Fixed and dilated: the history of a classic pupil abnormality.

The aim of this study was to investigate the development of ideas about the nature and mechanism of the fixed dilated pupil, paying particular attention to experimental conditions and clinical observations in the 19th century. Starting from Kocher's standard review in 1901, the authors studied German, English, and French texts for historical information. Medical and neurological textbooks from the 19th and 20th centuries were reviewed to investigate when and how this information percolated through neurological and neurosurgical practices. Cooper experimented with intracranial pressure (ICP) in a dog in the 1830s, but did not mention the pupils. He described dilated pupils in clinical cases without referring to the effect of light. Bright demonstrated to have some knowledge of the pupil sign (clinical observations). Realizing the unreliability of the pupil sign, Hutchinson in 1867-1868 tried to reason in which cases trepanation would be advisable. Von Leyden's 1866 animal experiments, in which he increased CSF volume by injecting protein solutions intracranially, was the first observation in which the association between fixed dilated pupils and increased ICP was established. Along with bradycardia and motor and respiratory effects, he noticed wide pupils were usually present in a comatose state. Asymmetrical dilation could not always be attributed to increased ICP, but to an oculomotor nerve lesion. Pagenstecher in 1871 extended knowledge by meticulously studying consecutive pupil phenomena with increasing pressure. In 1880, von Bergmann emphasized the significance of the ipsilateral dilation in experiments as well as in clinical cases. He distinguished the extent of pressure increase and its duration. Probably confusing irritation (epileptic head turning to the other side with pupil dilation) and lesion effects, he suggested a cortical area responsible for oculomotor phenomena, indicating what is now known as the frontal eye field. Naunyn and Schreiber (1881) understood the relationship between increased ICP with pupil dilation and decreased pulse frequency and blood pressure, warning not to decrease the latter. Concentrating on experimental traumatic effects, Duret (1878) investigated compression and commotion, in which he distinguished two phases, notably pupil constriction by bulbar lesions, due to CSF shock, followed by dilation from congestion and inflammation, due to blood around the oculomotor nerve. The key observation of a fixed dilated pupil as a sign of acute mass effect came gradually and after some localization stumbles. Following the period of extensive experimental research in ICP, the results of which were translated to clinical observations, the prognostic significance was gradually acknowledged by authors of neurological textbooks. It is well known that Cushing did similar experiments in Berne (1900-1901), and later suggested he would not have done so if he had studied the literature.

Blindness of Johann Sebastian Bach.

Johann Sebastian Bach (1685-1750) was one of the greatest composers of all time. Apart from performing as a brilliant organist, he composed over 1.100 works in almost every musical genre. He was known as a hardworking, deeply Christian person, who had to support his family of 20 children and many students staying at his home. At the age of 64 years, his vision started to decline. Old biographies claim that it was the result of overstressing his vision in poor illumination. By persuasion of his friends, he had his both eyes operated by a travelling British eye surgeon. A cataract couching was performed. After surgery, Bach was totally blind and unable to play an organ, compose or direct choirs and orchestras. He was confined to bed and suffering from immense pain of the eyes and the body. He died <4 months after surgery. In this paper, as the plausible diagnosis, intractable glaucoma because of pupillary block or secondary to phacoanaphylactic endophthalmitis is suggested.

[A royal eye lesion]. / En kongelig ojenlaesion.

The 67-year old danish King Christian IV commanded one of the battle ships called "Trefoldigheden" ("Trinity") in a confrontation with the Swedish navy at Kolberger Heide july 1.st 1644. He himself was injured when a Swedish canon ball hit the dolphin (the handle of the canon) and both exploded. A large number of metal fragments were spread over the deck and 2 noble men standing beside the king were mortally injured. Christian's lesions were in his right face, forehead and eye and he lost his vision on this eye. It has been suggested that this was due to a later infection with shrinking of the eye. The King however claimed that nothing was to observe on the eye ball. The King lived until 1648. No paintings exist from the period 1644-48 that reveals his right eye. The author has found a medal portrait of the king from 1645 made by Johan Blum showing a dilated pupil on the right site and some eksophtalmus leading to the conclusion that a direct lesion of the optic nerve either caused by a metal piece or a retro-bulbar haematoma was responsible for the permanent loss of vision. The dilated pupil was caused by a Marcus Gunn phenomena or paradox pupil reaction which the king could not observe himself when he looked in his mirror.

The historical roots of the visual examination.

The history of the visual examination is discussed in five parts. The history of visual acuity is followed from the minimum separable of Persian scientists to the 19th-century charts. Events in the history of the examination of the pupil include the late discovery of the significance of anisocoria in trauma, the description of the pupillary light reflex, the midbrain nucleus responsible for it, and the discovery of the neuroanatomic basis of pupillary abnormalities. Attempts to look into the eyeball date from the 17th century and are followed up to the discovery of the ophthalmoscope (1851). The evolution of the visual field examination is described from the first observations of hemianopia in the Hippocratic Corpus, to the present day visual field examination. Important landmarks in the history of central disorders of visual integration include "mind blindness" and the theories on aphasia that formed the basis for the study of disorders of central integration.