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Show Journal of Cll/ lical Neuro- ophtllJllmology 9( 1): 43- 45, 1989. Miotic Adie's Pupils Michael L. Rosenberg, M. D. Two young adults, aged 24 and 31, had a long history of small, poorly reactive pupilS. There was no history of large pupils, and a review of old photographs confirmed 10 and 5 years, respectively, of miosis. Both were found to have bilateral tonic pupils that were supersensitive to diluted pilocarpine. Although it is possible that they had an unusually early onset of bilateral Adie's syndrome with dilated pupils that was not noticed, it is suggested that some patients might have primary miotic Adie's pupils without ever passing through a mydriatic phase. Key Words: Adie's syndrome- Argyll Robertson pupils- Miosis. From the Neuro- ophthalmology Section, Departments of Neurology and Surgery, Uniformed Services University of the Health Sciences, Bethesda, Maryland. Address correspondence and reprint requests to Dr. M. Rosenberg, The Neuro- ophthalmology Section, Department of Neurology, Uniformed Services University of the Health Sciences, Bethesda, MD 20814- 4799. U. S. A. Work of the U. S. government. Not subject to copyright in the United States. The opinions contained herein are those of the author and are not to be construed as reflecting the views of the U. S. Air Force, the Department of Defense or the Uniformed Services University of the Health Sciences. 43 RilVen Press, Ltd., New York Adie's syndrome or, pupillotonia, is typically characterized by either unilaterally or bilaterally enlarged pupils that are unresponsive to light ( 1). The diagnosis is made clinically by watching for a tonic constriction to near stimulation followed by a tonic redilatation. Two young adults are described who were noted during routine examinations to have bilaterally miotic pupils that were thought to be fixed to light. They were both referred for the evaluation of Argyll Robertson pupils. Evaluation revealed bilateral tonic reactions to near stimulation in both patients, typical of Adie's tonic pupilS. The diagnosis of parasympathetic denervation was confirmed in both patients as their pupils constricted with diluted pilocarpine. The cases reinforce the principle that any pupil regardless of size should be evaluated for the possibility of pupillotonia. CASE 1 A 24- year- old man was referred for evaluation of Argyll Robertson pupilS. He had no history of ophthalmologic or neurologic problems. His general and neurologic examination results were normal, including all deep- tendon reflex results. Only the pupils were abnormal. In the dark, the right measured 3.0 mm and the left, 2.5 mm. There was no significant change in the light. Although no light reaction to direct stimulation was seen grossly, tonic asynchronous constriction was seen with the slit lamp. Stimulation with near targets caused a slow and tonic reaction that caused further miosis bilaterally to 1.5 mm, followed by a slow redilatation after refixation at distance. One drop of 1110% pilocarpine caused miosis to 1 mm in each pupil. Ten percent cocaine caused dilation of each pupil. A review of the patient's old photographs dating from 10 years prior to his evaluation showed consistently small pupils. The serum fluorescent treponemal antibody absorption ( FTA- ABS) test was negative. The final diagnosis was Adie's pu- 44 M. ROSENBERG pils that were atypical because of bilateral miosis, without a prior history of mydriasis. CASE 2 A 31- year- old man was also referred for the evaluation of asymptomatic but abnormal pupils. During a yearly physical examination, the pupils were noted to be miotic and were thought to be fixed to light. There was no prior history of enlarged pupils. In fact, during a routine evaluation 5 years earlier, the pupils were also noted to be unusually small. Complete evaluation results, including the deep- tendon reflex results, were normal except for the pupils. These were 3.5 mm bilaterally, irregular, and had no visible reaction using a transilluminator light. With the slit lamp however, small asynchronous movements were noted with light stimulation. Reaction near stimulation was tonic and resulted in constriction to 2.5 mm bilaterally. Both pupils constricted briskly to 0.1% pilocarpine to 2 mm and enlarged to 6 mm with 10% cocaine. The serum FTA- ABS test was nonreactive. DISCUSSION It is not surprising that these patients were first considered to have Argyll Robertson pupilS. Both patients' pupils fit the classic description of small, irregular pupils with near light dissociation ( 2). The reaction to near stimulation in Argyll Robertson pupils, however, is normally brisk rather than tonic. Long- standing diabetics may also have small, poorly reactive pupils; however, neither of these patients was diabetic ( 3). Furthermore, there was no evidence of peripheral neuropathy of any type. The serum FTA- ABS test was nonreactive in both cases, and neither patient had any other evidence of neurosyphilis. In both cases, a clinical diagnosis of Adie's pupillotonia was made by noting the tonic reaction to near stimulation. The diagnosis was confirmed in each by pharmacologic testing. The pathophysiologic condition of such patients results in abnormalities involving the ciliary ganglion in the orbit and a decrease in the parasympathetic input to the iris sphincter. Because these cells are third- order parasympathetic neurons, the iris sphincter develops a denervation hypersensitivity and becomes supersensitive to cholinergic agonists ( 4). A number of different problems have been associated with tonic pupils, and Thompson has grouped the patients into three categories that help to classify the different causes ( 5). . · ..'