Papilledema Without Increased Intra- Cranial Pressure

PAPILLEDEMA WITHOUT INCREASED INTRA- CRANIAL PRESSURE

ARTHUR J. BEDELL, M.D. Albany, N. Y. Swelling of the optic nerve is neither rare nor difficult to diagnose, but as yet no all ipclusive etiologic theory has been propounded that satisfactorily explains the variety of its manifestations. Papilledema is most often associated with increased intracranial pressure or intra-orbital vein compression. Many cases, however, occur without either of these. The six cases recorded here include four patients in whom the nerve involvement was unilateral and two in whom both eyes were affected. Tenderness of the eyeball and pain on movement of the globe were present in varying degrees in all the cases, as was also early visual disturbance, ranging from a slight blur to total blindness. In one patient the condition might have been attributed to an orbital cause, but in the others nothing was found to account for the eye changes. There were so many possibilities in each case that our in- terest was aroused and our curiosity stimulated, so that in- vestigations were undertaken, but even with these incentives and exhaustive studies the etiology of this disorder was not determined. The changes in the disc, the unpredictable course, the response to treatment or the complete failure of medication, all added zest to the search for truth and increased the urge to overcome the difficulties that made solu- tion of the problem seem impossible. Appreciating the fact that these cases are not unique, for we have had other cases, they are presented here as a clinical photographic contribu- tion to arouse a discussion regarding diagnosis. CASE 1.-For three weeks a male, aged thirty-eight years, had noticed that the sight of his left eye was failing. He had slight 242 BEDELL: Papilledema 243 ocular pain. The discomfort was almost inconsequential, and was admitted only after close questioning. He seemed to be in excellent health, although he was subject to colds which were followed by asthma. He had no other symptoms of allergy, and had never had any nasal treatments. His past medical history disclosed nothing of note. The vision of the right eye was 20/15 and Type 1. The pupil was 3 mm., regular, and active. The media were clear, and the disc was distinctly outlined. Examination of the fundus was negative. The vision of the left eye was 20/30, with correction 20/20, Type 1. The pupil was 3 mm., regular, and active. The cornea, aqueous, and lens were clear, but there were fine, dust-like opacities in the vitreous. The retina was edematous. The disc projected about 112 diopters, and was almost as red as the remainder of the fundus, with which it merged. Over the disc the dilated, but not tortuous, veins were traced. The arteries were smaller than normal, and could be followed only by carefully focusing on the different retinal levels. There were no hemorrhages or exudates. The visual field was slightly contracted. Report of the x-ray examination was as follows: "In the vault of the skull the bone structure is normal and there is normal distribution of diploic and vascular markings. In the base of the skull the outstanding finding is a bilateral flattening of the anterior clinoid processes and absorption of their tips to needle-like points. The absorption is more prominent in the left clinoid process than in the right. This finding is strongly indicative of a suprasellar mass. There are no intracranial calcifications. All the sinuses and the mastoid processes are normal. . The optic foramina are equal in size and are clearly outlined." Three days later the swelling of both the disc and the retina had increased and there were several prominent wrinkles near the temporal side of the disc. Because of the progressing edema and the radiographic findings, serious consideration was given to a diagnosis of intracranial disease. Five days later the retinal edema was less marked, but the disc was paler and more swollen, and a thin cloud, like mist on a moun- tain, was present over its lower edge; this partially obscured the border. There were several thin, striate hemorrhages contiguous to the nasal side of the disc. The inferior temporal artery dipped into the retina and appeared irregular in caliber, but otherwise the arteries were much clearer. The veins were less distended. At this time his physician made another complete examination, and re- 244 BEDELL: Papilledema ported that he could find no abnormalities, and that neurologic tests were negative. All sensory transmissions were intact. Motor reactions were normal, the gait was normal, and the Romberg and Babinski tests were negative. According to his dentist, there was no disease of the teeth. Blood examination: Hemoglobin, 94 per cent.; white blood cells, 6,400; red blood cells, 4,820,000; polymorphonuclears, 62 per cent.; lymphocytes, 37 per cent.; myelocytes, 1 per cent. Urinaly- sis negative. Wassermann negative to all reagents. One month after my first examination the vision of the left eye was 20/15. The disc was flat, clearly outlined, and of normal color. The retinal vessels were normal. Up to the present, three years later, there has been no recurrence. CASE 2.