Joseph W. Schmitz

BASICS

DESCRIPTION

• Idiopathic, postganglionic parasympathetic denervation of the iris sphincter, followed by aberrant reinnervation, which results in segmental and sluggish pupillary reaction to light, pupillary light-near dissociation, and slow redilation after accommodation effort

• On initial presentation, 80% of cases are unilateral.

• 4% of unilateral cases per year become bilateral.

• Affected pupil is initially larger but then tends to undergo a gradual miosis becoming smaller than unaffected pupil over years.

EPIDEMIOLOGY

Incidence

Estimated at 4.7 per 100,000 per year

Prevalence

Estimated at 2.0 per 1,000

RISK FACTORS

• Approximately 70% female

• Mean age 32 years

PATHOPHYSIOLOGY

• Postganglionic parasympathetic denervation of iris sphincter

• Aberrant reinnervation of the iris sphincter by ciliary ganglion axons originally destined for ciliary body

ETIOLOGY

Idiopathic

COMMONLY ASSOCIATED CONDITIONS

• Holmes Adie syndrome-

– Tendon Areflexia

• Ross Syndrome-

– Holmes Adie syndrome

– Segmental hypohidrosis

DIAGNOSIS

HISTORY

• Incidental anisocoria

• Blurred near vision

• Photophobia

PHYSICAL EXAM

• Affected pupil constricts more with accommodation than with light and redilates slowly

• If unilateral, anisocoria that is greater in light than dark

• Segmental contraction of pupil sphincter that is best seen at slit-lamp

• Approximately 10% of patients may have no light reaction.

• Deep tendon reflexes to assess for Holmes Adie syndrome

• Relative accommodative weakness compared to contralateral eye

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