.; 1,,, 11' 101, V" I. 9, No. 1, 1989 Any local pathologic condition within the orbit may involve the ciliary ganglia and thereby cause denervation of the pupil. Most commonly, these conditions are unilateral and are due to inflammatory processes such as herpes, syphilis, or sarcoidosis. Neuropathic tonic pupils are typically bilateral and may be related to any type of diffuse peripheral or autonomic neuropathy. Syphilis, diabetes, and Guillain - Barre syndrome have all been associated with this condition, as have several hereditary neuropathies such as Shy Drager and Charcot Marie Tooth disease. The remaining 80% of patients with tonic pupils will have a syndrome similar to that of my patients termed the idiopathic type, or true Adie's syndrome. These patients begin unilaterally, but at least 20% will develop the syndrome bilaterally. In a longitudinal study, Thompson found that the contralateral side will become involved at a rate of approximately 4% per year ( 5). These patients represent a unique variant of the idiopathic Adie's syndrome for several reasons. They are unusually young to have a bilateral syndrome. Although Adie's has been reported in children, the average age of onset is approximately 32 years. Seventy percent of the affected patients are women ( 5). More strikingly unusual in these patients is the marked miosis with which the patients initially appeared. It has been well described that an Adie's pupil, although initially enlarged, will get progressively smaller over the ensuing years ( 1,5). Typical of this progression was the first patient with tonic pupils and areflexia described in English in 1906 and then reevaluated 27 years later. The pupils had changed from 8 mm to 4mm ( 1). Loewenfeld and Thompson, observing a large number of patients, noted a consistent tendency toward decreased size, typically with a change on the order of 2- 3 mm ( 1,5). The cause of the progressive miosis in a typical Adie's syndrome is unclear. Additional sympathetic defects have been proposed, although testing with cocaine has not revealed evidence of such a defect. The examination in our two patients added no clues to the causes of the miosis. Current thought regarding Adie's pupils would favor the hypothesis that the patients both had typical acquired Adie's pupils with initially dilated pupils that proceeded to shrink over time to their current miotic state. There was no history of enlarged pupils, although certainly these might have been missed. However, old photographs in the first case and a prior examination in the second confirm that the MIOTIC ADIE'S PUPILS 45 pupils had not changed during 10 or 5 years, respectively. This supports the hypothesis that perhaps the pupils were primarily miotic, that is, became tonic without ever passing through the more typical mydriatic phase. This may be analogous to primary aberrant regeneration of the third nerve. In that case, chronic compression results in compensatory regenerative phenomena that prevent the axonal destruction and diplopia from being manifested. In Adie's syndrome, if the neuronS destined for the iris sphincter are affected more slowly than usual, whatever causes the miosis may progress more rapidly and result in miosis without prior mydriasis. No matter what the etiology of the miosis, these cases demonstrate that the diagnosis of tonic pupils should be considered in any patient with pupils that are poorly reactive to light, whether they are large or small. It is important to consider this diagnosis at the bedside, as routine testing for the reaction of the pupils to near stimulation is typically done for only a few seconds. Tonic pupils may have such a slow near reaction that the movements and the miosis may not be visible for 5- 10 s, causing the diagnosis to be missed. REFERENCES 1. Loewenfeld IE, Thompson HS. The tonic pupil; a reevaluation. AM JOphtha/ mo/ 1967; 63: 46- 87. 2. Loewenfeld IE. The Argyll Robertson pupil, 1869- 1969. A critical survey of the literature. Sun' Ophtha/ mol 1969; 14: 199- 299. 3. Sigsbee B, Torkelson B, Kadis G, Wright JW, Reeves AG. Parasympathetic denervation of the iris in diabetes mellitus. JNeurol Neurosurg Psychiatr 1974; 37: 1031- 5. 4. Harriman DGF, Garland H. The pathology of Adie's syndrome. Brain 1968; 91: 20- 418. 5. Thompson HS. Adie's syndrome: some new observations. Trans Am Ophthalmo/ Soc 1977; 75: 587~ 26. 1Clin Neuro- ophthalmol, Vol. 9, No. 1, 1989 |