-A month before examination, a butcher, aged twenty- eight years, noticed that his right eye was bloodshot. He covered his left eye, and discovered that colors were not clearly seen with his right eye. He had a dull frontal headache, but no actual pain. V.R.E. was 20/30; with correction, 20/20, Type 1. The pupil was 3 mm., regular, and active. The media were clear. The edges of the swollen disc sloped to the retinal level of the nerve-head, which was readily demarcated from the remainder of the fundus by its light pink color. The vessels on the disc were heavily sheathed with a white exudate; the veins were distended, but not tortuous, and the arteries were less readily defined than the veins, especially in the lower half of the fundus. There was a bright, curved vitreous reflex over the lower nasal portion of the disc. The blind spot was about three times its normal size. V.L.E. was 20/20. The pupil was 3 mm., regular, and active, the media were clear, and the disc was sharply outlined, with normal vessels. His complete physical and neurologic examinations were negative. Blood examination: Red blood cells, 5,720,000; hemoglobin, 114 per cent.; color index, 1.00; white blood cells, 9,850; differential count: polymorphonuclears, 48 per cent.; eosinophiles, 1 per cent.; basophiles, 1 per cent.; large lymphocytes, 30 per cent.; small lymphocytes, 16 per cent;. neutrophilic myelocytes, 1 per cent.; megamyelocytes, 3 per cent. Urinary examination was completely negative. The x-ray examination showed normal bone structure throughout the calvarium, with normal vascular and diploic markings. The sella turcica was normal in structure, and the pituitary fossa was also normal. Immediately above the dorsum sellae, and extending BEDELL: Papilledema 245 backward for a very short distance, were two linear markings evi- dently the shadows of the free margins of the tentorium cerebelli partially calcified. The accessory nasal sinuses were relatively large. The left frontal cells contained several transverse septa in addition to the usual longitudinal ones. The most lateral cells on the left side were more opaque than any of the other frontal cells, but a study of the film proved that this was due to very broad anterior terminations of the several horizontal septa. The ethmoid cells on the right side did not contain fluid. The antra and sphenoid sinuses were clear. Three weeks later the vision was 20/20. The vitreous reflex had disappeared, but the disc was covered with a- definite translucent edema through which, on a deeper level, the enlarged disc was clearly seen. His condition improved steadily. The disc became flat, returned to its normal size, and the border was clearly defined. The vessels on the disc were less sheathed, and they, like the retinal vessels, were otherwise negative. The treatment consisted of the administration of mercury, iodids, and atropin. The eye has remained quiet for the past year. After a pains- taking search no etiologic excitant could be discovered. CASE 3. -A male, aged eighteen years, was first seen September 28, 1929, at which time he gave a history of diplopia, with pain in his left eye, which had begun two weeks previously. He complained of dizziness, and had a slight weakness in his legs, so that his gait was uncertain. His physician declared that there was ocular muscle weakness, with slight paresis of the right side of his face, loss of abdominal reflexes, and numbness with tingling sensations in the fingers of the right hand. The patient stated further that the vision of the right eye failed five days before he came for examination, and that moving his eye gave rise to pain. V.R.E. = 1/200. The pupil was 4 mm., regular, and active. The media were clear; the entire fundus was gray. The disc projected several diopters, and the superior temporal portion was the most prominent, and the nasal side the least swollen. The retinal veins were normal in size, and distribution could be traced through the edematous nerve-head into the central excavation. The arteries were on a deeper level in the disc, and were corre- spondingly a little more veiled and a trifle smaller than normal. There was a large central scotoma and the blind spot was enlarged. 246 BEDELL: Papilledemna The left eye was normal, and remained so dluring the entire two years the patient was under observation. V.L.E. = 20,'20. The pupil was 4 mm., regular, and active, and the media were clear. The disc was clearly outlined, and the fundus was normal. The patient was admitted to St. Peter's Hospital. Urinalysis was negative and the Wassermann test was also negative. The x-ray of his head disclosed no pathologic changes. Spinal fluid Wassermann test was negative. Colloidal gold test No. 0012210000. No increase in sugar; slight trace of globuliin. The spinal fluid was negative for tubercle bacilli. Blood sugar, 100. Blood examination: Hemoglobin, 100 per cent.; red blood cells, 4,744,000; white blood cells, 6,600; polymorphonuclears, 70 per cent.; small monocytes, 21 per cent.; large monocytes, 5 per cent.; eosinophiles, 4 per cent. There was no nystagmus or facial weakness, and, with the exception of the absent radial and biceps reflexes, his neurologic examination was negative. Nasal examination was negative. The disc swelling decreased so rapidly that eighteen days later the edge was seen clearly. However, the vision was only 2/200, andl the diminution in the size of the central scotoma and the blind spot was negligible. Gradually the disc became flat, and the central scotoma was reduced to less than 1 degree, but the blind spot remained large. When last seen, two years after his first visit, the vision was 20/70. The disc was white, with a well-defined border and without any overlying veiling. This was another case of unilateral papilledema. The most striking feature was the great swelling of the temporal portion of the disc. There has been no recrudescence. CASE 4. -A boy, aged thirteen years, awoke oine morning to find that he could not see with his right eye. The history was completely negative. He had had no illness, had not complained of pain, or of anything referable to the head or eyes. A complete physical examination was negative, as were all the laboratory tests, including the Wassermann. The vision of the right eye was 5/200. The pupil was 3.5 mm., regular, and active. The media were clear. The retinal veins were about three times as large as the slightly narrowed arteries. The dominant fundus feature was the three full, not tortuous, veins as they converged on the disc and disappeared in the well-maintained central excavation of the edematous nerve-head, which had a fine BEDELL: Papilledema 247 vascular crown. The swelling extended beyond the disc. Several striate hemorrhages ran parallel to the superior vessels. Within eleven days the picture had changed. The swollen disc was much paler and more prominent, with several recent hemorrhages on an