J Am Board Fam Pract: first published as 10.3122/jabfm.10.6.439 on 1 November 1997. Downloaded from Adie Syndrome: A Case Report

T. Micheal Sherrill, MD, and DavidJ Lutz

In 1932 Adie 1 described a syndrome in which were roughly equal, and the right corneal reflex unilateral and pupillary contrac­ was decreased. A rapid plasma reagin was ob­ tion were abnormal. It typically was observed in tained and found to be negative. The patient was young women with no history or evidence of subsequently referred to an ophthalmologist for syphilis and involved a unilateral dilated confirmation of the diagnosis and to rule out that was unresponsive to light. He noted that other abnormalities. Confirmation was per­ deep tendon reflexes were absent or markedly di­ formed using'a dilute solution of . minished in these patients. He concluded that it was "a benign disorder sui generis." We report a Discussion recent case of Adie syndrome. Patients with Adie syndrome have characterized by a dilated pupil that is minimally Case Report reactive or unresponsive to light. Deep tendon A 37-year-old woman came to the office with reflexes (knees and ankles) are either diminished right pupil dilatation. It was noticed by a nurse or absent.2 This disorder is most often seen in for the first time earlier that morning and had not young women, who have an average age of onset been present previously according to the patient's in their 30s. It usually occurs in one eye, but oc­ report. The patient's only visual complaint was curs bilaterally in up to 4 percent of patients.3 mild in sunlight. She also had com­ Ciliary muscle denervation causes accommoda­ plaints of sinus congestion, headaches, and inter­ tion impairment, while sphincter pupillae dener­ mittent mucopurulent rhinorrhea believed to be vation causes . The resulting anisocoria due to an exacerbation of seasonal allergies. She leads to photophobia in bright light, impaired denied fever, extremity weakness, paresthesias, or dark adaptation, and a cosmetic defect.3 Head­ a history of sexually transmitted diseases. The pa­ aches and blurring of vision are also common. It tient had a remote history (more than 10 years has been suggested that the deep tendon hypore­ ago) of an episode ofleft arm and facial weakness, flexia could be a dysfunction of somatosensory

which resolved. large-diameter afferent fibers at the spinal level. http://www.jabfm.org/ On a physical examination she had anisocoria Neural losses of the lumbosacral dorsal root gan­ with a 5-mm right pupil and a 3-mm left pupil. glia and dorsal columns have been described.4 Pupillary light response was absent on the right The differential diagnosis of anisocoria with a and decreased accommodation was present. Ex­ dilated pupil includes sphincter muscle damage, traocular movements were intact bilaterally. third nerve palsy, severe intracranial pathology, Findings on a fundal examination were normal, unilateral use of a mydriatic, physiologic anisoco­ on 28 September 2021 by guest. Protected copyright. and her blood pressure was 128/84 mmHg. No ria, Horner syndrome, , other abnormalities were noted. Magnetic reso­ and Adie (tonic) pupil. Adie pupil results from a nance imaging (MRI) was ordered and findings partial denervation of the postganglionic para­ were normal except for sinusitis. sympathetic neurons of the sphincter pupillae Findings on a physical examination the follow­ muscle and the ciliary muscle.3,5 Although most ing day were unchanged. Examination of the ex­ cases are thought to be a result of a viral infection tremities showed absent ankle or knee reflexes on in the ,6 ganglion damage can also the ipsilateral (right) side, with decreased (1 +) result from trauma, tumors, and other infectious contralateral reflexes. Brachioradialis reflexes agents. Vascular compromise and ischemia from quinine, giant cell arteritis, and reversible is­ chemia from migraine headaches have also been Submitted, revised, 8 July 1997. 6 From a private family practice, Raleigh, NC. Address reprint described. Other causes include idiopathic dis­ requests to T. Micheal Sherrill, MD, Lakeside Family Physicians, ease, orbital trauma or infection, herpes zoster, 16627 Birkdale Commons Pkwy, Huntersville, NC 28031. diabetes mellitus, autonomic neuropathies, and

Adie Syndrome 439 J Am Board Fam Pract: first published as 10.3122/jabfm.10.6.439 on 1 November 1997. Downloaded from

