Cuban Epidemic Optic Neuropathy (1991-1993) and José Saramago's Novel Blindness (1995).

PURPOSE: This article reviews the history of Cuban epidemic optic neuropathy (1991-1993), which caused visual loss, peripheral neuralgias, and other neurologic symptoms in over 50,000 persons, an incidence of almost 0.5% of the entire population. The clinical findings, etiology, and treatment are described. We then relate the Cuban epidemic to the fictional epidemic of contagious blindness depicted by Nobel Laureate José Saramago in his 1995 novel Blindness. This novel describes an unnamed modern city in which all inhabitants, except the ophthalmologist's wife, are affected with a white, not black, blindness. DESIGN: Historical review and literary essay. METHODS: The sources for the Cuban epidemic were an extensive review of the published literature and personal communications with physicians who treated these patients. Both authors have analyzed the novel and the critical literature about Saramago's writings. RESULTS: Though Saramago uses the epidemic of blindness as an allegory to comment on human weakness and immorality, he may also have known of the actual Cuban epidemic. Saramago was a lifelong member of the Communist party, as well as a friend of Fidel Castro and admirer of the Cuban government. We have no proof that Blindness was influenced by the Cuban epidemic, but we find it plausible. CONCLUSION: It is valuable to examine the real and fictional epidemics side by side, not least because Saramago's novel depicts the actions of an ophthalmologist during an epidemic of blindness. Ophthalmologists may be interested in a novel that uses the language of eyes, vision, sight, and blindness extensively.

Tobacco optic neuropathy (TON) - the historical and present concept of the disease.

This article reviews the historical and current concepts of 'tobacco optic neuropathy' (TON) a rare disorder of optic nerve function related to the toxic effects of an unidentified constituent of tobacco. It is considered to be an entity distinct from that often described as 'tobacco-alcohol amblyopia', a disorder better described as a nutritional optic neuropathy. It is suggested that 'tobacco-alcohol amblyopia' is an inappropriate term, because the condition to which it refers is not an amblyopia, and there is little evidence to implicate a toxic effect of either tobacco or alcohol in the pathogenesis of that disorder.

[Development of neurology in Japan and its contribution to elucidate and resolve the sociomedical problems].

Japanese Society of Neurology (JSN) was established in 1960 with 643 members, and in 2009 it has grown up to a big society having more than 8,000 members including 3,600 neurology board specialists. JSN has greatly contributed in elucidating and resolving many socio-medical problems. I will take three topics including SMON (subacute myelo-optico-neuropathy), infectious Creutzfeldt-Jakob disease (CJD) and Minamata disease. SMON was a new epidemic disease characterized by subacute optic neuritis and myeloneuropathy associated with diarrhea and abdominal symptoms. The research committee clarified that it was a neurological complication of chinoform, a drug for gastroenteritis. CJD surveillance started in 1996 for variant CJD, and uncovered many patients who developed CJD after human dura draft. The government prohibited to use non-inactivated human dura. Minamata disease is an organic mercury poisoning of people who took fish contaminated by mercury in Minamata bay in Kumamoto or in Aganogawa river in Niigata. The factories discharged water contaminated with mercury which was accumulated in fish and shellfish. Still many victims claim for compensation to the companies and government. Neurologists in Kumamoto and Niigata greatly contributed to diagnose and treat the victims and to clarify the cause of the disease.

Evaluation of the retinal nerve fiber layer: descriptive or predictive?

For nearly a century, ophthalmologists have recognized that thinning of the retinal nerve fiber layer (rNFL) could be observed ophthalmoscopically in diseases of the optic nerve. Using high-resolution red-free fundus photography, Hoyt found slit-like rNFL defects that corresponded to visual field defects in glaucoma. Frisén extended these observations to multiple sclerosis, predicting the later discovery that axonal loss occurs in the retina without clinical bouts of optic neuritis. In measurement of the rNFL, red-free fundus photography has been superseded by more widely available, robust, and quantitative retinal imaging techniques, including Heidelberg retinal tomography, scanning laser polarimetry, and optical coherence tomography (OCT). Having emerged as the technique of choice in measuring the rNFL, OCT has shown that the degree of preoperative rNFL thinning reliably predicts whether vision will recover after surgery for pituitary adenoma. Such quantitative studies of the rNFL have the potential, therefore, of providing descriptive and predictive information that will be valuable in clinical care.

Neurosurgery 100 years ago: the Queen Square letters of Foster Kennedy.

The prominent New York City neurologist Foster Kennedy (1884-1952) trained at London's Queen Square from 1906 to 1910. He was exposed to a number of eminent neurologists including, Sir Victor Horsley and other surgeons. A previously published but little known group of Kennedy's personal letters written during his neurological training is reviewed in light of their historic neurosurgical context. These often entertaining although at times unflattering letters capture the elation and frustration of neurosurgical experience during that important developmental period. Descriptions of the personalities involved give rare insight to this era. The Foster Kennedy syndrome--homolateral optic atrophy and contralateral papilledema with an inferior frontal tumor--was characterized under the stimulating support of Gowers and Horsley.

A history of the optic nerve and its diseases.