exudate cap. The upper retinal veins approached the normal size more nearly than the inferior veins of the retina, especially the temporal branch, which remained large. Eight months later the disc was almost completely covered by a translucent exudate; the veins were less distended. After five months the disc was clearly outlined, and the vessels were normal in size, but a connective-tissue film; roughly triangular in shape, covered the central portion of the disc, the base of the triangle overhanging the upper disc border. The vision was 20/20 and there were no field defects. The left eye was always free from disturbance. The pupil was 3 mm., regular, and active. The media were clear. The disc was flat, and the fundus was negative. The patient experienced no further annoyance for three years, and then, for some unknown reason, he developed a frontal headache, with hypertension, 150/110, but no demonstrable kidney involvement. When his refractive error was corrected, his headache disappeared. In 1934 he had a transitory blurring of vision, with a macular edema which vanished in a few days. Three years later, on February 26, 1937, he suffered an attack in which the vision was reduced for two days and his eyes were painful on motion. At that time he had a slight coryza. The vision was 20/20, and externally the eye was negative, but the fundus presented a marked change. The disc was so swollen and gray that the margins were not visible. The veins, particularly the inferior ones, were again distended, and several long, thick streak hemorrhages were present on the temporal side of the disc and beneath the triangular cap of exudate, which was not only larger, but much denser than it had ever been before. In a few days the hemorrhages were absorbed, but there was a thick, glistening, dotted vitreous veil overlying the disc. An inspection of the fundus on May 14, 1937, showed that the disc veil was almost the same as it was before the flare-up. When last seen, on June 28, 1939, the vision was 20/20. The fundus was mottled, the disc slightly pale, but the veil was not only transparent, but also much smaller. This was a case of unilateral papilledema with a connective- 248 BEDELL: Papilledema tissue remnant which increased in size and thickness during a recurrence of the edema, and then disappeared almost entirely. No cause for the condition was discovered. The teeth, the tonsils, the sinuses, and everything else were without pathologic evidences, and with the exception of the evanescent hypertension, no circula- tory symptoms were ever present. CASE 5. -Sudden bilateral blindness is not uncommon, but when papilledema is the outstanding fundus change, an emergency exists that calls for prompt, intelligent investigation. A male, aged forty-three years, was seen first when his vision was reduced to light perception in each eye. He had never had any difficulty with his sight until September 5, 1935, when both his eyes were sore, that is, were tender to the touch and on motion. Four days later the vision of the right eye was hazy. A week later the patient complained of nausea, and because he had a tooth- ache, he went to a dentist who advised immediate extraction of several abscessed teeth. Four teeth were removed from the upper jaw under novocain anesthesia. That night he had pain in the left eye, and vision began to fail. The following day two more teeth were extracted, and his vision became much worse in each eye, although he was able to drive his car to the dentist's office. On September 18, 1935, vision in the right eye consisted of light perception. The pupil was 6 mm., and there was no reaction to light or accommodation. The media were clear. The disc was edematous, with rounded edges, and the retinal veins were so much fuller than normal that they appeared like large, dark red bands. No hemorrhages or exudates were present. Vision in the left eye was light perception. The pupil was 6 mm., stationary. The media were clear. The disc was swollen and gray, and the margins were undefined. The retinal arteries were small, and the straight veins were greatly dilated. There was practically no visual field in the left eye, but in the right eye a small nasal arc was outlined with a 4 degree target. The patient entered St. Peter's Hospital. The x-ray report was as follows: "No abnormalities are seen in the bony structure of the skull. The pituitary fossa is normal in size, and the sella turcica is entirely normal in structure. The sphenoid sinus is normal. All ethmoid cells and the antra are clear. There is calcification in the anterior part of the falx cerebri." Laboratory tests: The spinal fluid was clear, and there were eight cells per cubic millimeter. There was no demonstrable de- BEDELL: Papilledema 249 crease in the sugar content, the Wassermann test was negative, and the colloidal gold test, 000,000,000. Blood examination: Hemoglobin, 68 per cent.; red blood cells, 3,512,000; white blood cells, 9,600; polymorphonuclears, 72 per cent.; small lymphocytes, 24 per cent.; eosinophiles, 4 per cent. The Wassermann test was negative to all antigens. The blood-nonprotein nitrogen was 38.2; urea, 15.7; uric acid, 3.7; chlorids, 399; blood-sugar, 72 mg. The urine was negative; specific gravity, 1016; no albumin, sugar, or pathologic elements were present. The patient had had whooping-cough, scarlet fever, measles, and chicken-pox as a child, but during adult life the only pathologic condition had been a left-sided chronic suppurative otitis media. The family history was negative. The blood-pressure was 140/66. A complete physical examination was negative. Two weeks after the first examination the right disc was pale and flat, with clear outlines. The retinal veins were of normal size, but the arteries, especially on the disc, were narrowed. The left nerve-head was white, and the edges were a little fuzzy; the arteries were very narrow, and the veins were not abnormal. When last seen, on September 6, 1940, the vision of the right eye was 10/200. The disc was pallid and the arteries were narrow. There was an inferior hemianopsia with a central scotoma. The vision of the left eye was light perception. The disc was white, and the retinal arteries were small. In this case both nerves were involved, and there was a rapidly developing edema which was so severe that vision was permanently impaired. At no time were there any signs of inflammation, such as the presence of exudates or hemorrhage. CASE 6.