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Objective: This study aimed to analyze the denervation and reinnervation history of individual segments of the iris sphincter in patients with Adie's syndrome. Design: The irises of these patients were retroilluminated by shining an infrared-rich light through the lower eyelid and sclera and viewing the transilluminated iris from the front with an infrared-sensitive video camera. The irises of the same group of patients also were videotaped through a slit-lamp camera using routine frontal illumination. Both of these techniques also were used to examine a series of normal subjects. Participants: A total of 61 patients with Adie's syndrome or Adie's-like denervation of the iris sphincter (from surgery, trauma, or radiation) and 10 normal subjects were studied. Main Outcome Measures: Slit-lamp examination results of the segmental movement of the iris were compared to the infrared transillumination pattern of the iris sphincter before and during the light reflex, before and during the near-vision reaction, before and during eye movement, and before and after the application of dilute pilocarpine and 1% pilocarpine. Results: Whenever an iris sphincter segment contracted, it also became denser by using these techniques. Reinnervated iris segments failed to contract to light but did contract and became denser with a near effort or with eye movement. Segments supersensitive to pilocarpine became denser than adjacent segments without supersensitivity, but atrophic-appearing segments looked translucent and thin, failing to become denser, even with 1% pilocarpine. Conclusions: The innervational history and current status of each clock-hour segment of the iris sphincter can be determined using this technique of videographic infrared transillumination, and the progression and pattern of iris denervation and reinnervation can be determined in patients with Adie's pupil. A miotic Adie's pupil that is "tonic," even in darkness, was found to be associated with a dense pattern of infrared transillumination in sphincter segments, which the authors interpret as being associated with a rich reinnervation from accommodative fibers. Segments that become densely reinnervated appear to lose their cholinergic supersensitivity.

William Lawrence was a leading English ophthalmic surgeon in the middle of the 19th century. This article briefly discusses his life, career, and well-known textbook (Treatise on Diseases of the Eye). His book and 3 others were the best-known English texts on diseases of the eye of the 1830s and 1840s. The 3 others were William Mackenzie's Practical Treatise on Diseases of the Eye, Richard Middlemore's Treatise on the Diseases of the Eye and Its Appendages, and Thomas Wharton Jones' Manual of the Principles and Practice of Ophthalmic Medicine and Surgery.

Perimetry and visual field testing have been used as clinical ophthalmic diagnostic tools for many years, and this manuscript will provide a brief historical overview of these procedures and the individuals who developed them. Today, we have many different forms of perimetry that are designed to evaluate different locations within the visual pathways and various mechanisms and subsets of mechanisms within the visual system. However, the most widely used method of performing perimetry and visual field testing has not substantially changed for more than 150 years, consisting of detecting a small target superimposed on a uniform background at different locations within the field of view. Although the basic test procedure has remained similar throughout the ages, there have been many advances in test administration, standardization, statistical evaluation, clinical analysis, interpretation, and prediction of outcome based on visual field findings.

Background and Methods. The use of corticosteroids to treat optic neuritis is controversial. At 15 clinical centers, we randomly assigned 457 patients with acute optic neuritis to receive oral prednisone (1 mg per kilogram of body weight per day) for 14 days; intravenous methylprednisolone (1 g per day) for 3 days, followed by oral prednisone (1 mg per kilogram per day) for 11 days; or oral placebo for 14 days. Visual function was assessed over a six-month follow-up period. Results. Visual function recovered faster in the group receiving intravenous methylprednisolone than in the placebo group; this was particularly true for the reversal of visual-field defects (P = 0.0001). Although the differences between the groups decreased with time, at six months the group that received intravenous methylprednisolone still had slightly better visual fields (P = 0.054), contrast sensitivity (P = 0.026), and color vision (P = 0.033) but not better visual acuity (P = 0.66). The outcome in the oral-prednisone group did ...

The authors developed an automated method of pupil perimetry by linking an infrared video pupillometer to a Humphrey Field Analyzer. Software was developed to automatically analyze the pupil responses to focal light stimuli and display the results graphically. All 76 locations of Humphrey program 30-2 could be tested twice within 5.5 minutes and the relative sensitivity of the field was determined by comparing the amplitude of pupil constriction or latency time at each stimulus location. The mean pupil responses within annular areas at 3 degrees, 9 degrees, 15 degrees, 21 degrees, and 27 degrees were shown to be linearly related to log stimulus intensity over a 15 dB range under low level mesopic conditions (3.15 asb bowl background). In normal subjects, the superior temporal quadrant usually had the greatest mean pupillomotor response and the inferior nasal quadrant had the least. Pupil responses in the temporal field were larger than corresponding locations in the nasal field. Patients with visual field defects who underwent testing by pupil perimetry showed pupillary deficits in the same location within the field, providing evidence that pupil perimetry may be a useful, objective means of assessing visual field function.

