Adie syndrome

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Adie's syndrome
Other namesHolmes–Adie syndrome, Adie's tonic pupil, Holmes–Adie pupil
Bilateral mydriasis given the observational diagnosis Adie's pupils by an ophthalmologist
Pronunciation
SpecialtyOphthalmology Edit this on Wikidata

Adie syndrome, also known as Holmes–Adie syndrome, is a neurological disorder characterized by a tonically dilated pupil that reacts slowly to light but shows a more definite response to accommodation (i.e., light-near dissociation).[1] It is frequently seen in females with absent knee or ankle jerks and impaired sweating.

The syndrome is caused by damage to the

bacterial infection that causes inflammation, and affects the pupil of the eye and the autonomic nervous system.[1] It is named after the British neurologists William John Adie and Gordon Morgan Holmes, who independently described the same disease in 1931.[2]

Signs and symptoms

Adie syndrome presents with three hallmark symptoms, namely at least one abnormally dilated pupil (

cardiovascular abnormalities.[4]

Pathophysiology

Pupillary symptoms of Holmes–Adie syndrome are thought to be the result of a viral or bacterial infection that causes inflammation and damage to neurons in the

dorsal root ganglia of the spinal cord. Adie's pupil is supersensitive to ACh so a muscarinic agonist (e.g. pilocarpine) whose dose would not be able to cause pupillary constriction in a normal patient, would cause it in a patient with Adie's Syndrome. The circuitry for the pupillary constriction does not descend below the upper midbrain, henceforth impaired pupillary constriction is extremely important to detect as it can be an early sign of brainstem herniation.[1]

Diagnosis

Clinical exam may reveal sectoral paresis of the iris sphincter or vermiform iris movements. The tonic pupil may become smaller (miotic) over time which is referred to as "little old Adie's".

MRI scans may be useful in the diagnostic testing of focal hypoactive reflexes.[6]

Treatment

The usual treatment of a standardised Adie syndrome is to prescribe reading glasses to correct for impairment of the eye(s).

drug therapy.[1]

Prognosis

Adie's syndrome is not life-threatening or disabling.[1] As such, there is no mortality rate relating to the condition; however, loss of deep tendon reflexes is permanent and may progress over time.[1]

Epidemiology

It most commonly affects younger women (2.6:1 female preponderance) and is unilateral in 80% of cases.[5] Average age of onset is 32 years.[citation needed]

See also

References

  1. ^ a b c d e f g h i j "Holmes-Adie syndrome Information Page". National Institute of Neurological Disorders and Stroke. Archived from the original on 2007-10-16. Retrieved 2008-01-21.
  2. PMID 24533698
    .
  3. ISBN 978-0-683-40007-6
    .
  4. ^ "Adie syndrome". Genetic and Rare Diseases Information Center (GARD) – an NCATS Program. Archived from the original on 2021-03-19. Retrieved 2018-04-17.
  5. ^
    ISBN 978-0-443-06603-0
    .
  6. ^ "Diagnosis of Adie syndrome WrongDiagnosis.com". Retrieved 2008-01-21.

Further reading

External links

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