-This last case is reported because it varied clinically from the others. A woman, aged twenty years, had had an attack of red eyes accompanied by poor vision before she came under my observation. Four years ago she was in a hospital for three weeks because her "right eye was paralyzed." Three weeks preceding her first visit to me she had a "cold" with bronchitis, which was followed by severe pain, first in one eye and then in the other, and at times there was an intense faceache. There was no history of any constitutional disease. On October 2, 1937, vision in the right eye was 4/200. The eyeball was proptosed several millimeters. There was extreme limita- tion of motion in all directions. The globe was greatly congested, with marked distention of several large conjunctival veins, and 250 BEDELL: Papilledema edema of the palpebral conjunctiva. The pupil was 3 mm., regular, but reacted sluggishly to light. The media were clear. The disc was edematous, swollen about 6 diopters, and there was marked dilatation of the veins but little increase in the diameter of the arteries. There were no hemorrhages or exudates. Vision in the left eye was 20/20, with almost the same conjunctival congestion, globe proptosis, and immobility as in the right eye. The pupil was 3 mm., and the media were clear. The papilledema was marked-6 diopters with a small central excavation. The veins were greatly distended, but there was no exudate or hemorrhages. The patient was admitted to St. Peter's Hospital. On October 4, 1937, the x-ray report was that: "All bone structure in thQ vault of the skull is normal. The vascular grooves are normal, and there is nothing unusual about the diploic markings. There are no intracranial calcifications. The sella turcica is normal for size and outline; all of the processes are well demon- strated and are negative for erosion." The anteroposterior stereograms of the skull show nothing that is in any way suggestive of intracranial pathology. None of the sinuses show evidence of acute or chronic disease. The teeth show no evidence of abscess. The Wassermann reaction to all antigens was negative. The spinal fluid was clear, and no cells were present; there was no decrease in sugar; globulin was negative and there were no organisms on smear or in culture. The urine at times showed a trace of albumin and a few granular casts. Blood examination: Hemoglobin, 70 per cent.; red blood cells, 4,312,000; white blood cells, 8,200; polymorphonuclears, 78 per cent.; small lymphocytes, 18 per cent.; large lymphocytes, 1 per cent.; eosinophiles, 3 per cent. On admission the patient complained of intense pain in the frontal region, which was so severe that she did not attempt to move her eyeballs, because this induced nausea. Her general physical examination was completely negative. On spinal puncture, the fluid was found to be clear, with a ques- tionable increase in pressure. There was no evidence of block in the Queckenstedt test. When the patient was discharged from the hospital on October 25, there was marked proptosis of the right globe, with almost complete loss of motion. The eyelids were red and swollen, with severe ciliary congestion. The disc was immensely swollen, and the retinal vessels were tortuous and distended. There were no hemorrhages or exudates. BEDELL: Papilledema 251 The left globe was more prominent than the right. The bulbar congestion and the papilledema were extreme, with several hemorrhages on the sides of the disc. Her condition was so alarming that she was sent to Dr. Horrax, in Boston, who reported as follows: Normal sensation in the face, and no facial weakness. Hearing was intact, and all the other cranial nerves were negative. No sensory or motor disparities of the upper or lower extremities existed. The ventriculogram showed that the ventricular system was entirely within the normal limits. Ventricular fluid was normal. Blood studies: Blood sugar, 60 mg.; nonprotein nitrogen, 29 mg.; sodium chlorid, 586; white blood cells, 8,550; red blood cells, 4,300,000; hemoglobin, 70 per cent. Differential blood count: polymorphonuclears, 66 per cent.; lymphocytes, 24 per cent.; large monocytes, 9 per cent.; eosinophiles, 1 per cent. Urinalysis: Specific gravity, 1,016; acid reaction; very slight trace of albumin; negative for sugar or acetone; an occa- sional red blood cell; from four to six white blood cells per high- powered field. On her return from Boston, four days later, the patient showed marked improvement. The orbital edema was much reduced. The right disc, although swollen, with pale rounded edges, did not mushroom as far as previously. The veins were smaller, and there was a very delicate macular star. The disc was so swollen that it was paler than it had been at the height of the disease. By November 30, 1937, there had been progressive improvement; the disc became paler, and was elevated about 3 diopters as com- pared with 6 diopters in October. The vessels were well outlined and almost normal in size. The left fundus was more congested than the right. The pink disc was elevated 3 diopters, and the retinal vessels were dilated, but were only half their former size. Three weeks later the mottled background of the fundus became visible for the first time; the disc was flat, pink, and sharply outlined, with many vessel branches on its surface. The left fundus was hazy-the cloudiness of edema-the disc was flat, and the vessels were normal. The patient was seen again one year later, when a follicular con- junctivitis was present. The fundi were negative then, except for a few fine, bright dots in the macular region. Another year passed uneventfully, but on December 12, 1939, she