Seneca and the First Description of Anton Syndrome

Seneca was a Roman philosopher, politician, and mentor to the young Nero. He later fell in disgrace and was sentenced to death by the Emperor. Seneca left many texts, one of the most influential being his Moral Letters to Lucilius (63 CE). In Letter 50, he describes the case of Harpaste, his wife's foolish slave who acutely became blind. She denied her illness and argued irrationally about room darkness, constantly asking attendants to change her quarters. Harpaste's case, consisting of acutely acquired blindness and anosognosia in the presence of relatively well-preserved cognition, fulfills the clinical criteria for the diagnosis of Anton syndrome, and probably constitutes its first description.

Historical Note Seneca and the First Description of Anton Syndrome Charles André, MD, PhD Abstract: Seneca was a Roman philosopher, politician, and mentor to the young Nero. He later fell in disgrace and was sentenced to death by the Emperor. Seneca left many texts, one of the most influential being his Moral Letters to Lucilius (63 CE). In Letter 50, he describes the case of Harpaste, his wife's foolish slave who acutely became blind. She denied her illness and argued irrationally about room darkness, constantly asking attendants to change her quarters. Harpaste's case, consisting of acutely acquired blindness and anosognosia in the presence of relatively well-preserved cognition, fulfills the clinical criteria for the diagnosis of Anton syndrome, and probably constitutes its first description. Journal of Neuro-Ophthalmology 2018;38:511-513 doi: 10.1097/WNO.0000000000000682 © 2018 by North American Neuro-Ophthalmology Society D eficit unawareness was initially described by a number of authors such as Wernicke (aphasia) and Westphal (hemianopic and proprioceptive deficits) (1). Von Monakow, Déjerine and Vialet, Rossolimo, and F. Müller were among the first to describe it after cortical blindness (2-4). However, Gabriel Anton (1858-1933) and Joseph Jules François Felix Babinski (1857-1932) were the first authors to highlight the importance of anosognosia ("anosodiaphoria") in patients with specific deficits from acquired focal cerebral disorders and exhibiting relatively preserved cognition. While Babinski concentrated on anosognosia in hemiplegic patients (5), Anton must be praised for describing in detail the psychology of these focal diseases and for attempting to develop a pathogenetic theory based on neuropathology (6). He described patients with acquired severe blindness or deafness who were unaware of Department of Neurology (CA), Faculdade de Medicina, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil; and Department of Neurology, Sinapse Neurologia e Reabilitação (CA), Rio de Janeiro, Brazil. The author reports no conflicts of interest. Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the full text and PDF versions of this article on the journal's Web site (www. jneuro- ophthalmology.com). Address correspondence to Charles André, MD, PhD, Rua Visconde de Pirajá 414, sala 821, Rio de Janeiro CEP 22410-002, RJ, Brazil; E-mail: [e-mail] André: J Neuro-Ophthalmol 2018; 38: 511-513 their deficits, attributing them to external factors despite being confronted by reality (7-9). I present evidence that the Roman politician and philosopher Seneca the Young (c. 4 BC-65 AD) described the case of Harpaste, a woman who was unaware of her acquired blindness, at the start of the Christian era. THE CASE OF HARPASTE The "Moral Letters to Lucilius" were probably written in 63 AD, during Seneca's political exile (10). Their main focus was to discuss the principles that should guide a sage's life. In Letter 50, Seneca described how one of the maids of his second wife -"my wife's female clown" (uxoris meae fatuam), called Harpaste- was kept in his house as "a burden incurred from a legacy" (11) (see Supplemental Digital Content, http:// links.lww.com/WNO/A322). Although omitting particulars such as age or any disease characteristics, Seneca clearly described how "this clown" (Haec fatua) suddenly became blind. The story sounds incredible, but I assure you that it is true: she does not know that she is blind. She keeps asking her attendant to change her quarters; she says that her apartments are "too dark." Seneca stated that we should not laugh at Harpaste because we also tend to deny and give excuses for our defects and sins and do not look for help to avoid these behaviors. DISCUSSION In a troubled period when the Roman Empire was suffering economically, Britain was up in arms against it, and Roman temples were being seized, Seneca fell in disgrace. He became aware that Nero and the praetorian prefect Tigellinus intended to kill him (Fig. 1). As stated by the Historian Publius Tacitus (c. 56 AD-c. 120 AD): "According to some writers, poison was prepared for him (Seneca) at Nero's command by his own