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Home , Adie syndrome, Miosis, Mydriasis

Journal of Cll/lical Neuro-ophtllJllmology 9(1): 43-45, 1989. RilVen Press, Ltd., New York

Miotic Adie's

Michael L. Rosenberg, M.D.

Two young adults, aged 24 and 31, had a long history of Adie's syndrome or, pupillotonia, is typically small, poorly reactive pupilS. There was no history of characterized by either unilaterally or bilaterally large pupils, and a review of old photographs confirmed enlarged pupils that are unresponsive to (1). 10 and 5 years, respectively, of . Both were found to have bilateral tonic pupils that were supersensitive to The diagnosis is made clinically by watching for a diluted . Although it is possible that they had tonic constriction to near stimulation followed by a an unusually early onset of bilateral Adie's syndrome tonic redilatation. with dilated pupils that was not noticed, it is suggested Two young adults are described who were noted that some patients might have primary miotic Adie's during routine examinations to have bilaterally mi­ pupils without ever passing through a mydriatic phase. Key Words: Adie's syndrome-Argyll Robertson pu­ otic pupils that were thought to be fixed to light. pils-Miosis. They were both referred for the evaluation of Ar­ gyll Robertson pupils. Evaluation revealed bilat­ eral tonic reactions to near stimulation in both pa­ tients, typical of Adie's tonic pupilS. The diagnosis of parasympathetic denervation was confirmed in both patients as their pupils constricted with di­ luted pilocarpine. The cases reinforce the principle that any regardless of size should be evalu­ ated 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 oph­ thalmologic 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 mea­ sured 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 . Stimulation with near targets caused a slow and tonic reaction that caused further miosis From the Neuro- Section, Departments of bilaterally to 1.5 mm, followed by a slow redilata­ and Surgery, Uniformed Services University of the Health Sciences, Bethesda, Maryland. tion after refixation at distance. One drop of 1110% Address correspondence and reprint requests to Dr. M. pilocarpine caused miosis to 1 mm in each pupil. Rosenberg, The Neuro-ophthalmology Section, Department of Ten percent caused dilation of each pupil. Neurology, Uniformed Services University of the Health Sci­ ences, Bethesda, MD 20814-4799. U.S.A. A review of the patient's old photographs dating Work of the U.S. government. Not subject to copyright in the from 10 years prior to his evaluation showed con­ United States. The opinions contained herein are those of the sistently small pupils. The serum fluorescent author and are not to be construed as reflecting the views of the U.S. Air Force, the Department of Defense or the Uniformed treponemal antibody absorption (FTA-ABS) test Services University of the Health Sciences. was negative. The final diagnosis was Adie's pu-