reported that her left eye had been painful for six days and that the vision had been blurred for three days. The vision of the 252 BEDELL: Papilledema right eye was 20/20. The pupil was 3.5 mm., regular, and active. The media were clear; the disc was clearly and sharply outlined, and was of normal color. The retinal vessels were negative. The eyeball was white and freely movable in all directions. The left eyelids were swollen, and a narrow palpebral fissure was present. The left eyeball was only 0.5 mm. more prominent than the right, but was intensely congested, with large conjunctival veins. The vision was 20/20. The pupil was 4 mm., regular, and active. The fundus was peculiar in that the pale pink, well- defined disc formed the border of a large, irregularly oval retinal elevation which extended to the macula, but did not go beyond it. Through the translucent retina bright subretinal reflexes were observed. These, combined with the remarkably brilliant red of both the arteries and the veins, lent an unreal, almost artificial appear- ance to the entire fundus. There was an absolute scotoma corre- sponding to the retinal lesion. The patient was three months pregnant. Five days later the entire left retina was edematous, with radiat- ing wrinkles at the macula. The disc was elevated and the vessels were distended. The right eye remained clear. On December 28, 1939, vision in the left eye was 20/50. The papilledema was 3 diopters, and the vessels curved over its prominent edges. The great retinal swelling made all fundus details in- distinct. There was a very fine granular partial macular star. She was seen the last time on January 6, 1940, three weeks after the recrudescence, at which time the bulbar congestion had disappeared. The disc was flat, and the vision 20/30. The patient was delivered at term of a healthy baby, and for the following thirteen months, according to her letter, she has been free from any ocular annoyance. That this most alarming train of signs and symptoms was not caused by an intracranial tumor was conclusively proved by the course the disease ran subsequent to the ventriculography. That the condition was not the result of active sinus disease was proved by the absence of any visible nasal pathologic findings and by the negative x-ray films. It may be that there was some compression of the central retinal vein in the orbit, but even if this were so, the etiology is, neverthe- less, a mystery. So far as is known she was not allergic and there was no marked recession after the administration of adrenalin. It is to be noted especially that she has had three attacks. The sec- BEDELL: Papilledema 253 ond was the most severe, and yet it subsided with complete physical and functional recovery. The third attack was unilateral and extreme, but was of short duration. These cases are presented to prove the value of colored stereoscopic fundus photographs when used to differentiate an inflammation of the optic nerve, a papillitis, from an edema-papilledema. Such a differential diagnosis is made with difficulty for at least two reasons: first, because clini- cians and authors confuse the terms by employing the names neuritis and choked disc interchangeably, without consider- ing the structural alterations that take place in the nerve. The second reason is the frequent similarity presented by the ophthalmoscopic picture. Years ago the statement was made that the degree of disc elevation was the essential difference, but every careful observer must admit that this is fallacious. The literature on papilledema is voluminous, and the pub- lished conclusions are contradictory. What appears to be an optic neuritis to one physician may be called papilledema by another, and a frank neuritis is not infrequently referred to as a retrobulbar neuritis. One of the most illuminating, exhaustive, and well-au- thenticated resum6s on papilledema without intracranial pressure was presented by Dandy. In his second report he recorded his experience with 44 cases, 42 of which were bilateral and two unilateral. In only one case did the papilledema recur. Dandy stated that the condition seemed to involve no danger to life, that no operation was indicated, and, so far as he knew, no treatment was of benefit. When attempting to explain these cases, the demyelinat- ing diseases-disseminated sclerosis, acute disseminated en- cephalomyelitis, neuromyelitis optica, and encephalitis peri- axialis diffusa-were constantly kept in mind, and it was impossible completely to exclude disseminated sclerosis. Berliner's review in 1935, a comprehensive, well-sustained dissertation, brought the literature up to date. Unfortunately, we cannot enter into a lengthy discussion 254 BEDELL: Papilledema here of papillitis or papilledema, so that the cases must be judged on photographic evidence, which, of course, must be used in conjunction with the clinical histories. A careful analytical study of this report supplies material for prolonged discussion, although an examination of the photographs seems to leave but little doubt that the process was basically an edema. In Case 1 there was a swelling of the nerve-head, with a small amount of exudate and a few hemorrhages. In Case 2 the nerve on the level with the retina was pink and partially obscured by considerable overlying edema, whereas in Case 3 there was a marked edema of the disc, with preservation of the large central excavation, but at no time were there hemorrhages or exudates. Case 4 showed primarily a great dilatation of the retinal veins, and later an infiltration with organization of exudate. The dilatation of the retinal veins was somewhat similar in Case 5, but the involvement was bilateral and partial optic atrophy resulted. In the last case described the papilledema was bilateral and characteristic. Later there was a recurrence in one eye, with the identical type of swelling. Whenever the optic nerve is involved, the ophthalmoscopic report should be based on the knowledge of the anatomic structures of the fundus, and careful attention should be given to the normal, thick nasal half of the disc as well as to the flatter temporal portion. Papilledema is generally unaccompanied by marked visual change unless it has been present for a considerable time. The cases here reported show that, under unusual conditions, a severe compression of the nerve fibers may lead to the initial symptom of early visual loss. Ordinarily restoration of function is prompt, but if the strangulation of the nerve is protracted, secondary atrophy may result. BEDELL: Papilledema 255 REFERENCES (The literature is too voluminous even to condense. The appended list has been selected as representative.) Allbutt, T. C.: Lancet, 1: 76, 1870 Balser, B. H.: Brain, 59: 353, 1939. Beck, G. M.: Brain, 50: 687, 1927. Bedell, A. J.: Tr. Am. Ophth. Soc. 31: 77, 1933. Bedell, A. J. Pennsylvania M. J. 35: 831, 1932. Bedell, A. J.: South. M. J. 30: 37, 1937. Behr, C.: Arch. f. Ophth. 