The incidence of Adie syndrome is approximately 4.7/100,000 population/year with a prevalence of two cases/1000 population (approximately). Young adults usually between the ages of 25 to 45 (mean age of 32 years) are most commonly affected. This is thought to occur due to a direct invasion of the ciliary nerves or ganglion by the virus due to its neurotropism, or due to a delayed immune-mediated mechanism. Recently, 2 cases of Adie syndrome associated with severe acute respiratory syndrome coronavirus 2 infection (SARS-CoV-2) have been reported. An association with familial dysautonomia has also been reported. It may also be caused by retinal photocoagulation that results in damage to the ciliary nerves within the suprachoroidal space. EtiologyĪdie syndrome is mostly idiopathic with no identifiable cause but may rarely be caused by local disorders involving the orbit that affect the ciliary ganglion including infections such as syphilis, varicella, human parvovirus-B19, human immunodeficiency virus, and Lyme disease, ischemia due to lymphomatoid granulomatosis, migraine, and giant cell arteritis, autoimmune disorders such as Sjogren syndrome, polyarteritis nodosa, sarcoidosis, systemic lupus erythematosus, amyloidosis, Guillain-Barre syndrome, and Vogt-Koyanagi-Harada disease, cardiovascular disorders, local or general anesthesia, orbital or choroidal tumors, orbital surgery for orbital floor fractures, neuromuscular conditions such as Lambert-Eaton myasthenic syndrome, and paraneoplastic in association with anti-Hu antibodies. It can be associated with hypohidrosis, in this case, it is known as Ross syndrome. The patient tends to have progressive miosis, bilateral affection (4% each year), and progressive loss of deep tendon reflexes. The symptoms result from autonomic disturbances, affecting vasomotor and sudomotor functions. It has a female preponderance with absent or reduced deep tendon reflexes. In 1914, Oloff had shown that tonic pupils could be caused by factors other than syphilis.Īdie syndrome is a relatively common neurological disorder of unknown etiology comprising unilateral or bilateral tonically dilated pupils with light-near dissociation and tendon areflexia. Earlier in 1881, Hughlings Jackson had described mydriasis with pupillary paralysis, while in 1906, Markus first described the tonic pupil. They both reported the condition in 1931, where Adie named it Pseudo-Argyll Robertson pupil. Summarize the importance of collaboration and communication amongst interprofessional team members to enhance care coordination for patients with Adie syndrome.Īdie 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.Outline the evaluation of Adie syndrome.Describe the pathophysiology of Adie syndrome.Review the potential etiologies of Adie syndrome that should be ruled out when Adie syndrome is present. This activity outlines the evaluation and management of Adie syndrome and highlights the role of the interprofessional team in treating patients with this condition. Adie syndrome, also known as the Holmes-Adie syndrome, is a neurological disorder of unknown etiology comprising unilateral or bilateral tonically dilated pupils with light-near dissociation and tendon areflexia.
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