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New York Society for Clinical Ophthalmology
SOCIETY PROCEEDINGS
92
whether anyone had seen anything like this before. Discussion. Mr. Adams said that he presumed it might have been traumatic if, as a result of concussion, he had extensive choroidal hemorrhage with damage to the macula, the retinitis having developed subsequent to that. QUESTIONARLE CONJUNCTIVAL EPITHELIOMA. QUESTIONABLE ATYPICAL MOORE N ' S ULCER SUPERIMPOSED ON OLD M U S TARD-GAS DAMAGE MR. ALAN HOLMES-SMITH- presented
a man, aged 60 years. There was a history of damage to the left eye by mustard gas in 1918. At that time he was hospitalized and treated for three months. During the past two or three months the lids of the left eye had become swollen. The swelling receded with the use of hot compresses. For three weeks prior to this examination there was ptosis of the left eyelid. The vision was R.E. 6/24, L.E. ability to count fingers. The right eye was normal on examination. The left eye showed an inactive pupil and no red reflex. The tension was normal. There was a firm raised area of conjunctiva below and medial to the limbus, extending into the lower fornix; the edge was raised. The lower nasal quadrant of the cornea was involved in the lesion, and an opaque area separated it from normal cornea. No enlarged glands were palpable in the neck. Mr. Holmes-Smith said that there was some difference of opinion as to the diagnosis, whether it was an atypical Mooren's ulcer or a new growth. It was finally decided to enucleate the eye, and that was done completely with as much conjunctiva as could be taken from the lower fornix. On section the tumor was thought to be an epithelioma with considerable inflammatory reaction, with the sclera
undergoing invasion at the limbus. A course of radiotherapeutic treatment has been advised. NEW YORK SOCIETY FOR CLINICAL OPHTHALMOLOGY December 4, 1944 DR. MILTON SYPHILITIC
L.
OPTIC
BERLINER, ATROPHY
presiding AND
TRY-
PARSAMIDE AMBLYOPIA DR. H. HOUSTON MERRITT said that syphilitic optic atrophy may be secondary to meningeal involvement of the nerve or luetic chorioretinitis, but more frequently it is primary. Primary luetic optic atrophy may be the only manifestation of the disease, although more frequently it is seen with tabes dorsalis. The exact pathogenesis is not clearly elucidated but it is probably due to primary involvement of the nerve itself or its blood vessels by the disease. Spirochetes have been found pathologically in only two cases, both by Igersheimer. The characteristic visual defect consists of loss of central field with concentric or sector-shaped peripheral constriction. The course is progressively downhill and if one eye is involved the other may be expected to show the same condition within seven years. Useful vision may likewise be expected to be lost within seven years after the condition is recognized. Until recent years treatment has been ineffective, whether in the form of trivalent arsenicals, tryparsamide, or the Swift-Ellis method of arsenical treatment. Fever therapy is accepted today and if instituted sufficiently early, offers a 60-percent chance of arresting the condition. Penicillin has not been in use for a long enough period of time to determine its usefulness.
