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Adie syndrome, often referred to as Adie tonic pupil, is a neurological condition that impacts the eye’s pupils. The deep tendon reflex in the leg is weak or nonexistent, and the pupil reacts slowly to changes in light. Sensation loss in the foot is a common side effect of the illness. Adie syndrome mainly affects adults between the ages of 20 and 40, and it is more prevalent in women than in men.

Adie Syndrome :‎ [MEDNOTES+MINDMAP]

Definition:

  • Adie syndrome is characterized by a slow or sluggish pupil response to light (pupillary light reflex), known as tonic pupil.
  • It can also cause reduced or absent deep tendon reflexes and abnormal sensations in the legs and feet (sensory ataxia).

Epidemiology:

  • Adie syndrome is rare, with an estimated prevalence of 1-3 cases per 100,000 individuals.
  • It is more common in women and usually affects people between the ages of 20 and 40.

Natural history:

  • Adie syndrome is a benign condition and does not typically progress or cause any long-term vision problems.
  • The tonic pupil may gradually improve over time, but it may take several years for the pupil to return to normal size and reactivity.

Classification:

  • Adie syndrome is classified as a type of tonic pupil, which is further divided into two subtypes:
    1. Adie tonic pupil: This type is characterized by the slow or sluggish pupil response to light, as well as reduced or absent deep tendon reflexes and abnormal sensations in the legs and feet.
    2. Holmes-Adie syndrome: This type is similar to Adie tonic pupil, but also includes decreased sweating (anhidrosis) on the affected side.
  • Risk Factors:

    • Age (more common in young adults)
    • Gender (more common in females)
    • Family history
  • Etiology/Causes:

    • Damage to the oculomotor nerve (CN III)
    • Viral infections (e.g. mumps, measles, rubella)
    • Traumatic brain injury
    • Multiple sclerosis
    • Lyme disease
    • Anterior ischemic optic neuropathy
    • Scleritis
  • Signs:

    • Pupillary dilatation (mydriasis)
    • Pupillary light-near dissociation (PLND)
    • Reduced pupillary reflex
    • Decreased accommodation (ability to focus on objects at different distances)
  • Symptoms:

    • Blurred vision
    • Light sensitivity
    • Difficulty focusing on objects at different distances
    • Headaches
  • Pathology/Pathogenesis:

    • Damage to the oculomotor nerve leads to reduced innervation of the iris sphincter muscle, resulting in dilated pupils and PLND.
    • The damaged nerve also causes decreased innervation of the ciliary muscle, leading to decreased accommodation.
    • The exact mechanism of how viral infections, traumatic brain injury, and other etiologies lead to Adie syndrome is unknown.
  • Diagnosis

    • Clinical examination of eye and pupil function
    • Testing for abnormal pupil responses to light and near vision
    • Neurophysiological tests (e.g. electromyography) to assess muscle tone and reflexes
  • Tests Required

    • Pupillary light reflex test
    • Near vision test
    • Neurophysiological tests
  • Associated with

    • Autoimmune disorders (e.g. multiple sclerosis)
    • Migraines
    • Traumatic brain injury
    • Previous ocular surgery
  • Similar diseases/syndromes

    • Horner’s Syndrome
    • Marcus Gunn Syndrome
    • Argyll Robertson Pupil
  • Treatment

    • Eye patches or glasses to improve near vision
    • Medications to improve pupil function (e.g. pilocarpine)
    • Physical therapy to improve muscle tone and reflexes
    • Surgery to repair damaged nerves (rarely necessary)

Dr. Arin Nandi

Passionate About Medical Science & Helping Future Doctors Achieve Top Ranks In Medical Exams. He is professionally a dentist as well as a public health expert from JIPMER working in govt.health department
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