137: 1, 1937. Berliner, M. L.: Arch. Ophth. 13: 83, 1935. Calmettes, L., and Deodati, F.: Progres m6d. 2: 1425, 1938. Champion de Crespigny, C. T.: M. J. Australia, 2: 911, 1937. Dandy, W. E.: Ann. Surg. 106: 492, 1937. Dandy, WV. E. Ann. Surg. 110: 161, 1939. Donahue, H. C.: Am. J. Ophth. 22: 661, 1939. Duke-Elder, Sir W. S.: Text-Book of Ophthalmology, St. Louis, Mosby, 3: 2944, 1941. Fetterman, J. L., and Chamberlain, W. P., Jr.: Arch. Ophth. 23: 577, 1940. Fralick, F. B., and De Jong, R. N.: Am. J. Ophth. 20: 1119, 1937. Fritch, J. S.: M. Clin. North America, 23: 1695, 1939. Goulden, C.: Tr. Ophth. Soc. U. Kingdom, 34: 229, 1914. Gowers, Sir W. R.: A Manual and Atlas of Medical Ophthalmoseopy, Phila., Blakiston, 1890. Griffith, J. Q., Jr.; Fry, W=. E., and McGuinness, A.: Am. J. Ophth. 23: 245, 1940. Griffith, J. Q., Jr.; Jeffers, W. A., and Fry, W. E.: Arch. Int. Med. 61: 880,1938. Holmes, G.: Brit. J. Ophth. 21: 337, 1937. Joyce, A.: M. J. Australia, 2: 906, 1937. Kamman, G. R.: Am. J. Ophth. 20: 1132, 1937. Lamb, H. D.: Am. J. Ophth. 23: 408, 1940. Marburg, O.: Ztschr. f. Augenh. 44: 125, 1920. Marshall, D., and Laird, R. G.: Am. J. Ophth. 19: 1085, 1936. Noyes, H. D.: Arch. f. Augenh. 10: 331, 1880-81. Paton, L.: Arch. Ophth. 15: 1, 1936. Perritt, R. A.: Arch. Ophth. 11: 492, 1934. Reese, A. B.: Tr. Am. Ophth. Soc. 28: 341, 1930. Rehwald, E.: Med. Klin. 35: 1108, 1939. Samuels, B.: Tr. Ophth. Soc. U. Kingdom, 57: pt. 2, 529, 1937. Schieck, F.: Arch. f. Ophth. 138: 48, 1937. Seguin, E. C.: J. Nerv. & Ment. Dis. 7: 177, 1880. Stough, J. T.: Arch. Ophth. 8: 821, 1932. Sugar, S.: Am. J. Ophth. 22: 135, 1939. Van Heuven, J. A., and Fischer, P. F.: Brit. J. Ophth. 20: 204, 1936. Van Heuven, J. A.: Tr. Ophth. Soc. U. Kingdom, 58: pt. 2, 549, 1938. Walsh, F. B.: Bull. Johns Hopkins Hosp. 56: 183, 1935. Williamson-Noble, F. A.: Clin. J. 62: 483, 1933. DISCUSSION DR. ALBERT N. LEMOINE, Kansas City, Mo.: I have seen three cases of papilledema with apparently no increased intracranial pressure. The first two were seen thirteen years ago. The first patient was a girl, aged twelve years, whose past history was essentially negative, except that she had never menstruated. She con- 256 BEDELL: Papilledema sulted me for headaches and failing vision in both eyes. On the first examination she had five diopters of swelling of each disc, a bitemporal contraction of the visual fields of 20 degrees, and the vision was reduced to 20/30 - in both eyes. She was sent to the hospital, and a complete physical and laboratory examination was made, including a Wassermann test and x-rays of the sinuses and skull. The results were entirely negative, except that the sella turcica showed an increase in the fossa of about 2 mm. Suspecting that she might have a pituitary tumor, a neuro-surgeon who was called in consultation suggested a spinal puncture. The morning on which we were to do the spinal puncture the patient's mother met me and said: " You are not going to do a spinal puncture. I have told you that as soon as my daughter menstruates she will be all right, and she is menstruating this morning." She came in to see me six weeks later-the papilledema was gone and the fields and vision were normal, and they have remained so. The second case was seen during the same year. The patient, a woman aged twenty-one years, had four diopters of swelling of the optic discs, and 25 degrees of bitemporal contraction of the visual fields with a slight enlargement of the sella turcica. The vision was reduced to 20/30- in each eye. Complete physical and laboratory examinations were made, and were all negative. This patient's mother suggested-" All that is the matter with my daughter is that she is in love; she will get well when she gets married." No therapeutic measures were used. I observed her for three months without finding any change in her condition. The girl married and came back two months later, having, in the meantime, become pregnant. The papilledema was gone; the fields were normal; the vision was normal. The third case is under my observation at the present time. I saw the patient on March 27. She is a girl, aged thirteen years. When she came she was totally blind in the right eye, with only light perception in the left eye. She had four diopters of swelling in the left eye, and five diopters in the right eye. The laboratory examinations and x-ray examination of the sinuses and skull were completely negative. A neuro-surgeon insisted that there must be something to account for this, and a spinal puncture was made; this measured 13 mm. of mercury; not being satisfied with this, an encephalogram was made which was negative. This girl's history was essentially negative, except that she had never menstruated, and had had blurred vision for a few weeks preceding the onset of her blindness, which came on suddenly, and was accom- BEDELL: Papilledema 257 panied by severe pelvic pain. She received no treatmenit. I saw her on May 27, and her vision at that time was 20/200; the fields were normal, and the swelling was completely gone. DR. WALTER I. LILLIE, Philadelphia, Pa.: Apparently there is considerable confusion among ophthalmologists