Adie Syndrome

From Anvita Health Wiki

Contents 1 Etiology 2 Epidemiology 3 Pathology 4 Genetics 5 Clinical-manifestations 6 Diagnostic-procedures 7 Radiology 8 Management 9 More General Terms 10 Internet Database 11 References

Etiology

• inflammation from viral infection or bacterial infection
• Charcot-Marie-Tooth disease type 2J

Epidemiology

• affects younger women & is unilateral in 80% of cases

Pathology

• benign condition, not life threatening or disabling
• loss of deep tendon reflexes is permanent & may progress over time
• affects the pupil of the eye & the autonomic nervous system
• damage to the postganglionic fibers of the parasympathetic innervation of the eye

Genetics

• associated with defects in MPZ gene ( Charcot-Marie-Tooth disease type 2J)

Clinical-manifestations

• anisocoria
• loss of deep tendon reflexes
• diaphoresis
• variable findings

• hyperopia due to accommodative paresis
• photophobia
• difficulty reading

• affected pupil is:

• larger than the contralateral pupil
• poorly reactive to light
• vigorously reactive to near stimuli

• near response in an Adie pupil is tonic, thus miosis persists longer in the affected eye with slow redilation after removal of the near stimulus

Diagnostic-procedures

• slit lamp examination

• sector palsy of the pupil
• vermiform movements of the iris often are visible

• Adie pupil typically reacts to weak pilocarpine solution (1/8-1/16%) due to cholinergic denervation supersensitivity; a normal pupil does not react
• acute onset Adie pupil may not constrict with dilute pilocarpine

Radiology

• computed tomography or MRI scans may be useful for evaluation of focal hypoactive reflexes

Management

• reading glasses
• pilocarpine drops TID
• thoracic sympathectomy for intolerable diaphoresis not responsive to drug therapy (see perspiration)

More General Terms

• syndrome
• eye disease (ophthalmopathy)

Internet Database

OMIM: 103100

References

1. Wikipedia: Adie syndrome [1]
2. Eggenberger ER eMedicine: Anisocoria [2]
3. NINDS Holmes-Adie syndrome Information Page [3]

Adie syndrome (Adie pupil, Holmes-Adie's syndrome, Adie's tonic pupil, tonic pupil)