Optic atrophy may be caused by tryparsamide. It is known that other pentavalent arsenicals have an affinity for the
SOCIETY PROCEEDINGS optic nerve in experimental animals. It has been found that 8 percent of patients under tryparsamide treatment experience some visual phenomenon. In half of these patients this is purely subjective and not serious. A few hours after the injection the patient's vision is blurred for several hours by a shining mist, such as may be seen over a hot radiator. This will not recur if the tryparsamide is discontinued, but if the drug is continued blindness can develop with the fundus appearing normal. This blindness improves later with the return of central vision, but the visual field continues to be of a funnel type. Atrophy becomes detectable after several weeks. This visual disturbance usually occurs after the first few tryparsamide injections and is unusual, but possible, after the eighth injection. Although it has been claimed that the tryparsamide does not directly induce the blindness but lights up syphilis already in the eye, the great speed of the appearance of the blindness (three hours) indicates that the drug itself is at fault. Also, there have been cases of blindness after tryparsamide administration to nonsyphilitic individuals. When tryparsamide is administered there are three safeguards against these complications: (1) ophthalmoscopic and visual-field examinations are performed to weed out abnormal cases, as these show 10 times the frequency of the complications; (2) only small doses should be administered at first; and (3) be on the alert for subjective symptoms. Discussion. Dr. James W. Smith thought that Dr. Merritt stressed a point of practical importance regarding the poor visual prognosis in tabes either with or without therapy. If the inexperienced ophthalmologist fails to recognize this, he will be blamed by the patient for authorizing a therapy that induced blindness. Close cooperation is essential between the syphilologist and eye physician before
93
therapy with tryparsamide is started. Unfortunately, in some cases wherein there is vision and field loss other systemic disease may contraindicate fever therapy. Unless penicillin is proved effective against cerebrospinal syphilis, the doctor will still be confronted with a dilemma. Dr. Merritt thanked Dr. Smith for reemphasizing the importance of close cooperation between the ophthalmologist and syphilologist in the treatment of these cases of optic atrophy. Although some claim that all patients with syphilitic optic atrophy will be blind within seven years, he said that he did not believe that this was necessarily true. It is probable that some cases come to a spontaneous arrest. He did not believe justified the feeling of some ophthalmologists and syphilologists that tryparsamide can be used in the treatment of patients with optic atrophy. At the present time fever treatment is the method of choice. When using tryparsamide in the treatment of neurosyphilis, one should be very careful to watch for the development of visual symptoms, as it is a powerful and dangerous weapon. INVOLVEMENT OF THE OCULAR SYSTEM IN DEMYELINATING DISEASES
DR. ARMANDO FERRARO presented a
table summarizing the most important manifestations of involvement of the ocular system in various types of demyelinating diseases; such as multiple sclerosis, diffuse sclerosis, Schilder's disease, Devic's disease, Balo's disease, Krabbe's disease, and so forth. He attempted to establish that from the clinical as well as the pathologic standpoint, all these various supposedly clinico-pathologic entities are nothing else but variants of the same fundamental process of demyelination of the central nervous system, in relation to age at onset, resistance of the individual, and virulence of the pathogenic factor.
94
SOCIETY PROCEEDINGS
He attempted further to make a comparison between the most important features of the histopathologic process of the various demyelinating diseases with the main features of allergic reaction of the brain. The material for such a comparison came from the Department of Neuropathology of the New York State Psychiatric Institute where since 1940 Dr. Ferraro, first in collaboration with Jervis, and then with the Kopeloffs, investigated the reaction of the nervous tissue to experimental allergy. Briefly, the essential features of experimental allergy, such as demyelination, vascular infiltration—first with polymorphonuclear cells and then with lymphocytes and histiocytes—reactive pliosis, presence of edema, occasional hemorrhages, presence of granulomatouslike formation with giant cells, occasional thrombi of small blood vessels, and occasional endarteritis, are all features which may be found in the so-called demyelinating diseases. Dr. Ferraro drew the conclusion that the pathology of the demyelinating diseases including the pathology of the ocular system may be viewed also in the light of an allergic reaction. Discussion. Dr. Daniel Kravitz said that ophthalmologists are dismayed by the large number of names which describe apparently similar conditions. The situation is analogous to the practice of attaching names to every corneal spot which appears somewhat different from the others. Regarding the possible allergic basis for the causation of the demyelinating diseases, Dr. Kravitz believed it too great a simplification. Here again one is reminded of the variety of ocular conditions explained by some as due to a single cause, vasoconstriction. Allergy as a cause for the demyelinating diseases does not explain the large number of familial cases in which patients reach a certain station in life and then are attacked by these diseases.
T O P I C A L DIAGNOSIS OF DISTURBED OCULOMOTOR MOTILITY DR.