concerning the in- terpretation of edema of the disc. An inflammation, regardless of whether it is in the optic nerve or in the soft tissues elsewhere, has five cardinal characteristics. Whether we use the Greek or the English terms, the four important features are swelling, loss of function, hyperemia, and heat. Dr. Bedell's presentation is, in my opinion, a classic description of five cases of acute optic neuritis. We see many fundus conditions that exhibit edema of the disc, or papilledema. It occurs in thrombosis of the central retinal vein; in increased intracranial pressure, commonly called choked disc; in the retinitis of angiospastic and malignant hypertension; in the retinitis of nephritis, and in optic neuritis. In the case of an individual who has a precipitous loss of central visual acuity and in whom the fundal examination shows edema of the disc, the diagnosis, so far as I am concerned, is optic neuritis. In the group of cases with neurologic findings, headaches, an& the like, in whom we see as much edema of the disc as, or more than, we might see in acute optic neuritis, when due to increased intracranial pressure, there is no precipitous loss of central visual acuity. Edema of the disc per se does not cause loss of central visual acuity, and when it is lost, and lost precipitously, it is one of the cardinal signs of inflammation, namely, loss of function in that particular tissue at that particular time. Dr. Bedell had no time to give a detailed report of all his cases, but from his description of the fundal changes I would not hesitate to say that each one is a case of acute optic neuritis. The process is the same in both retrobulbar neuritis and optic neuritis, but in retrobulbar neuritis the inflammation is behind the globe. The clinical course is similar except that the prog- nosis in retrobulbar neuritis is very much better than it is in optic neuritis because the fibers can readily stand swelling in the optic nerve where there is no compression. But when the inflammation is at the optic disc, these nerve fibers are pinched against the lamina cribrosa and are damaged much more rapidly, and the return of good central visual acuity in optic neuritis is usually much slower than it is in retrobulbar neuritis. Permanent visual changes in retrobulbar neuritis generally occur when the optic nerve is involved in the optic canal, as here again the inflammation is confined to a small bony canal and destruction of the fibers occurs much 258 BEDELL: Papilledema more readily. Dr. Bedell has shown very excellent photographs of papilledema in cases with precipitously lost central visual acuity. To me this is a typical clinical syndrome of acute optic neuritis, and there should, be no associated increased intracranial pressure. DR. RALPH 0. RYCHENER, Memphis, Tenn.: It is with some hesitancy that I discuss Dr. Bedell's presentation, as I am not certain that the patient whose optic discs I wish to show correctly falls into this particular group. However, it seems to me that one eye must be classified correctly as papilledema, whereas the other is apparently a true optic neuritis. As this has been a puzzling case to us, further discussion may prove valuable in determining the true etiology. W. M., male, aged twenty-three years, a book- keeper, suddenly, about December 1, 1940, lost good central vision in the left eye. He was referred to us by Dr. J. D. Carlton on January 14, 1941, when the visual acuity recordings were: O.D. = 6/7.5 and Jaeger 6, improved by +3.75 sph. to 6/5 and Jaeger 1. O.S. = 3/60, improved by +4 sph. to 6/60 and Jaeger 8. The refractive error under homatropin was: O.D. = +4.50 sph. O.S. = +4.50 +0.50 cyl. ax. 750 with similar visual acuity ratings. The left eye exhibited an absolute centrocaecal scotoma with full form field. The optic disc was edematous six to seven diopters, the edema covering the vessels on the surface of the disc, and the retina between the disc and fovea being drawn so tautly as to produce retinal striae. Larger plaques of deep retinal hemorrhage encircled the swollen nerve-head. Al- though there were no visual symptoms, field or acuity changes, other than an enlarged blind spot, associated with the right eye, we were astonished to find there a papilledema of five to six diopters, with some cytoid bodies on the temporal border. Since the onset of the present illness there had been absolutely no physical signs or symptoms save the sudden visual failure of the left eye, and during the course of observation to date not even a headache has been noticed. X-rays of the skull showed normal sinuses, sella, suture lines, and optic foramina, the last being 4 x 5 mm. in diameter. The general physical examination, including spinal fluid tests, blood studies, and tests for undulant fever, was entirely normal. The patient was treated for the optic neuritis of the left eye by foreign protein therapy with typhoid H antigen, huge intra- muscular injections of thiamin chlorid, and by internal medication BEDELL: Papilledema 259 with potassium iodid and mercury bichlorid. Oni February 4 visual acuity had improved to 6/20, and two wieeks later it was 6/7.5 and J. 1, at which point it has remained to (late. Frequent perimetrie examinations have beeni recorded, and the central scotoma disappeared from the left field synchronously with the improvement in central acuity. An enlarged blind spot is now the only abnormal recording on the left eye. The right eye has remained normal both as to visual acuity and as to visual field. There has been a slight recession in the papilledema on both sides during the past two months, the right disc now being elevated four diopters, and the left six diopters. It is possible that future investigation will teaeh uis more concerning the etiology of this unusual eondition, but at present this patient is at work, and feels and believes that he is entirely well. Because of the fact that this individual had four to five diopters of hyperopia, we believed that we might be dealing with a pseudo- papillitis in a patient who happened to develop