Hydroxyamphetamine eyedrops are used to help localize the lesion in Horner's syndrome. Because normal variability in the response to the eyedrops may influence the interpretation of test results in patients with Horner's syndrome, we studied both the interocular variability of the drug's mydriatic effect within each normal subject and the variation between individuals. We used photographs to document the variability among 26 normal subjects. Hydroxyamphetamine hydrobromide 1% eyedrops (Paredrine) were placed in both eyes of normal subjects in the same way that patients with Horner's syndrome are tested. The drug produced a mean increase in pupil size of 1.96 mm (+/- 0.61 S.D.) in the 52 eyes tested. In normal subjects, the mydriatic effect of hydroxyamphetamine was symmetric in each pair of eyes. The mean interocular asymmetry of mydriasis as measured by the difference in dilation (right eye dilation minus left eye dilation) was -0.087 mm (+/- 0.29 S.D.). Thus, the variability of hydroxyamphetamine mydriasis from one eye to the other in a single subject was much lower than the variability between subjects.

We studied hydroxyamphetamine hydrobromide 1% (Paredrine) mydriasis in 54 patients with Horner's syndrome to determine its effectiveness in distinguishing preganglionic lesions from postganglionic lesions. The difference in pupillary dilation between the unaffected and affected sides was used as a measure of the hydroxyamphetamine effect. We found that patients who had clinical evidence of damage to the postganglionic neuron of the oculosympathetic pathway had less pupillary dilation on the affected side. In contrast, almost all patients judged to have clinical evidence of preganglionic lesions dilated more on the affected side. We determined the probability that a given difference in pupillary dilation between the involved and uninvolved side is the result of a postganglionic lesion.

Publisher Summary At the very beginning of autonomic pharmacology, the pupil was frequently used as an indicator of drug action. Parasympathetic and sympathetic neural impulses to the iris muscles could be modified by drugs at the synapses and at the effector sites because it was at these locations that the transmission of the impulse depended on chemical mediators. The more pigment in the iris, the more slowly the drug takes effect and the longer its action lingers. This is probably because of the drug being bound to iris melanin and then slowly released. There are wide individual differences in pupillary responses to topical drugs. There is probably a greater range of responses among blue eyes than there is between the average response of blue eyes and the average response of dark brown eyes. Some of these individual differences are because of the corneal penetration of the drug.

We set ourselves to this project because it promised to be interesting and enjoyable. It has, in addition, turned out to be intensely instructive for us, and we sense that the job will remain permanently unfinished because we have, finally, had to make some coin-toss decisions about rounding out the collection to exactly 100 titles.

Cecil Starling O’Brien, M.D. (1889–1977), was the founder (1927) of the Department of Ophthalmology at the University of Iowa, and he is credited with the establishment of a strong department that has made many important contributions to ophthalmology. The surviving O’Brien residents were interviewed on videotape, and this article is a composite of their memories together with a brief biographical sketch of Dr. O’Brien.

Purpose: Because simple anisocoria is believed to decrease in bright light, the authors determined the prevalence of simple anisocoria under different lighting conditions. Methods: The authors measured the pupil size of 104 healthy subjects with infrared videography at four clinically accessible light levels: darkness; darkness with a hand-held light shining from below; room light; and room light with the hand light shining from below. Results: Of the 104 subjects, 40 (38%) were men and 64 (62%) were women. The ages ranged from 12 to 71 years (mean, 36.3 ± 12.5 years). The mean decrease in pupillary diameter from darkness to the brightest condition was 1.89 mm. Based on the traditional definition of a pupillary diameter difference of 0.4 mm or greater, the prevalence of simple anisocoria decreased from 18% in darkness to 8% in room light with the hand-held light shining from below. The prevalence of anisocoria varied considerably when other definitions were used. Repeated measures analysis of variance showed that pupillary area difference decreased with brighter conditions ( P = 0.026). However, the ratio of the pupillary areas did not change with brighter conditions ( P = 0.666). Conclusions: The prevalence of simple anisocoria decreases with brighter conditions based on pupillary diameter difference. However, this decrease is not apparent when anisocoria is expressed as pupillary area ratio. Those clinicians who measure pupils will find that simple anisocoria decreases in bright light. However, with gross observation where perception of an anisocoria may be related more to the ratio of the pupillary areas, simple anisocoria may not seem to change much with brighter conditions.