freedman, whose name was Cleonicus. This Seneca avoided through the freedman's disclosure, or his own apprehension, while he survived on a very simple diet of wild fruits, with water from a running stream when thirst prompted." (12). A romantic version of this event, by "Malachi Mouldi" (actually Henry Godwin [1811-1874]) put Harpaste in the center of this episode (13). Here, she was said to have 511 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Historical Note FIG. 1. Nero and Seneca, by the Spanish sculptor Eduardo Barrón (Moraleja del Vino, Zamora, 1858-Madrid, 1911). Museo del Prado, Madrid, Spain. The sculpture (1904) dramatically highlights the differences in character between the emperor and his tutor. accompanied Cleonicus and the praetorian Julius Pollius in their visit to obtain the venom from the famous Gallic hag, Locusta. The visit terrified Cleonicus and only ended when Locusta, after giving instructions on how to use the venom (which had been useful on other occasions for Nero), danced and chanted the last couplet of a dreadful Gallic chant, took the venom herself and immediately died. In the aftermath of this visit to Locusta, Harpaste would have repeated short fragments of what had been said in an unconnected manner. On the day of the planned poisoning of Seneca, at the exact moment Cleonicus gave him a glass of water with the venom, Harpaste, who was "winding some silken cord for Seneca's wife, Claudia," unexpectedly sang Locusta's last couplet: "To these four scores I add but one, and then the sacrifice is done" All this frightened Cleonicus so much that he "dashed the cup to the ground; fell at his master's feet, and confessed the whole!" (13). Mouldi described Harpaste as a young and blind idiot girl, a half-witted jester whose singular fantasies sometimes provoked a smile in Seneca's grave countenance. She was capable of "extraordinary sayings". Her malady was said to be of recent origin. The famous Letter 50 to Lucilius was reproduced with a short comment on the philosophical implications of Harpaste's denial of her blindness. Also, the author mentioned that the witch, Locusta, held a burning brand right in front of Harpaste's eyes and that the blind girl reacted exclaiming: 512 "Tis lighter!-"tis lighter! I told you it was not dark out of doors." There is no doubt that Harpaste is a historical personage (14) and as such she was described by later authors (15,16). Seneca does not give a clue as to her age at symptom onset. His short report indicates an acquired disorder restricted to the nervous system. Whether or not she would nowadays be considered demented is controversial. Seneca considered her a jester. The words used to describe her-fatuam, fatua are feminine derivatives of fatuus, which does not mean demented, but foolish, simple, silly ("Ego me ipsum stultum existimo, fatuum esse non opinor:" I consider myself stupid, not silly.) (17). If we lend credit to Mouldi's description, Harpaste had no significant motor deficit, aphasia, or apraxia, and did not suffer from short-term memory problems. Of course, no firm statement about executive functions and a number of other cognitive aspects can be made, but Harpaste apparently could take care for herself. The core symptom in Anton syndrome is unawareness of a major sensory deficit, for which a number of mechanisms have been proposed (18,19). Patients may seem inattentive or not fully aware of their deficits; or may exhibit anosognosia, complaining of poor lighting, or giving confabulated descriptions of scenes or faces (20). Anton justly emphasized the specificity of the problem, stressing that patients should not exhibit frank cognitive decline. However, all the patients described by him exhibited additional neurological deficits including language dysfunction, and these additional manifestations should not exclude the diagnosis (19,20). Harpaste suffered from acutely acquired blindness and vigorously negated her condition. Whether this was caused André: J Neuro-Ophthalmol 2018; 38: 511-513 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Historical Note by a stroke or another disease process causing cortical blindness (19-23) is a matter of speculation. Seneca's description, however, should probably be acknowledged as the first case report of Anton Syndrome. ACKNOWLEDGMENTS The author thanks Professor Péricles Maranhão Filho, MD, PhD, for insightful discussion and comments, and Dr. Sergio Telles Ribeiro Filho, MD, for English editing. REFERENCES 1. Adeyemo BO, Nesathural S. The original description of Anton syndrome. PM R. 2013;5:74. 2. David A, Owen AM, Förstl H. An annotated summary and translation of "on the self-awareness of focal brain diseases by the patient in cortical blindness and cortical deafness" by Gabriel Anton (1899). Cogn Neuropsychol. 1993;10:263-272. 3. Förstl H, Owen AM, David AS. 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