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Holmes-Adie syndrome

ORPHA:454718· ICD-10 H57.0

Definition

A rare ophthalmic disorder characterized by the unilateral or bilateral occurrence of a tonic pupil (showing sectorial denervation of the sphincter pupillae, so that the pupil constricts poorly to light, while the response to near is present but abnormally prolonged), in association with the absence of deep tendon reflexes. In some patients, patchy hypo- or anhidrosis may also be present (a variant known as Ross syndrome). The condition typically occurs in young adults, with a female preponderance.

Prevalence
1-9 / 100 000
Inheritance
Not applicable
Age of onset
Adult