Guillain-Barre syndrollle'? Adie pupil is thought an improvement in accommodation, which does to represent mild dysfunction of the autonomic not always return to normal because of only par­ nervous system. Other entities can involve more tial regeneration of the damaged neurons. The widely spre,ld and troublesome autonomic dys­ affected pupil might become smaller than the function. Ross syndrome involves tonic , al­ normal pupil with time. If the diagnosis is estab­ tered deep tendon reflexes, and subsequential hy­ lished with certainty, follow-up is routine'? pohidrosis.H Acute pandysautonomia and Fisher syndrome are more complicated autonomic ill­ References nesses that can also manifest tonic pupils. 1. Adie W. 'Ionic pupils and absent tendon reflexes: a The appropriate workup for anisocoria begins henign disorder sui generis; its complete and incom­ with the history. It is vital to ask the patient when plete forms. Brain 1932;55:98-113. the symptoms began, whether trauma was in­ 2. Scheinberg IH, Adler Rl. Adie's syndrome: case re­ volved, whether drops or ointments were used, port. Ann Ophthalmol 1979;11:247-8. and whether there i<; a history of syphilis or 3. Tonic pupil. In: Newell F. : principles and concepts. 6th ed. St. Louis: Mosby Year Book, headaches. A tumor, intracranial hemorrhage, or 1986:281. other midbrain abnormality must be nIled out by 4. Pavesi G, Macaluso GM, Medici D, Ventura P, Lus­ computed tomography or MRU The physical ex­ vardi M, Gemignani I<~ et al. On the cause of tendon amination first includes a determination of the af­ areflexia in the Holmes-Adie syndrome. Electro­ fectecl pupil. The affected pupil is usually the myogr Clin Neurophysiol 1994;34: 111-5. larger one in bright light and the smaller one in 5. Unilateral dilated pupil. In: Albert DM, ]akohiec FA, editors. Principles and practice of ophthalmology: dim light. Evaluate the pupil margin with a slit clinical practice. Philadelphia: WB Saunders, 1994; lamp for irregularity, to rule out , and to test 4:2476-77. ocular motility. Clinical diagnosis can be made by 6. Purvin VA. Adie's tonic pupil secondary to migraine.] demonstrating the denervation hypersensitivity NeuroophthalmoI1995;15:43-4. of the pupil to parasympathomimetic agents such 7. Allie's tonic pupil. In: Friedberg R. Wills Eye Hospi­ as 0.125 percent pilocarpineY When such an tal office and emergency room diagnosis and treat­ agent is instilled onto the affected eye, the tonic ment of . Philadelphia: ]B Lippincott, 1990:23 3-39. pupil will noticeably constrict. 10 If the patient is 8. Caparros-Lefebvre D, Hache ]C, Hurtevent ]F, younger than 1 year, a pediatric neurologic con­ Dereeper 0, Bille F, Petit H. Unilateral loss of facial sultation is necessary to nIle out familial dysau­ flushing and sweating with contralateral anhidrosis: tonomia (Riley-Day syndrome).H or Adie's syndrome? Clin Auton http://www.jabfm.org/ The condition is benign, and there is no effec­ Res 1993;3:239-41. tive treatment. Miotic agents such as pilocarpine 9. Younge BR, Buski Z]. 'ionic pupil: a simple screening 0.125 percent applied three times a dayll have test. Can] Ophthalmol 1976; 11 :295-9. been used to reduce the anisocoria in some pa­ 10. Cohen DN, Zakov ZN. The diagnosis of Ad ie's pupil using O.0625'YC, pilocarpine solution. Am] Ophthal­ tients, but side effects including ciliary muscle mol 1975;79:883-5. 3 spasm have been reported. Currently there are 11. Flach A], Dolan B]. Adie's syndrome: a medical treat­ on 28 September 2021 by guest. Protected copyright. no treatment guidelines established in the litera­ ment for symptomatic patients. Ann Ophthalmol ture. Within a few weeks of onset, there is often 1984;16:1151-4.

440 JABFP Nov.-Dcc.1997 Vol. 10 No.6