43 44 M. ROSENBERG pils that were atypical because of bilateral miosis, Any local pathologic condition within the without a prior history of . may involve the ciliary ganglia and thereby cause denervation of the pupil. Most commonly, these conditions are unilateral and are due to inflamma­ CASE 2 tory processes such as herpes, , or sarcoid­ A 31-year-old man was also referred for the eval­ osis. uation of asymptomatic but abnormal pupils. Dur­ Neuropathic tonic pupils are typically bilateral ing a yearly physical examination, the pupils were and may be related to any type of diffuse periph­ noted to be miotic and were thought to be fixed to eral or autonomic neuropathy. Syphilis, diabetes, light. There was no prior history of enlarged pu­ and Guillain -Barre syndrome have all been asso­ pils. In fact, during a routine evaluation 5 years ciated with this condition, as have several heredi­ earlier, the pupils were also noted to be unusually tary neuropathies such as Shy Drager and Charcot small. Marie Tooth . Complete evaluation results, including the The remaining 80% of patients with tonic pupils deep-tendon reflex results, were normal except for will have a syndrome similar to that of my patients the pupils. These were 3.5 mm bilaterally, irregu­ termed the idiopathic type, or true Adie's syn­ lar, and had no visible reaction using a transillu­ drome. These patients begin unilaterally, but at minator light. With the slit lamp however, small least 20% will develop the syndrome bilaterally. In asynchronous movements were noted with light a longitudinal study, Thompson found that the stimulation. Reaction near stimulation was tonic contralateral side will become involved at a rate of and resulted in constriction to 2.5 mm bilaterally. approximately 4% per year (5). Both pupils constricted briskly to 0.1 % pilocarpine These patients represent a unique variant of the to 2 mm and enlarged to 6 mm with 10% cocaine. idiopathic Adie's syndrome for several reasons. The serum FTA-ABS test was nonreactive. They are unusually young to have a bilateral syn­ drome. Although Adie's has been reported in chil­ dren, the average age of onset is approximately 32 DISCUSSION years. Seventy percent of the affected patients are It is not surprising that these patients were first women (5). More strikingly unusual in these pa­ considered to have Argyll Robertson pupilS. Both tients is the marked miosis with which the patients patients' pupils fit the classic description of small, initially appeared. irregular pupils with near light dissociation (2). It has been well described that an Adie's pupil, The reaction to near stimulation in Argyll Robert­ although initially enlarged, will get progressively son pupils, however, is normally brisk rather than smaller over the ensuing years (1,5). Typical of this tonic. Long-standing diabetics may also have progression was the first patient with tonic pupils small, poorly reactive pupils; however, neither of and areflexia described in English in 1906 and then these patients was diabetic (3). Furthermore, there reevaluated 27 years later. The pupils had changed was no evidence of peripheral neuropathy of any from 8 mm to 4mm (1). Loewenfeld and Thomp­ type. The serum FTA-ABS test was nonreactive in son, observing a large number of patients, noted a both cases, and neither patient had any other ev­ consistent tendency toward decreased size, typi­ idence of neurosyphilis. cally with a change on the order of 2-3 mm (1,5). In both cases, a clinical diagnosis of Adie's pu­ The cause of the progressive miosis in a typical pillotonia was made by noting the tonic reaction to Adie's syndrome is unclear. Additional sympa­ near stimulation. The diagnosis was confirmed in thetic defects have been proposed, although test­ each by pharmacologic testing. The pathophysio­ ing with cocaine has not revealed evidence of such logic condition of such patients results in abnor­ a defect. The examination in our two patients malities involving the in the orbit added no clues to the causes of the miosis. and a decrease in the parasympathetic input to the Current thought regarding Adie's pupils would sphincter. Because these cells are third-order favor the hypothesis that the patients both had parasympathetic neurons, the iris sphincter devel­ typical acquired Adie's pupils with initially dilated ops a denervation hypersensitivity and becomes pupils that proceeded to shrink over time to their supersensitive to agonists (4). current miotic state. There was no history of en­ A number of different problems have been asso­ larged pupils, although certainly these might have ciated with tonic pupils, and Thompson has been missed. grouped the patients into three categories that However, old photographs in the first case and a help to classify the different causes (5). prior examination in the second confirm that the

.· ..'.;1,,,11'101, V"I. 9, No.1, 1989 MIOTIC ADIE'S PUPILS 45 pupils had not changed during 10 or 5 years, re­ diagnosis at the bedside, as routine testing for the spectively. This supports the hypothesis that per­ reaction of the pupils to near stimulation is typi­ haps the pupils were primarily miotic, that is, be­ cally done for only a few seconds. Tonic pupils came tonic without ever passing through the more may have such a slow near reaction that the move­ typical mydriatic phase. This may be analogous to ments and the miosis may not be visible for 5-10 s, primary aberrant regeneration of the third nerve. causing the diagnosis to be missed. In that case, chronic compression results in com­ pensatory regenerative phenomena that prevent the axonal destruction and from being REFERENCES manifested. In Adie's syndrome, if the neuronS destined for the iris sphincter are affected more 1. Loewenfeld IE, Thompson HS. The tonic pupil; a re­ evaluation. AM JOphtha/mo/1967;63:46-87. slowly than usual, whatever causes the miosis may 2. Loewenfeld IE. The , 1869-1969. A progress more rapidly and result in miosis without critical survey of the literature. Sun' Ophtha/mol prior mydriasis. 1969;14:199-299. 3. Sigsbee B, Torkelson B, Kadis G, Wright JW, Reeves AG. No matter what the etiology of the miosis, these Parasympathetic denervation of the iris in diabetes melli­ cases demonstrate that the diagnosis of tonic pu­ tus. JNeurol Neurosurg Psychiatr 1974;37:1031-5. pils should be considered in any patient with pu­ 4. Harriman DGF, Garland H. The pathology of Adie's syn­ drome. Brain 1968;91:20-418. pils that are poorly reactive to light, whether they 5. Thompson HS. Adie's syndrome: some new observations. are large or small. It is important to consider this Trans Am Ophthalmo/ Soc 1977;75:587~26.

1Clin Neuro-ophthalmol, Vol. 9, No.1, 1989