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Adie syndrome (Adie pupil, Holmes-Adie's syndrome, Adie's tonic pupil, tonic pupil)
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
a) hyperopia due to accommodative paresis
b) photophobia
c) difficulty reading
- affected pupil is:
a) larger than the contralateral pupil
b) poorly reactive to light
c) 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
Special laboratory:
- slit lamp examination
a) sector palsy of the pupil
b) 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)
General
syndrome
eye disease (ophthalmopathy)
Database Correlations
OMIM 103100
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