The number of parasympathetic axons devoted to the innervation of the ciliary body for accommodation in humans is about 30 times the number devoted to pupillary function. As a result of damage to the dorsal root ganglion of the spinal cord, many patients also experience problems with autonomic control of the body. Pupillary symptoms result from damage to the postganglionic parasympathetic supply innervating the ciliary body and iris which first travel as preganglionic fibers along with the oculomotor nerve to synapse at the ciliary ganglion within the orbit. The pathophysiology of Adie syndrome involves damage to the ciliary ganglion most commonly by an inflammatory process. 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. Īdie 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. 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.
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