ALFRED
KESTENBAUM
differen-
tiated between types of palsy of muscles supplied by the oculomotor nerve: (1) Nuclear type: The arrangement of the centers for the individual eye muscles in the third-nerve nucleus is still controversial but it is more or less accepted that the cranial (superior) part represents the homolateral levator and superior rectus. Below these centers is the center for the inferior oblique of the same side. The inferior part of the nucleus and the neighboring trochlear nucleus represent the contralateral inferior rectus and superior oblique, respectively, and their fibers must cross the midline. The medial rectus is represented in the medial parts of both oculomotor nuclei. Briefly, the elevators have their centers homolaterally, the depressors contralaterally, and the medial rectus bilaterally. Owing to this complex arrangement a single focus in the nucleus cannot involve all the muscles supplied by the oculomotor nerve of one eye, and complete oculomotor palsy of one eye excludes a nuclear lesion. Partial oculomotor palsy is more frequent if the lesion is nuclear than if it is subnuclear. Bilateral paresis of single muscles indicates a nuclear lesion. (2) Dorsal fascicular type: A lesion here, usually accompanied by involvement of the nucleus ruber or of the substantia nigra, shows also a disturbance of the tonus, muscular coordination, and sometimes sensibility of the opposite half of the body. This corresponds to the old "Benedict's syndrome," oculomotor palsy, and crossed hemitremor. (3A) Ventral fascicle type: Here a lesion is usually associated with a pyramidal-tract lesion in the pes pedunculi, resulting in Weber's syndrome, oculomotor palsy, and crossed hemiplegia. (3B) Root type: A lesion of the root
SOCIETY PROCEEDINGS of the third nerve, after it leaves the brain stem at the medial side of the peduncle, also results in a Weber's syndrome, which therefore is ambiguous, being due to a lesion in the brainstem or one in the interpeduncular region. (4) Basal type: After leaving the brainstem, the third nerve passes forward in the subarachnoid space at the base of the brain, first mesial, then below the peduncle, and then between the posterior cerebral artery and the superior cerebellar artery for a total of 2 cm. before perforating the arachnoid and dura to enter the extradural space. In its "basal" portion the nerve may be damaged by basal meningitis, a tumor, or especially an aneurysm of one of the aforementioned arteries. This type of oculomotor palsy may be monosymptomatic and its topical diagnosis very difficult. A Wassermann test and arteriography, which is not harmless, may aid localization. (5) and (6) Posterior and anterior cavernous-sinus types: From the point of penetration of the dura until it reaches the superior orbital fissure, the nerve runs within the wall of the cavernous sinus. In addition to the oculomotor palsy, there may be signs of a lesion of the cavernous sinus and involvement of the neighboring fourth, fifth, and sixth nerves. The first and second branches of the trigeminal may be involved only in the posterior cavernous-sinus type of lesion, whereas in the more anterior lesions only the first branch of the fifth nerve is implicated; while a thrombosis of the cavernous sinus and an arteriovenous aneurysm of the carotid artery without venous communication may be symptomless except for the palsy and therefore resemble the basal type of lesion. (7) Orbital-fissure type: Here the third, fourth, fifth, and sixth nerves are involved, but there are no signs of a cavernous-sinus lesion.
95
(8) Orbital apex type: A lesion in the apex of the orbit presents signs of third-, fourth-, fifth-, and sixth-nerve disease and also optic-nerve involvement, with central scotoma progressing to blindness, temporal pallor becoming optic atrophy, and, if the lesion takes space, exophthalmos is also present. (9) Muscular type: The previously mentioned types of third-nerve palsy must be differentiated from lesions in the muscles themselves, as occurring in the nerve endings in myasthenia gravis, or with orbital tumors. In the muscular type of lesions either all or part of the extraocular muscles are involved, and in the latter case the muscles are involved independently of their innervation. For example, a tumor above the eye may involve the superior rectus and oblique muscles, which are innervated by different nerves. (10) Neuritis type: Neuritis, or other processes restricted to the nerve itself, as in diabetes, infections, intoxications, may produce monosymptomatic oculomotor palsy which may be confused with the basal type. Discussion. Dr. Percy Fridenberg pointed out that a very early symptom of fifth-nerve involvement in orbital-apex and fissure lesions is silent and must be elicited. This is anesthesia of the frontal branch, missed by most ophthalmologists, but later becoming a severe neuralgia. Dr. Kestenbaum stated that the diagnosis of anesthesia of the trigeminus cannot be based upon the loss of the corneal reflex alone, but all regions supplied by the nerve must be tested. X-ray studies are of great importance in topical diagnosis but were not discussed. It should be emphasized that the final diagnosis is made by the neurologist; that the ophthalmologist can only interpret the ocular sign to further his purpose. Leon H. Ehrlich, Secretary.
