Kelly-Sell, Michael BS; Liu, Grant T. MD
Division of Neuro-Ophthalmology, Departments of Neurology and Ophthalmology (MKS, GTL), University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania.
Presented as a poster at the North American Neuro-Ophthalmology Society meeting in Tucson, AZ, March 2010.
The authors report no conflicts of interest.
Address correspondence to Grant T. Liu, MD, Division of Neuro-Ophthalmology, Department of Neurology, University of Pennsylvania School of Medicine, 3400 Spruce Street, Philadelphia, PA 19104; E-mail: email@example.com
Abstract: Tonic pupils react poorly to light but constrict during viewing of a near stimulus. Adie's name is typically used in association with tonic pupils, but a review of Adie's articles reveals that he described the syndrome of tonic pupils and absent reflexes and not the pupillary abnormality per se. Therefore, it would be more appropriate to refer to a tonic pupil as simply a “tonic pupil” and leave Adie's name for the syndrome.
Today, a tonic pupil—a nonsyphilitic pupil that poorly reacts to light but contracts slowly to a near stimulus and slowly redilates—is often labeled an “Adie pupil,” and the symptom complex of a tonic pupil with absent deep tendon reflexes are labeled as “Adie syndrome.” These eponyms give credit to William John Adie (Fig. 1), an Australian-born neurologist who practiced in London in the early 20th century (1). However, a close reading of Adie's publications on the topic shows that Adie made little attempt to contribute to the literature on the tonic pupil, that its features were largely known prior to his publications, and that it is more appropriate to refer to the abnormal pupil as a “tonic” rather than as an “Adie” pupil.
In May 1931, Adie (2) published his first article on the tonic pupil (Fig. 2), which was a report of 5 patients he examined and a sixth case previously reported by Gehrcke (3,4). He made no claim to add to the existing literature on the tonic pupil, writing the “myotonic reaction is well known to ophthalmologists, and it has been firmly established that syphilis plays no part in its production” (2). Rather, Adie wrote at the beginning of this article, “I wish to draw attention to a benign symptomless disorder characterized by pupils which react on accommodation but not to light, and by absent tendon reflexes” (2). He felt this combination of symptoms was a new contribution to the literature as he knew of only 2 published cases, Gehrcke's case and 1 case by Moore (5), in which a patient was noted to have an abnormal pupil and absent tendon reflexes.
The following year, Adie expanded on his case report and published 2 articles discussing the disorder that we now refer to as “Adie syndrome” (6,7). In a 1932 article published in Brain (6), he wrote that there was a “complete form” of his syndrome “characterized by the presence of the tonic pupillary reaction in its most characteristic form and absence of 1 or more of the tendon reflexes.” He also delineated 4 “incomplete forms,” which include “(a) tonic pupil alone; (b) atypical phases of the tonic pupil alone (‘iridoplegia’; ‘internal ophthalmoplegia’); (c) atypical phases of the tonic pupil with absent reflexes; and (d) absent reflexes alone” (7). These “incomplete forms” expanded the definition of Adie syndrome so that many patients, even with differing symptoms, in his opinion could be diagnosed as having an atypical form of the syndrome (8).
Adie argued that many of the previously reported cases with tonic pupils might actually have had an incomplete form of his syndrome. He wrote, “I feel sure that if the patients described by Foster Moore and by Morgan and Symonds were re-examined it would be found in the first group, where the tonic pupillary reactions were detected, that many of the patients also have loss of reflexes” (6).
