost are benignoMay follow eye trauma

Epidemiology

●4-7:100,000●70% Women●20-40 y/o●Familial occurrence●Otherwise Healthy●80% start unilaterally●50-90% have decreased deep

tendon reflexes

Etiology

●Tonicity caused by damageoParasympathetic postganglionic

nerves from the ciliary body

●Affected sphincter sections oNo longer constrict properlyoPupil remains dilated

Etiology

Symptoms

●Aniscorsia●Poor AccommodationoTrouble focusing

●Photophobia●Decreased Deep Tendon

Reflexes

Differential Diagnosis

●Ross Syndrome●Sjogren’s Syndrome●CDIP●3rd Nerve Palsy●Neurosyphilis●Oculomotor Dysfunction●Pharmacologically Induced

Diagnostic Tests

●Ophthalmologic TestingoSlit LampoPilocarpine Drops

●Neurodiagnostic StudiesoAutonomic Reflex Screen (ARS)oThermoregulatory Sweat Test (TST)oNeuroimaging

Diagnostic Tests - Slit Lamp

●Illuminated microscopeoallows for high

magnification

●Segmental Palsyovermiform

movements of iris

Diagnostic Tests - Pilocarpine Drops

●12 yr old with recent onset of dilated right pupil.

●Top: Before instillation of 0.125% pilocarpine drops.

●Bottom: 30 minutes after instillation of 0.125% pilocarpine drops.

Diagnostic Tests - TST

●Left: Normal TSToResults Compatible with Adie’s Syndrome

●Right: TST showing Segmental AnhidrosisoResults Compatible with Ross Syndrome

Treatment

●If Asymptomatic:oNo treatment necessary

●If Symptomatic:oDark LensesoCorrective lensesoColored Contact LensesoPilocarpine Drops

Prognosis

●Good

●Accommodative ParesisoImproves over time

●Contraction to LightoRemains poor

Case Study

●24 y/o female●Presented with

oSevere headacheoRight dilated pupil

Nonreactive to light or accommodation

●History of:oGeneralized

areflexiaoAnisocoriaoInnocent heart

murmuroMigrainesoAsthma

●Pt deniedoVisual disturbancesoFamily history of

headachesoNumbnessoTinglingoWeaknessoNausea/vomiting

Case Study

●Tests Performed:oLumbar PunctureoHead CToMRI with gadoARS

●Tests Recommended, But Not Performed:oTST

Case Study

Case Study

Case Study

Trace below showing reduced foot response

QSART of patients right extremities

Absent QSART responses on the Left in 46 y/o Male with Ross Syndrome & B/L Adie’s pupils

QSART on the Right showing reduced forearm sweating and near absent sweating elsewhere

Case Study

●ResultsoLumbar Puncture, CT, & MRI

Normal

oARS Abnormal

Mild distal postganglionic sympathetic sudomotor impairment

Postural tachycardia No evidence of widespread autonomic failure

Case Study

●Current StatusoPeripheral vision decreasedoBlurred spot in visual fieldoWearing sunglasses oTrying 0.5% pilocarpine dropsoRight Pupil

Poor reaction to light Slow constriction to near response

oNext follow-up not schedule

Review

●Uncommon, idiopathic●Pupil(s) become tonic●Seen in young women●Otherwise asymptomatic●Cholinergic Supersensitivity

testing most common●No treatment needed if

asymptomatic●Good prognosis

Questions?