We will trace the history of ideas about optic nerve anatomy and function in the Western world from the ancient Greeks to the early 20th century and show how these influenced causal theories of optic nerve diseases. Greek and Roman humoral physiology needed a hollow optic nerve, the obstruction of which prevented the flow of visual spirit to and from the brain and resulted in blindness. Medieval physicians understood that the presence of a fixed dilated pupil indicated optic nerve obstruction, preventing the passage of visual spirit, and that cataract surgery in such cases would not restore sight. During the Renaissance, the organ of vision was transferred from the lens to the optic nerve, which was generally believed to be on the axis of the eye. The acuity of central vision (at the optic disc) was explained by the concentration of visual spirit where the optic nerve met the retina. The growth of anatomy and influence of mechanical philosophy from the 17th century led to visual spirit being replaced with the concept of nerve force, which later became associated with electricity travelling along nerve fibres. This coincided with discourse about the nature of the nervous system and a shift in orientation from understanding illness holistically in terms of an individual's humoral imbalance to the concept of organ-based diseases. Both the microscope and the ophthalmoscope allowed visualisation of the optic nerve, but problems of interpretation persisted until conceptual transformations in medical science were made.

Victor Horsley's contribution to the Foster Kennedy syndrome.

Although Foster Kennedy is the name given to the syndrome localizing an intracranial tumour with respect to papilloedema, it is important to recognize how Victor Horsley was one of the strongest proponents in the usefulness of that sign. Not only did he describe the constellation of findings before Kennedy did, he was also probably one of the people who had influenced Kennedy the most during the latter's time at the National Hospital for Nervous Diseases at Queen Square. It is therefore appropriate that Victor Horsley's characterization in 1910 of the findings that were later recorded as the Foster Kennedy syndrome, and that his altruistic encouragement of Kennedy's publication of the findings, receive recognition and appreciation in the medical and historical community.

[Tübingen 1895-1899. Incidence, diagnosis and therapy of selected optic nerve, uveal and retinal diseases 100 years ago]. / Tübingen 1895-1899. Häufigkeit, Diagnose und Therapie ausgewählter Erkrankungen des N. opticus, der Uvea und der Retina vor 100 Jahren.

BACKGROUND: The records of the University Eye Clinic are almost completely preserved at the historical archive of the University of Tübingen since 1865 and shell now be reduced. MATERIALS AND METHODS: 150 records from the historical archive of the University of Tübingen were selected. We were interested in those patients treated for the first time at the University Eye Clinic between 1895 and 1899. Only diseases of the optic nerve, the uveal tract and the retina were considered. The records were distributed into groups following historical classification and statistics of the same period. These statistics were also used to compare our selection with all patients treated. For information about frequency, diagnostics and therapy a questionnaire was developed. RESULTS: Glaucoma: The data of the subgroup "Glaucoma simplex" showed high correspondence with the historical data regarding the frequency (24.3 vs. 25.5%). The data of the subgroup "Glaucoma secundaria" differed (34.5 vs. 25.5%). Testing the visual acuity was the first step of examination here and in all groups (100%). Measurement of intraocular pressure with digital palpation followed (86.3%). First of all Eserin eye drops were applicated (76.5%). Iritis: The frequency of acute iritis was 16.8 vs. 25.0%, and of chronic iritis 59.6 vs. 52.2%. As typical symptoms ciliar injections, fixed anisocore pupil and exudation were often seen (80.0%). Chronic iritis showed as typical symptom posterior synechia (80.9%). Atropin eye drops were firstly applied in acute iritis (100%), chronical iritis was treated by iridectomy (57.1%). Myopia: In all subgroups our data differed from the historical data. Staphyloma posticum was the characteristic diagnosis (65.8%). Myopia was treated by decision of the lens. Retinal detachment: The frequency 37.2 vs. 28.0%. In nearly all cases retinal detachment was described after ophthalmoscopic examination (92.8%). The first therapeutical step was to keep the patients staying in bed (53.8%). CONCLUSIONS: As expected, diagnostic and therapeutical options were strongly limited in Tübingen 100 years ago. Nevertheless, therapeutic successes were achieved in certain cases.

[Raeder's syndrome]. / Raeders syndrom.

Raeder's syndrome was first described by the Norwegian ophthalmologist J.G. Raeder in 1918 and again in 1924 by the same author. The seminal report was a description of a young male patient with unilateral periocular pain combined with ipsilateral miosis and ptosis, and with slight objective signs of trigeminal nerve involvement. Autopsy demonstrated a tumour at the base of the skull in the middle cranial fossa. Raeder coined the term "paratrigeminal" for the reported clinical picture. Later case reports by Raeder and other authors have included patients experiencing a more benign clinical course, some with spontaneous remissions, with unilateral periocular pain and ipsilateral signs of oculosympathetic paresis as the common denominator. This article is a chronological survey of the main contributions to the medical literature. Various definitions of the syndrome are outlined, including the more recent classification, as well as some pathophysiological and prognostic considerations.

'On mind-blindness (optic agnosia)', a classical clinico-pathological report, and its author Wilhelm von Stauffenberg (1879-1918).

Two photographs from the beginning of our century still instructively illustrate the phenomenon of visual agnosia: the perplexed face of an old lady confronted with objects clears after touching them. This patient with bilateral posterior cerebral infarction was followed for seven years at the Munich Medicine Department of Friedrich von Müller (1858-1941) who had a strong interest in neurology. His assistant Wilhelm von Stauffenberg (1879-1918) reported the case in great detail, including anatomical analysis performed under Constantin von Monakow (1853-1930). Promoted to "Privatdozent" in 1913 on the basis of this report, Stauffenberg's work included several additional, similarly extensive clinico-anatomical case studies but was not limited to problems of cerebral localisation. Under the influence of the Burghölzli group in Zürich he started to practice psychotherapy. Stauffenberg, deeply interested also in the arts and in literature, was the physician of the poet Rainer Maria Rilke (1875-1926) from 1914. He succumbed to pneumonia in 1918.