Purpose/Methods A 47-year-old man with a postganglionic Horner's syndrome and severe right facial pain was found to have a normal carotid angiogram. We obtained a magnetic resonance angiogram of the neck because of our continued clinical suspicion of dissection. Result/Conclusion An area of hyperintensity was identified along the lumen of the right internal carotid artery, suggesting a subadventitial dissection. Magnetic resonance angiography is a noninvasive, sensitive technique for identifying some carotid dissections.

This study was undertaken to understand better how damage to the anterior visual pathway may affect the relationship between the visual and pupillomotor systems. The relative afferent pupillary defect and the interocular difference in visual field mean deviation (determined by the Humphrey Field Analyzer Statpac program) were correlated in 137 patients. A moderate linear correlation (r = .66) was found. In 25 patients tested by both static and kinetic perimetry, the correlation could not be significantly improved by considering field loss outside of 30 degrees. The correlation was further studied in four subcategories of diagnosis: optic neuritis (n = 36), idiopathic intracranial hypertension (n = 26), compressive optic neuropathy (n = 14), and anterior ischemic optic neuropathy (n = 7). In compressive optic neuropathy and idiopathic intracranial hypertension, the difference in mean deviation between the two eyes was associated with a larger relative afferent pupillary defect than in optic neuritis and anterior ischemic optic neuropathy. In optic neuritis, the correlation was the poorest. These results indicate that diseases of the afferent visual system may not necessarily affect visual threshold (as tested by static perimetry) and the pupillary light reflex (a suprathreshold test) in the same way.

Color vision testing is an important part of the assessment of both retinal and optic nerve function. The Farnsworth-Munsell (FM) 100-hue test is widely accepted as a sensitive tool for detecting subtle deficits in hue discrimination. Originally designed in 1943 by Dean Farnsworth to measure color discrimination and to detect congenital dyschromatopsias, the test has also been used to assess a wide variety of acquired dyschromatopsias. The test requires the arrangement of 85 colored chips in consecutive color order. We have made two fundamental changes in the FM 100-hue which improve the test’s specificity and sensitivity for detecting color vision deficits. First, two chips were removed from the original test which results in a more even error score distribution for normals across the entire set of hues. Second, the addition of a white-to-black reliability test allows non-color-vision-related variables such as motivation and understanding to be assessed. In this paper, we present normative data for the revised 83-chip color vision test and the reliability test. We also outline our procedure for administering the FM 100-hue including a description of the illuminant C light source and our computerized barcode-based scoring system.

Two patients with seizure-associated miosis and ptosis are described. In both there are magnetic resonance imaging abnormalities of the temporal lobe. In one patient, increased magnetic resonance imaging signal intensity is present in the temporal lobe contralateral to ptosis and miosis. In the other, there is temporal lobe asymmetry with the smaller temporal lobe ipsilateral to the miotic pupil and ptotic lid. The relevant human and experimental literature related to cortical control of pupil size and lid movement is reviewed. Based on the available literature and the findings in these two patients, it is proposed that the increased signal intensity in the temporal lobe of one patient represents an irritative stimulus causing contralateral miosis and ptosis, whereas the temporal lobe hypoplasia in the second patient permitted impulses from the contralateral normal temporal lobe to predominate, resulting in miosis and ptosis homolateral to the hypoplastic temporal lobe. ( J Child Neurol 1990;5:142-146).

The relative afferent pupillary defect (RAPD) was measured with neutral density filters in ten patients, each of whom had a dense cataract in one eye only. In each instance, the cataract reduced visual acuity to counting fingers or worse, whereas visual acuity in the other eye was 20/25 or better. All patients with mature or nuclear cataracts had a measureable RAPD in the other eye (mean, 0.44 log unit). In each instance, after extraction of the cataract, the RAPD disappeared. Thus, a dense cataract causes an RAPD in the contralateral eye by increasing the pupillomotor effectiveness of the stimulus light and that this is the reason why even a brunescent cataract does not cause an RAPD in the same eye. In addition, two clinical cases are reported. One case records the development of a cataract in an eye with a preexisting RAPD resulting from optic neuritis; as the cataract became more opaque, the afferent defect became smaller and finally was reversed. The other case shows that a traumatic cataract can produce an RAPD in the contralateral eye. Thus, when an afferent pupillary defect is seen in an eye with a cataract, a visual pathway defect in that eye should definitely be suspected.