92
whether anyone had seen anything like this before. Discussion. Mr. Adams said that he presumed it might have been traumatic if, as a result of concussion, he had extensive choroidal hemorrhage with damage to the macula, the retinitis having developed subsequent to that. QUESTIONARLE CONJUNCTIVAL EPITHELIOMA. QUESTIONABLE ATYPICAL MOORE N ' S ULCER SUPERIMPOSED ON OLD M U S TARD-GAS DAMAGE MR. ALAN HOLMES-SMITH- presented
a man, aged 60 years. There was a history of damage to the left eye by mustard gas in 1918. At that time he was hospitalized and treated for three months. During the past two or three months the lids of the left eye had become swollen. The swelling receded with the use of hot compresses. For three weeks prior to this examination there was ptosis of the left eyelid. The vision was R.E. 6/24, L.E. ability to count fingers. The right eye was normal on examination. The left eye showed an inactive pupil and no red reflex. The tension was normal. There was a firm raised area of conjunctiva below and medial to the limbus, extending into the lower fornix; the edge was raised. The lower nasal quadrant of the cornea was involved in the lesion, and an opaque area separated it from normal cornea. No enlarged glands were palpable in the neck. Mr. Holmes-Smith said that there was some difference of opinion as to the diagnosis, whether it was an atypical Mooren's ulcer or a new growth. It was finally decided to enucleate the eye, and that was done completely with as much conjunctiva as could be taken from the lower fornix. On section the tumor was thought to be an epithelioma with considerable inflammatory reaction, with the sclera
undergoing invasion at the limbus. A course of radiotherapeutic treatment has been advised. NEW YORK SOCIETY FOR CLINICAL OPHTHALMOLOGY December 4, 1944 DR. MILTON SYPHILITIC
L.
OPTIC
BERLINER, ATROPHY
presiding AND
TRY-
PARSAMIDE AMBLYOPIA DR. H. HOUSTON MERRITT said that syphilitic optic atrophy may be secondary to meningeal involvement of the nerve or luetic chorioretinitis, but more frequently it is primary. Primary luetic optic atrophy may be the only manifestation of the disease, although more frequently it is seen with tabes dorsalis. The exact pathogenesis is not clearly elucidated but it is probably due to primary involvement of the nerve itself or its blood vessels by the disease. Spirochetes have been found pathologically in only two cases, both by Igersheimer. The characteristic visual defect consists of loss of central field with concentric or sector-shaped peripheral constriction. The course is progressively downhill and if one eye is involved the other may be expected to show the same condition within seven years. Useful vision may likewise be expected to be lost within seven years after the condition is recognized. Until recent years treatment has been ineffective, whether in the form of trivalent arsenicals, tryparsamide, or the Swift-Ellis method of arsenical treatment. Fever therapy is accepted today and if instituted sufficiently early, offers a 60-percent chance of arresting the condition. Penicillin has not been in use for a long enough period of time to determine its usefulness.
