Holmes-Adie syndrome

Holmes-Adie syndrome (also known as Adie syndrome or Adie tonic pupil syndrome) is a neurological disorder characterized by a tonically dilated pupil (usually unilateral) that reacts poorly to light but shows a slow, tonic constriction to prolonged near-focus effort (light-near dissociation), along with diminished or absent deep tendon reflexes. The condition results from damage to the postganglionic parasympathetic fibers innervating the pupillary sphincter and ciliary muscle, as well as impairment of spinal reflex arc neurons responsible for deep tendon reflexes. The exact cause of this neuronal damage is not fully understood, but it is thought to involve a viral or inflammatory process affecting the ciliary ganglion and dorsal root ganglia. The syndrome predominantly affects the nervous system and the eye. Patients typically present with a dilated pupil that constricts sluggishly or incompletely to direct light stimulation but demonstrates a more robust, albeit slow and tonic, response to accommodation. Over time, the affected pupil may become smaller than the unaffected pupil ("little old Adie's"). Blurred vision at near distances and photosensitivity may occur. The loss of deep tendon reflexes (particularly at the knee and ankle) is a hallmark feature, and some patients may also experience mild autonomic dysfunction such as abnormal sweating. The condition most commonly affects young women. Holmes-Adie syndrome is considered a benign condition that does not typically progress to cause serious disability. There is no cure, and treatment is primarily symptomatic. Dilute pilocarpine eye drops (0.1%) may be used to constrict the affected pupil and improve near vision symptoms. Reading glasses may help with accommodation difficulties. The diagnosis is often clinical, supported by pharmacological testing with dilute pilocarpine, which causes exaggerated constriction of the denervated pupil due to cholinergic supersensitivity. Patients should be reassured about the benign nature of the condition, though periodic follow-up is recommended.

Common questions about Holmes-Adie syndrome