- Unilateral (80% cases)
Adie syndrome, also called the Holmes-Adie Syndrome, is named after William John Adie, the British neurologist of Australian descent, and Sir Gordon Morgan Holmes, an Irish neurologist. They both reported the condition in 1931, where Adie named it Pseudo-Argyll Robertson pupil. Earlier in 1881, Hughlings Jackson had described mydriasis with pupillary paralysis, while in 1906, Markus first described the tonic pupil. In 1914, Oloff had shown that tonic pupils could be caused by factors other than syphilis.
- Idiopathic (M/C)
Local disorders within the orbit affecting the ciliary ganglion:
- Infection (syphilis, varicella, human parvovirus (B19) infection, Lyme disease)
- Ischemia (lymphomatoid granulomatosis, migraine, giant cell arteritis)
- Autoimmune disorders (Sjogren syndrome, polyarteritis nodosa, sarcoidosis, amyloidosis, Guillain-Barre syndrome, Vogt-Koyanagi-Harada disease)
- Cardiovascular disorders (carotid dissection)
- Local/general anaesthesia
- Orbital/choroidal tumour
- Orbital surgery or laser therapy
- Orbital floor fracture
- Paraneoplastic syndrome (Lambert-Eaton myasthenic syndrome, congenital neuroblastoma with Hirschsprung disease and central hypoventilation syndrome, anti-Hu antibodies)
Adie pupil:Strong and tonic response to near stimulation with a slow and sustained relaxation due to iris sphincter aberrant regeneration and hypersensitivity to muscarinic receptor agonists (e.g., pilocarpine).
- At least one mydriatic pupil with poor or absent pupillary light reaction
- Tonic pupillary near response with light-near dissociation
- Decreased/loss of deep tendon reflexes (M/C achilles tendon reflex)
- Abnormalities of sweating (Ross variant)
- Difficulty reading due to hyperopia (accommodative paresis), segmental palsy of the sphincter, photophobia, cholinergic supersensitivity of the denervated muscles
- Cardiovascular abnormalities (orthostatic hypotension)
Adie pupil:Strong & tonic response of the near reaction with a slow & sustained relaxation
- Due to iris sphincter hypersensitivity to muscarinic receptor agonists (e.g. pilocarpine).
Little old Adie pupil:Tonic pupil, usually larger than the uninvolved fellow eye, tends to become smaller over time
- Can be diagnosed by the poor light reaction and the tonic near response
Low-Dose pilocarpine test:Demonstrate cholinergic denervation supersensitivity (80% cases) in the tonic pupil
Differential diagnosis:Systemic autonomic neuropathies: Can also affect the ciliary ganglion and produce the tonic pupil
- Ross syndrome (characterized by a triad of a tonic pupil, hyporeflexia, and segmental anhidrosis)
- Harlequin syndrome (characterized by the unilateral decreased/absence of flushing and sweating, particularly in the face, neck, arm, and chest in response to heat, exercise, or emotional factors)
- Idiopathic Adie syndrome (no treatment required)
- Underlying systemic cause should have treatment directed at their other autonomic neuropathies.
- Impairment of the eyes (due to accommodative paresis): Reading glasses
- Topical low-dose pilocarpine or physostigmine drops (for treatment as well as diagnosis)
- Failure of conservative management: Thoracic sympathectomy
- Does not have a progressive course
- Not a life-threatening condition
- Does not cause disabilities
- Not associated with any mortality rate
- Loss of deep tendon reflexes is permanent and may progress over time.