Optic atrophy may be caused by tryparsamide. It is known that other pentavalent arsenicals have an affinity for the
SOCIETY PROCEEDINGS optic nerve in experimental animals. It has been found that 8 percent of patients under tryparsamide treatment experience some visual phenomenon. In half of these patients this is purely subjective and not serious. A few hours after the injection the patient's vision is blurred for several hours by a shining mist, such as may be seen over a hot radiator. This will not recur if the tryparsamide is discontinued, but if the drug is continued blindness can develop with the fundus appearing normal. This blindness improves later with the return of central vision, but the visual field continues to be of a funnel type. Atrophy becomes detectable after several weeks. This visual disturbance usually occurs after the first few tryparsamide injections and is unusual, but possible, after the eighth injection. Although it has been claimed that the tryparsamide does not directly induce the blindness but lights up syphilis already in the eye, the great speed of the appearance of the blindness (three hours) indicates that the drug itself is at fault. Also, there have been cases of blindness after tryparsamide administration to nonsyphilitic individuals. When tryparsamide is administered there are three safeguards against these complications: (1) ophthalmoscopic and visual-field examinations are performed to weed out abnormal cases, as these show 10 times the frequency of the complications; (2) only small doses should be administered at first; and (3) be on the alert for subjective symptoms. Discussion. Dr. James W. Smith thought that Dr. Merritt stressed a point of practical importance regarding the poor visual prognosis in tabes either with or without therapy. If the inexperienced ophthalmologist fails to recognize this, he will be blamed by the patient for authorizing a therapy that induced blindness. Close cooperation is essential between the syphilologist and eye physician before
93
therapy with tryparsamide is started. Unfortunately, in some cases wherein there is vision and field loss other systemic disease may contraindicate fever therapy. Unless penicillin is proved effective against cerebrospinal syphilis, the doctor will still be confronted with a dilemma. Dr. Merritt thanked Dr. Smith for reemphasizing the importance of close cooperation between the ophthalmologist and syphilologist in the treatment of these cases of optic atrophy. Although some claim that all patients with syphilitic optic atrophy will be blind within seven years, he said that he did not believe that this was necessarily true. It is probable that some cases come to a spontaneous arrest. He did not believe justified the feeling of some ophthalmologists and syphilologists that tryparsamide can be used in the treatment of patients with optic atrophy. At the present time fever treatment is the method of choice. When using tryparsamide in the treatment of neurosyphilis, one should be very careful to watch for the development of visual symptoms, as it is a powerful and dangerous weapon. INVOLVEMENT OF THE OCULAR SYSTEM IN DEMYELINATING DISEASES
DR. ARMANDO FERRARO presented a
table summarizing the most important manifestations of involvement of the ocular system in various types of demyelinating diseases; such as multiple sclerosis, diffuse sclerosis, Schilder's disease, Devic's disease, Balo's disease, Krabbe's disease, and so forth. He attempted to establish that from the clinical as well as the pathologic standpoint, all these various supposedly clinico-pathologic entities are nothing else but variants of the same fundamental process of demyelination of the central nervous system, in relation to age at onset, resistance of the individual, and virulence of the pathogenic factor.
94
SOCIETY PROCEEDINGS
He attempted further to make a comparison between the most important features of the histopathologic process of the various demyelinating diseases with the main features of allergic reaction of the brain. The material for such a comparison came from the Department of Neuropathology of the New York State Psychiatric Institute where since 1940 Dr. Ferraro, first in collaboration with Jervis, and then with the Kopeloffs, investigated the reaction of the nervous tissue to experimental allergy. Briefly, the essential features of experimental allergy, such as demyelination, vascular infiltration—first with polymorphonuclear cells and then with lymphocytes and histiocytes—reactive pliosis, presence of edema, occasional hemorrhages, presence of granulomatouslike formation with giant cells, occasional thrombi of small blood vessels, and occasional endarteritis, are all features which may be found in the so-called demyelinating diseases. Dr. Ferraro drew the conclusion that the pathology of the demyelinating diseases including the pathology of the ocular system may be viewed also in the light of an allergic reaction. Discussion. Dr. Daniel Kravitz said that ophthalmologists are dismayed by the large number of names which describe apparently similar conditions. The situation is analogous to the practice of attaching names to every corneal spot which appears somewhat different from the others. Regarding the possible allergic basis for the causation of the demyelinating diseases, Dr. Kravitz believed it too great a simplification. Here again one is reminded of the variety of ocular conditions explained by some as due to a single cause, vasoconstriction. Allergy as a cause for the demyelinating diseases does not explain the large number of familial cases in which patients reach a certain station in life and then are attacked by these diseases.
T O P I C A L DIAGNOSIS OF DISTURBED OCULOMOTOR MOTILITY DR.
