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Adie syndrome

AI overview

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.

Key points
  • Caused by damage to postganglionic fibers of parasympathetic innervation of the eye, usually by viral or bacterial infection causing inflammation
    source quote
    The syndrome is caused by damage to the postganglionic fibers of the parasympathetic innervation of the eye, usually by a viral or bacterial infection that causes inflammation
  • Named after British neurologists William John Adie and Gordon Morgan Holmes who independently described the disease in 1931
    source quote
    It is named after the British neurologists William John Adie and Gordon Morgan Holmes , who independently described the same disease in 1931.
  • Most commonly affects younger women with 2.6:1 female preponderance and is unilateral in 80% of cases
    source quote
    It most commonly affects younger women (2.6:1 female preponderance) and is unilateral in 80% of cases.
  • Average age of onset is 32 years
    source quote
    Average age of onset is 32 years.
  • Adie's pupil is supersensitive to acetylcholine
    source quote
    Adie's pupil is supersensitive to acetylcholine 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.
Symptoms
  • Three hallmark symptoms: abnormally dilated pupil (mydriasis) not constricting to light, loss of deep tendon reflexes, and abnormalities of sweating
    source quote
    Adie syndrome presents with three hallmark symptoms, namely at least one abnormally dilated pupil ( mydriasis ) which does not constrict in response to light, loss of deep tendon reflexes, and abnormalities of sweating.
  • Other signs may include hyperopia due to accommodative paresis, photophobia and difficulty reading
    source quote
    Other signs may include hyperopia due to accommodative paresis , photophobia and difficulty reading.
  • Some individuals may also have cardiovascular abnormalities
    source quote
    Some individuals with Adie syndrome may also have cardiovascular abnormalities .
Diagnosis
  • Clinical exam may reveal sectoral paresis of the iris sphincter or vermiform iris movements
    source quote
    Clinical exam may reveal sectoral paresis of the iris sphincter or vermiform iris movements.
  • Tonic pupil may become smaller (miotic) over time, referred to as 'little old Adie's'
    source quote
    The tonic pupil may become smaller (miotic) over time which is referred to as "little old Adie's".
  • Testing with low dose (1/8%) pilocarpine may constrict the tonic pupil due to cholinergic denervation supersensitivity
    source quote
    Testing with low dose (1/8%) pilocarpine may constrict the tonic pupil due to cholinergic denervation supersensitivity .
  • A normal pupil will not constrict with the dilute dose of pilocarpine
    source quote
    A normal pupil will not constrict with the dilute dose of pilocarpine.
  • CT scans and MRI scans may be useful in diagnostic testing of focal hypoactive reflexes
    source quote
    CT scans and MRI scans may be useful in the diagnostic testing of focal hypoactive reflexes.
Treatment
  • Usual treatment is to prescribe reading glasses to correct for impairment of the eye(s)
    source quote
    The usual treatment of a standardised Adie syndrome is to prescribe reading glasses to correct for impairment of the eye(s).
  • Pilocarpine drops may be administered as treatment and diagnostic measure
    source quote
    Pilocarpine drops may be administered as a treatment as well as a diagnostic measure.
  • Thoracic sympathectomy is definitive treatment of diaphoresis if not treatable by drug therapy
    source quote
    Thoracic sympathectomy is the definitive treatment of diaphoresis , if the condition is not treatable by drug therapy .
Red flags
  • Impaired pupillary constriction can be an early sign of brainstem herniation
    source quote
    impaired pupillary constriction is extremely important to detect as it can be an early sign of brainstem herniation.
Prognosis

Adie's syndrome is not life-threatening or disabling; however, loss of deep tendon reflexes is permanent and may progress over time.

AI-synthesized from the Wikipedia article “Adie syndrome”. Not medical advice. Verify source →
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 

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 postganglionic fibers of the parasympathetic innervation of the eye, usually by a viral or 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]

Adie syndrome presents with three hallmark symptoms, namely at least one abnormally dilated pupil (mydriasis) which does not constrict in response to light, loss of deep tendon reflexes, and abnormalities of sweating.[1] Other signs may include hyperopia due to accommodative paresis, photophobia and difficulty reading.[3] Some individuals with Adie syndrome may also have cardiovascular abnormalities.[4]

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 ciliary ganglion, located in the posterior orbit, that provides parasympathetic control of eye constriction. Additionally, patients with Holmes-Adie Syndrome can also experience problems with autonomic control of the body. This second set of symptoms is caused by damage to the dorsal root ganglia of the spinal cord. Adie's pupil is supersensitive to acetylcholine 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]

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".[5] Testing with low dose (1/8%) pilocarpine may constrict the tonic pupil due to cholinergic denervation supersensitivity.[1] A normal pupil will not constrict with the dilute dose of pilocarpine.[5] CT scans and MRI scans may be useful in the diagnostic testing of focal hypoactive reflexes.[6]

The usual treatment of a standardised Adie syndrome is to prescribe reading glasses to correct for impairment of the eye(s).[1] Pilocarpine drops may be administered as a treatment as well as a diagnostic measure.[1] Thoracic sympathectomy is the definitive treatment of diaphoresis, if the condition is not treatable by drug therapy.[1]

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]

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.[7]

  1. 1 2 3 4 5 6 7 8 9 10 "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. ↑ Siddiqui AA, Clarke JC, Grzybowski A (November 2014). "William John Adie: the man behind the syndrome" (PDF). Clinical & Experimental Ophthalmology. 42 (8): 778–84. doi:10.1111/ceo.12301. PMID 24533698.
  3. ↑ Stedman's Medical Dictionary (27th ed.). Lippincott Williams & Wilkins. 2000. 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. 1 2 3 Haines DE (2002). Fundamental Neuroscience, 2nd edition. Churchill Livingstone. ISBN 978-0-443-06603-0.
  6. ↑ "Diagnosis of Adie syndrome WrongDiagnosis.com". Retrieved 2008-01-21.
  7. ↑ Thompson, H S (1977). "Adie's syndrome: some new observations". Transactions of the American Ophthalmological Society. 75. American Ophthalmological Society: 587–626. PMC 1311565. PMID 613531.