Georg Bartisch (1535-1606) was apprenticed to a barber surgeon as a 13-year-old boy. He took a particular interest in diseases of the eye and over the years made himself into a specialist. In his 40s Bartisch put his special knowledge into this book, apparently doing the illustrations himself, and in 1583, it was printed for him by Matthes Stockel of Dresden. His book was widely read by physicians and students, and its very existence suggested that it might be possible to make a career out of "the service of the eyes." In 1588, at the age of 53, Bartisch was appointed court oculist to the Elector of Saxony, an important position for someone who had started out as an unlettered barber surgeon. Bartisch based his method of eye care on an effort to understand the anatomy, physiology, and optics of the eye. His anatomical plates are famous for flaps that

In Reply. —When we began our evaluation of the cocaine test for Horner's syndrome, we assumed that the net change in anisocoria (anisocoria after cocaine instillation minus baseline anisocoria) would be the best indicator to distinguish patients with an oculosympathetic deficit from patients with pseudo-Horner's syndrome (ptosis and/or miosis). However, at the end of our study, the logistic regression analysis indicated that measuring anisocoria after cocaine instillation was just as good, if not better, in distinguishing the patients with Horner's syndrome from the normal control group. The Figure shows how this might be possible. We have plotted the initial anisocoria vs the anisocoria after cocaine instillation for both the patients with Horner's syndrome and the normal control subjects reported in our study. There was, indeed, a baseline anisocoria in many of our normal control subjects, and some overlap with the patients with Horner's syndrome (Figure). Of equal importance is that

• We have developed a technique of detecting and recording iris transillumination defects using an infrared sensitive video camera. This method is useful for assessing the loss of posterior iris pigment in patients with the pigmentary dispersion syndrome and pigmentary glaucoma. Some patients with no transillumination defects on slit-lamp examination were noted to have discrete defects on infrared videography. This technique is a rapid, convenient, and reproducible method of permanently recording posterior iris pigment changes. These highquality video images can then be followed to determine the natural course and the results of therapy in these patients.

We examined the pupils of 55 amblyopic subjects to determine whether a pupillary defect could be detected in the amblyopic eye. We found relative afferent pupillary defects in 45 of the subjects; these defects were equal to or larger than 0.3 log unit (that is, easily visible) in 29 subjects. The pupillary defect was always in the amblyopic eye, but it could not be correlated with the severity or the cause of the amblyopia, with visual-evoked potential abnormalities, or with color vision defects.

The accurate measurement of pupillary defects is of value in following patients with optic nerve disease, as well as for comparing patients, groups, diseases and tests. The numerous problems encountered in measuring the pupil are considered, and a measurement technique using neutral density filters with the swinging light test is described and illustrated.

When anisocoria is present, the ophthalmologist knows that something is wrong. However, location of the lesion and diagnosis of the problem can be difficult. A step-by-step flow chart for diagnosis of unequal pupils is presented, with explantation of various signs and descriptions of appropriate diagnostic tests.

We compared loss of visual acuity, loss of visual field, and loss of pupillary function in 64 patients with varied damage to the optic nerve. Loss of visual acuity depended on the location of the visual field defect, but the pupillary defect was proportional to the amount of visual field loss. Using this information, we constructed a pattern of dots representing the retinal distribution of pupillomotor force (in log units). This pattern can be used to estimate the approximate amount of pupillary defect expected from a particular visual field defect.

Patients with functional loss of visual acuity or visual fields range from the "deliberate malingerer" to the "suggestible innocent." Between these extremes are patients with varying mixtures of fraud and suggestibility. These patients do not, as a rule, have psychiatric disease and do not need to see a psychiatrist. The ophthalmologist must be able to control frustration with these patients to prove that the patient has better visual fields and visual acuity than admitted to, and so that he can perform a careful, dispassionate examination to establish that no organic disease is present. This examination makes it possible to offer believable reassurance to the patient. Simple reassurance seems to be effective therapy.