an optic neuritis. However pseudo-papillitis does not recede under observation, and in this case there has been a recession of two diopters of swelling while the patient was under observation. A suggestion has been made that our patient might have the drusen which Dr. Samuels has described. However, here again I do not see how we can expect a recession of the swelling of the optic disc. Another suggestion was that we were dealing with an allergy, as such cases of allergic papilledema had been observed. I have not recognized any such cases, but I see no good reason why it could not be so. Perhaps Dr. Bedell's last case, in which the photograph shows an edema of the lids, illustrates such an example. If the discussion which is to follow will throw light upon what is the trouble with our patient, I will be grateful. DR. F. H. VERHOEFF, Boston, Mass.: I wish to express my appreciation of Dr. Lillie's discussion. I tried to get recognition before he did, but I am glad that I failed, since he has expressed what I had in mind much better than I could have expressed it, and has brought out some points that I would not have mentioned but in which I concur. However, he failed to mention one important symptom, namely, pain. Dr. Bedell and the other speakers also neglected to refer to this. Pain on pressure and on movement is characteristic of acute retrobulbar neuritis. I use this term whether the lesion is immediately behind the globe or further back; that is to say, whether or not there is edema of the optic disc. I always regard such cases as cases of multiple sclerosis, even if the patient 260 BEDELL: Papilledema never develops any other symptom of this disease. I believe that multiple sclerosis can be confined chiefly to the optic nerve. DR. H. M. LANGDON, Philadelphia, Pa.: In his interesting talk Dr. Bedell has raised the question of differential diagnosis between papilledema and optic neuritis. In many of his cases-most of them, I believe-there was a very abrupt loss of vision. Our classic example of papilledema is that which occurs in a frank intracranial neoplasm with increased intracranial pressure. There the vision does not deteriorate rapidly. In many cases we watch the patient for weeks before we find that there is any decided loss in central vision. Some of Dr. Bedell's cases lasted only twelve to thirteen days, during which time, I understand, there was a distinct loss of vision, and then an abrupt return to normal conditions. In his last patient, - the one who had a swollen lid and was pregnant, - he did not, so far as I could gather, describe the visual condition before the attacks; in the other cases it seemed to me that there was a very abrupt loss of vision, and an abrupt return of vision. That is not, in my opinion, consonant with a mere papilledema without actual inflammation of the tissues, but it does fit in perfectly with conditions which we find in acute ocular diseases. I can recall two cases- one which I saw a week ago; this man came in saying that he had had an abrupt loss of vision in the right eye three days before. His vision was 4/60, with a frank paracentral scotoma. He gave a history of acute phlegmonous tonsillitis-quinsy, he called it- six weeks earlier. His physician told him that his tonsils were in bad condition. The tonsils were removed on Monday, and on Wednesday his vision had improved from 4/60 to 5/12. He had a distinct edema of the disc, and the margins were definitely blurred. When I saw him on Wednesday the discs were very much clearer, and the visual improvement seemed quite miraculous. I believe that we had here a frank inflammation of the nerves, involving the central fibers, which presented a picture that would have been comparable to some of those which Dr. Bedell showedl. Like Dr. Lemoine, I cannot believe that most of these cases are papilledema, but I regard them as examples of a definite inflammation of the nerve with definite swelling. DR. A. J. BEDELL, closing: I thank all those who have taken part in the discussion, for, as I stated in the paper, the object was to promote discussion. I was very glad to hear Dr. Lemoine's excellent and pointed dliscussionl. GIFFORD: Ring Ulcer of the Cornea 261 Dr. Lillie spoke of the classic signs of inflammation and stressed edema. He also stated that the good end result in retrobulbar neuritis was in marked contrast to the poor result following optic neuritis. He did not assert that papilledema was absent in the cases reported by me. In answer to Dr. Rychener's question: Patient No. 6 was thor- oughly tested by a competent allergist, who reported that there were no reactions. Dr. Verhoeff raised a very interesting question, one that proved how often anatomy is forgotten. He stated that one of the characteristics of inflammation was pain on motion of the eyeball and tenderness of the globe to touch. That statement is found in text- books; but surely swelling of the optic nerve as it impinges on the fine nerve fibers which encircle the nerve in the region of the optic foramen may come from fluid pressure as well as from inflammatory exudate. Dr. Langdon declared that an abrupt improvement of vision was a sign that the condition was inflammatory. Greater experience will teach him that sudden release of fluid reduces pressure much more rapidly than does the resolution of inflammatory products. I am grateful to those who discussed the paper, especially to Dr. Lemoine and Dr. Rychener.

RING ULCER OF THE CORNEA*

SANFORD R. GIFFORD Chicago, Ill. A review of the literature reveals the fact that a certain confusion exists with regard to conditions that come under the head of ring ulcer of the cornea. Fuchs,' in 1893, described what he called "metastatic marginal ulcer." This ulcer occurred in aged persons with multiple fine punctate infiltrations in the superficial parenchyma near the limbus. "For a time," Fuchs states, "they resemble marginal superficial punctate keratitis, but frequently break down to form * From the Department of Ophthalmology, Northwestern University Med- ical School, Chicago, Illinois.