ALFRED
KESTENBAUM
differen-
tiated between types of palsy of muscles supplied by the oculomotor nerve: (1) Nuclear type: The arrangement of the centers for the individual eye muscles in the third-nerve nucleus is still controversial but it is more or less accepted that the cranial (superior) part represents the homolateral levator and superior rectus. Below these centers is the center for the inferior oblique of the same side. The inferior part of the nucleus and the neighboring trochlear nucleus represent the contralateral inferior rectus and superior oblique, respectively, and their fibers must cross the midline. The medial rectus is represented in the medial parts of both oculomotor nuclei. Briefly, the elevators have their centers homolaterally, the depressors contralaterally, and the medial rectus bilaterally. Owing to this complex arrangement a single focus in the nucleus cannot involve all the muscles supplied by the oculomotor nerve of one eye, and complete oculomotor palsy of one eye excludes a nuclear lesion. Partial oculomotor palsy is more frequent if the lesion is nuclear than if it is subnuclear. Bilateral paresis of single muscles indicates a nuclear lesion. (2) Dorsal fascicular type: A lesion here, usually accompanied by involvement of the nucleus ruber or of the substantia nigra, shows also a disturbance of the tonus, muscular coordination, and sometimes sensibility of the opposite half of the body. This corresponds to the old "Benedict's syndrome," oculomotor palsy, and crossed hemitremor. (3A) Ventral fascicle type: Here a lesion is usually associated with a pyramidal-tract lesion in the pes pedunculi, resulting in Weber's syndrome, oculomotor palsy, and crossed hemiplegia. (3B) Root type: A lesion of the root
SOCIETY PROCEEDINGS of the third nerve, after it leaves the brain stem at the medial side of the peduncle, also results in a Weber's syndrome, which therefore is ambiguous, being due to a lesion in the brainstem or one in the interpeduncular region. (4) Basal type: After leaving the brainstem, the third nerve passes forward in the subarachnoid space at the base of the brain, first mesial, then below the peduncle, and then between the posterior cerebral artery and the superior cerebellar artery for a total of 2 cm. before perforating the arachnoid and dura to enter the extradural space. In its "basal" portion the nerve may be damaged by basal meningitis, a tumor, or especially an aneurysm of one of the aforementioned arteries. This type of oculomotor palsy may be monosymptomatic and its topical diagnosis very difficult. A Wassermann test and arteriography, which is not harmless, may aid localization. (5) and (6) Posterior and anterior cavernous-sinus types: From the point of penetration of the dura until it reaches the superior orbital fissure, the nerve runs within the wall of the cavernous sinus. In addition to the oculomotor palsy, there may be signs of a lesion of the cavernous sinus and involvement of the neighboring fourth, fifth, and sixth nerves. The first and second branches of the trigeminal may be involved only in the posterior cavernous-sinus type of lesion, whereas in the more anterior lesions only the first branch of the fifth nerve is implicated; while a thrombosis of the cavernous sinus and an arteriovenous aneurysm of the carotid artery without venous communication may be symptomless except for the palsy and therefore resemble the basal type of lesion. (7) Orbital-fissure type: Here the third, fourth, fifth, and sixth nerves are involved, but there are no signs of a cavernous-sinus lesion.
95
(8) Orbital apex type: A lesion in the apex of the orbit presents signs of third-, fourth-, fifth-, and sixth-nerve disease and also optic-nerve involvement, with central scotoma progressing to blindness, temporal pallor becoming optic atrophy, and, if the lesion takes space, exophthalmos is also present. (9) Muscular type: The previously mentioned types of third-nerve palsy must be differentiated from lesions in the muscles themselves, as occurring in the nerve endings in myasthenia gravis, or with orbital tumors. In the muscular type of lesions either all or part of the extraocular muscles are involved, and in the latter case the muscles are involved independently of their innervation. For example, a tumor above the eye may involve the superior rectus and oblique muscles, which are innervated by different nerves. (10) Neuritis type: Neuritis, or other processes restricted to the nerve itself, as in diabetes, infections, intoxications, may produce monosymptomatic oculomotor palsy which may be confused with the basal type. Discussion. Dr. Percy Fridenberg pointed out that a very early symptom of fifth-nerve involvement in orbital-apex and fissure lesions is silent and must be elicited. This is anesthesia of the frontal branch, missed by most ophthalmologists, but later becoming a severe neuralgia. Dr. Kestenbaum stated that the diagnosis of anesthesia of the trigeminus cannot be based upon the loss of the corneal reflex alone, but all regions supplied by the nerve must be tested. X-ray studies are of great importance in topical diagnosis but were not discussed. It should be emphasized that the final diagnosis is made by the neurologist; that the ophthalmologist can only interpret the ocular sign to further his purpose. Leon H. Ehrlich, Secretary.