Segmental spasms of the iris dilator muscle produce intermittent pupillary distortion. A study of 26 case histories (six previously reported and 19 reported for the first time) showed that the episodes are brief (lasting less than five minutes in 23 of 26 patients, less than two minutes in 18 patients, and less than one minute in eight patients) but can occur several times a day. The patients ranged in age from 24 to 48 years (the age of one patient was not known); five were men and 21 were women. Eleven patients had Horner's syndrome, four had Adie's tonic pupil, and eight had definite or probable migraine (another three had possible migraine). The episodes eventually stopped, leaving no serious sequelae. Focal application of phenylephrine produced a similar distortion in a normal human eye.

Oculomotor paresis with cyclic spasms is a rare syndrome, usually noticeable at birth or developing during the first year of life. On a background of oculomotor paresis, spastic movements occur at regular intervals: about every second minute the lid is raised, the globe wanders toward midline, the pupil contracts, and accommodation increases. These spasms last from 10 to 30 seconds, and then give way again to paresis. Since this phenomenon was first described, opinions were divided about its mechanism and about the site of the responsible defect. Based on a quantitative analysis of a new case, and of all previously published reports, a new theory is proposed which can account for all features of the syndrome.

A thin slit-lamp beam illuminating the pupil margin produced clearly visible pupil oscillations. These oscillations were timed with a stopwatch, thus producing a measurement of the "pupil cycle time". The pupil cycle time was remarkably stable in various testing situations and repeatable within +/- 3% over extended periods of time. When the iris muscles were normally innervated and responsive, the pupil cycle time was dependent on the speed of conduction and the number and strength of optic nerve impulses. Pupil cycle time can be measured in most persons with active or inactive optic neuritis. In the few patients whose light reflex is so poor that the pupil cycle time cannot be measured, the inability to induce cycling can itself be taken as a definite abnormality of the light reflex arc. Only 5% of normal persons 12 to 50 years of age are expected to have a pupil cycle time in either eye longer than 954 msec, or a difference in pupil cycle time between the two eyes longer than 70 msec. Pupil cycle time was significantly longer in patients with optic neuritis, with a P-value less than .001. The pupil cycle time is similar to visual evoked response latency time in that it can detect and quantitate subclinical defects in optic nerve conduction time. Pupil cycle time is objective and quantitative for each eye individually. It is a fast, simple, and reliable clinic test of optic nerve function.

We photographed the pupils of 128 normal subjects in dim light, morning and afternoon, for five consecutive days. Fifty-two of the subjects (41%) had an anisocoria of 0.4 mm or more at one time or another during these five days, but only four (3%) had unequal pupils of 0.4 mm or more in all ten photography sessions. At any given examination, a fairly constant 19% (24 of 128) of the subjects showed this amount of anisocoria. These numbers shifted dramatically when anisocoria was defined as a pupillary inequality of greater than, or less than, 0.4 mm. The prevalence of anisocoria did not vary with the time of day, from day to day, or from week to week, nor was it influenced by the sex, age, or iris color of the subject.

Using the Cochet-Bonnet esthesiometer, we evaluated the corneal sensation of 11 patients with unilateral Adie's tonic pupil. We eliminated six other patients with bilateral Adie's pupil or disease that lowered corneal sensation. An observed unfamiliar with each patient's condition tested 12 clock hour positions in the midperiphery in both eyes of all patients. We studied 30 normal subjects (60 eyes) in a similar fashion. Of those 11 patients with unilateral Adie's syndrome, ten had a regional decrease in corneal sensation. The 30 normal subjects examined did not exhibit any significant decrease in corneal sensation in any areas. Our study supports the concept that the lesion of Adie's tonic pupil is in the ciliary ganglion or short location where the innervation of the iris sphincter and corneal sensation are found together.

The authors evaluated nine affected males and six female carriers from a four-generation family with X-linked cone dystrophy. As the affected males grew older, visual acuity deteriorated, central scotomas deepened, and macular changes became more prominent. There was granularity of the macula in younger individuals and bull's eye lesions and central geographic atrophy of the retinal pigment epithelium (RPE) in the older subjects. The retinas of some affected males had a bronze-green tapetal-like sheen. Color vision was impaired in all affected males and resembled an acquired type II defect (Verriest classification). One younger subject had paradoxical pupillary constriction to darkness. Visual-evoked potential (VEP) latencies were prolonged in some affected males, suggesting that photoreceptor degeneration caused transsynaptic degeneration of ganglion cells. All female carriers had visual acuities of 20/30 or better, but some showed mild ophthalmoscopic changes and abnormalities of color vision, electroretinograms (ERGs), and VEPs.