Adie syndrome (Adie pupil, Holmes-Adie's syndrome, Adie's tonic pupil, tonic pupil)
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Etiology
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
- 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
References
- ↑ Wikipedia: Adie syndrome http://en.wikipedia.org/wiki/Adie_syndrome
- ↑ Eggenberger ER eMedicine: Anisocoria http://emedicine.medscape.com/article/1219991-overview
- ↑ NINDS Holmes-Adie syndrome Information Page http://www.ninds.nih.gov/disorders/holmes_adie/holmes_adie.htm