The diagnosis of Adie’s tonic pupil is clinical, based on history and physical exam.
These fibers provide parasympathetic innervation to various muscles depending on the reflex involved. A similar process is felt to occur in the dorsal root ganglion of the spinal cord where pre- and post-ganglionic parasympathetic fibers synapse. Over time the neurons regenerate allowing for recovery of the near response. This denervation leads to sectoral palsy of the iris sphincter muscle, stromal thinning, and occasionally iris atrophy. The affected short ciliary neurons are postganglionic parasympathetic neurons which synapse within the ciliary ganglion and innervate the iris sphincter muscles. Histologic examination of patients with Adie’s Syndrome has shown a reduction of ciliary ganglion cells. These symptoms do not always occur simultaneously, and the onset of areflexia often occurs after the onset of the tonic pupil. Damage to the dorsal root ganglion leads to areflexia. Roughly 80% of cases are unilateral, but can become bilateral (4% chance per year). This damage leads to sectoral denervation of the iris sphincter muscle and poor or sluggish constriction of the pupil in bright light but with normal near response and slow redilation.
It is thought that either a bacterial or viral infection causes inflammation which damages neurons in the ciliary ganglion of the orbit as well as the dorsal root ganglion in the spinal cord. When paired with segmental anhidrosis it is termed Ross’ Syndrome.Īt this time, the underlying etiology of this disease is unknown.Īdie’s tonic pupil occurs in a 3:1 female to male ratio with an average age of onset of 32 years. The entity is named after William John Adie, an Australian neurologist who extensively described the features. “Adie’s Tonic Pupil, also known as Adie’s Syndrome or Holmes-Adie Syndrome, is a disorder of the autonomic nervous system characterized by mydriasis with poor or sluggish pupillary constriction in bright light with slow redilation and decreased deep tendon reflexes. An isolated unilateral mydriasis as the sole manifestation of an oculomotor nerve palsy is exceedingly rare.” 1Īdie Pupil is the most likely diagnosis with the lesion in the ciliary ganglion.Ī pupil dilated due to a 3rd nerve palsy will also be supersensitive to pilocarpine 0.125%, however other signs of a 3rd nerve palsy would be present (ptosis, reduced ocular movements, or ocular misalignment).įor more information, refer to page 1011 of the CONTINUUM article “Diagnostic Approach to Pupillary Abnormalities.” In this case, the denervation is likely etiologic, hence a diagnosis of Adie pupil. In the absence of associated ptosis or ocular motility deficits, a positive dilute pilocarpine test indicates postganglionic parasympathetic denervation of the iris. Following 1 drop placed in each eye, a positive response to dilute pilocarpine is either (1) the larger pupil constricts 0.5 mm more than the normal pupil or (2) the larger pupil becomes the smaller pupil. Within the first 1 or 2 weeks of its denervation, the iris sphincter will become supersensitive to cholinergic agents such as pilocarpine diluted to a concentration of 0.125% or less. The affected pupil is dilated and poorly reactive to light. “This patient has a right Adie tonic pupil.
Which of the following is the most likely location of the lesion? Upon local instillation of 0.125% pilocarpine, the larger pupil becomes smaller than the contralateral pupil. There is no diplopia and no evidence of extraocular muscle palsy. Examination shows absent right pupillary response to light stimulation of either eye. A 35-year-old woman is evaluated for a 2-week history of photophobia and awareness that her right pupil is larger than the left.
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