Thirty-three years later, Lowenstein and Lowenfeld (8) published a comprehensive review on the tonic pupil chronicling its initial description in the scientific literature and the subsequent discoveries that contributed to our understanding of the phenomenon. They credit the first description of the tonic pupil to Ware in 1812 (9), as he described a patient who likely had the condition—a woman with a single dilated pupil that did not respond to light but did contract to a near stimulus—even though Ware did not focus on the tonic pupil's features. The first major set of articles published on the subject were at the turn of the 20th century when Piltz (10,11), Strasburger (12), and Saenger (13) began describing the characteristics of this pupillary disorder. These authors described a nonluetic pupil with abnormally long-lasting pupillary constriction to the light reflex, or absent light reflex, paired with slow extensive contraction to a near stimulus and sluggish redilation upon returning to a far stimulus. In the early 20th century, Markus (14,15) gave the first description in the English literature of 2 patients whose pupils did not react to light but became “quite small” while viewing a near stimulus and subsequent redilation was prolonged. Over the next 15 years, other cases with tonic pupils were detailed (8).
Lowenstein and Lowenfeld (8) critically argued that many of the characteristics of the tonic pupil were known prior to Adie's publications, including its increased prevalence in women, unilateral presentation, etiology other than syphilis, and characterization by an abnormally reacting iris sphincter. They concluded in their review that Adie “had no priority for any one of the features of the pupillotonic syndrome which bears his name” rather “it was Adie's main contribution to have focused interest upon this syndrome.” Furthermore, we share Lowenstein and Lowenfeld's skepticism that Adie's claim that instances of isolated tonic pupils or isolated absent deep tendon reflexes are incomplete “atypical forms” of his syndrome.
We are not the first to suggest that isolated tonic pupils should be distinguished from those associated with defective deep tendon reflexes. In his personal series, Thompson (16) did separate tonic pupils due to known ocular or orbital disease, such as trauma (“local tonic pupils”), those due to generalized neuropathic conditions, such as diabetes (“neuropathic tonic pupil”), and those due to “obscure origin,” which he labeled as having “Adie's syndrome.” However, of 122 patients he included in this last group, 114 had reduced or absent deep tendon reflexes, while 8 had normal reflexes! He called these 8 patients as having “Adie's with normal reflexes.” While consistent with Adie's original classification, we would suggest that these patients simply had “tonic pupils” without having a syndrome.
Parenthetically, it could also be argued that Adie was not the only author to suggest an association between a tonic pupil and absent reflexes. For instance, one of Markus' patients reported in 1905 (14) also exhibited absent knee jerks, which Markus noted but did not extensively comment upon. Also in 1931, Holmes (17) reported 19 patients having tonic pupils associated with loss of deep tendon reflexes, prompting many to call this the Holmes–Adie syndrome.
In no way are we advocating that eponyms be abandoned. Instead, we are recommending that we attribute a named syndrome or disease correctly to what the person actually described (18,19). Eponyms add color, humanism, and a historical basis to our medical diagnoses, and the shortened terminology facilitates communication among physicians and patients (20). Although the use of eponyms has been discouraged because in part they may be too nebulous or honor infamous individuals (21), designations such as Alzheimer disease, Parkinson disease, Babinski sign, and Susac syndrome will always be part of our neurologic and ophthalmologic lexicon. Certainly, these eponymic designations are preferred to dementia due to neuritic plaques and tangles; bradykinesia, tremor, and rigidity due to dopaminergic neuron depletion; large toe extension following plantar stimulation; and idiopathic retinocochleocerebral microangiopathy. Eponyms do not necessarily have to be ascribed to the person who provided the first description but rather to honor the person(s) who clarified, synthesized, or popularized the disorder, syndrome, or sign.
Thus, upon closer inspection of the historical record, Adie was neither the first to describe the tonic pupil, nor did he do anything more than make it part of his syndrome. He understood that the characteristics of the tonic pupil were well established when he published on the subject. In summary, it would be more appropriate to refer to a tonic pupil as simply a “tonic pupil” and leave Adie's name for the syndrome he carefully described and popularized.
The authors acknowledge Bryan Kaminski for his help with translating the German references and both Jessica Silver at the Imperial College Archives and Melody McDonald at the Geelong Heritage Center for their help with obtaining biographical information regarding Dr. Adie.
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