Adie’s Syndrome Focus Presentation.ppt

1. Adie’s Syndrome Tonic Pupil

2. Objectives Define Epidemiology Etiology Symptoms Testing Treatment

3. Eye Anatomy Overview

4. Features One, or both, pupils become tonic Decreased deep tendon reflexes Otherwise asymptomatic Found in young women Exact cause is unknown Most are benign May follow eye trauma

5. 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

6. Etiology Tonicity caused by damage Parasympathetic postganglionic nerves from the ciliary body Affected sphincter sections No longer constrict properly Pupil remains dilated

7. Etiology

8. Symptoms Aniscorsia Poor Accommodation Trouble focusing Photophobia Decreased Deep Tendon Reflexes

9. Differential Diagnosis Ross Syndrome Sjogren’s Syndrome CDIP 3 rd Nerve Palsy Neurosyphilis Oculomotor Dysfunction Pharmacologically Induced

10. Diagnostic Tests Ophthalmologic Testing Slit Lamp Pilocarpine Drops Neurodiagnostic Studies Autonomic Reflex Screen (ARS) Thermoregulatory Sweat Test (TST) Neuroimaging

11. Diagnostic Tests - Slit Lamp Illuminated microscope allows for high magnification Segmental Palsy vermiform movements of iris

12. 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.

13. Diagnostic Tests - TST Left: Normal TST Results Compatible with Adie’s Syndrome Right: TST showing Segmental Anhidrosis Results Compatible with Ross Syndrome

14. Treatment If Asymptomatic: No treatment necessary If Symptomatic: Dark Lenses Corrective lenses Colored Contact Lenses Pilocarpine Drops

15. Prognosis Good Accommodative Paresis Improves over time Contraction to Light Remains poor

16. Case Study 24 y/o female Presented with Severe headache Right dilated pupil Nonreactive to light or accommodation History of: Generalized areflexia Anisocoria Innocent heart murmur Migraines Asthma Pt denied Visual disturbances Family history of headaches Numbness Tingling Weakness Nausea/vomiting

17. Case Study Tests Performed: Lumbar Puncture Head CT MRI with gad ARS Tests Recommended, But Not Performed: TST

18. Case Study

19. Case Study

20. Case Study Trace below showing reduced foot response QSART of patients right extremities

21. 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

22. Case Study Results Lumbar Puncture, CT, & MRI Normal ARS Abnormal Mild distal postganglionic sympathetic sudomotor impairment Postural tachycardia No evidence of widespread autonomic failure

23. Case Study Current Status Peripheral vision decreased Blurred spot in visual field Wearing sunglasses Trying 0.5% pilocarpine drops Right Pupil Poor reaction to light Slow constriction to near response Next follow-up not schedule

24. 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

25. Questions?