EYE PARALYSES. HY / JOHN AMOKV JEFFRIES, M. I). Reprinted from the Boston Medical and Surgical Journal of October 20 and 27, i8q2. BOSTON: DAMRELL & UPHAM, Publishers, 283 Washington Strest. 1892. //, //,///,,,/' ■ w. /'y/'"' S. J. PARKHILL A CO., PRINTERS BOSTON BY JOHN AMORY JRKFKIK8, M.D. EYE PARALYSES* [Tliis paper, which Dr. Jeffries had nearly finished at the time of his death, has been put in my hands to prepare for publica- tion. Although 1 knew something of his intentions in regard to it, a study of tlie paper itself and of the mass of material which lie had collected on the subject has convinced me of the diffi- culty of completing the task as he would have done it. With the exception of a few verbal changes, I have done little ex- cept to prepare a bibliography from the notes which Dr. Jeffries left, and to indicate in brackets the references to illustrative cases. Not having the original articles at hand and depending almost wholly upon these notes 1 fear that the classification of the cases, which I have tried to make, is not always erred, and that it might not agree with Dr. .Jeffries’ greater knowledge. I have added Bleuler's diagram, which Dr. Jeffries had copied, probably with the intention of inserting it. — p. c. K.] Probably every practitioner has at times been in 8 110 Peters,112 Stoltiug,160 Grafe,45 Pilz,115 De Watteville,21 and Binsler.7] Some of the cases are suggestive of an hysterical state, but the mass of them point to gross disease. A centre of convergence has been assumed to exist in the anterior part of the third nuclei, but as yet nothing definite has been shown. Hensen and Vbickers’55 experiments—which do not tally with others — at the most do not demonstrate a centre : irri- tation at a point of crossing of fibres would produce the same effect. Thus, if, as suggested above, the fibres governing convergence come down to near the sixth nucleus, ascend and cross to their nuclei, stimulation at this crossing would produce convergence. What- ever the mechanism this paralysis is just the obverse of lateral conjugate paralysis. With both eyes open, neither will turn in, but cover one and the other comes 19 in at once, while the covered turns out to a parallel position. The case reported by Rinsler7 may betaken as a typical example. A hind man, previously well, ran to an appointment, and while blowing on the horn, suddenly lo>t sight of the music and had to stop play- ing. Careful examination showed that distant vision was good, but near vision poor and accompanied by crossed diplopia. When one eye was covered, the other turned in well for near vision, the covered eye turning out at the saute time. The right pupil reacted to light, but did not to accommodation. Sense of cold less distinct on the right side than on the left. No other symptoms, no change in six mouths. We have left to consider the great mass of eye par- alyses, which simply affect one or more muscles and do not tend to select associated functions. In these Cases we are deprived of a most valuable guide in local- ization, ami are obliged to fall back on what other symptoms iu»j he present, and a few isolated points of value. As is now well known, the nuclei of the fourth nerves lie just behind those of the third nerves, and practically form a unit with them. The nerves themselves, however, instead of passing down from the nuclei to the lower surface of the pons, go up and above the aqueduct before issuing from the brain. As a result, the crossing gives an occasion for double fourth nerve paralysis, without involvement of the other nerves ; which the peripheral course of the nerves does not readily lend itself to. Though no cases of the isolated paralysis of the superior obliques have been reported, there are those of Christ’s n and others in which this symptom has been used with good success. There are few other factors of importance in the distribution of the paralysis. Mauthner*1 ami others have held that a peripheral paralysis of the nerve trunk was complete or practically evenly distributed among the various muscles supplied by the nerve, while a 20 paralysis due to disease in the base of the brain was commonly incomplete or irregularly distributed. Thus the intrinsic or extrinsic muscles of the eye alone might be affected or some of the muscles of the third and not the others; the reason being that while the nerve is compact and small and therefore not easily partly af- fected, the nuclei of the third nerves are strung along the floor of the aqueduct and the posterior part of the third ventricle in the form of several sub-nuclei. Vari- ous efforts have been made to determine the functions of these sub-nuclei, but so far little, if anything, be- yond speculative results has been obtained. Anatomy shows that there is a median nucleus common to both third nerves, and that the posterior dorsal nucleus sends fibres across the raphe to the opposite third nerve. As a general axiom Mauthner’s position is undoubtedly true ; but, as the case of Thomsen already cited 154 and the following show, it is not absolute. Oppenlieim102 has reported two cases of brain tumor which appear probably to belong here. In the one there was paresis of the right third and sixth nerves, aud of the left in- ternal rectus from a tumor the size of an apple chiefly in the basal part of the right frontal lobe, but extend- ing some to the left frontal lobe. In the other there was complete paralysis of right internal rectus with a tumor in the right temporal lobe which pressed upon the lower parts of the brain and third nerve. Meyer’s 84 cases of multiple neuritis also bear on this point. Another symptom of value is the variableness of the paralysis. Most true peripheral paralyses tend to run a definite course, and do not change rapidly, indeed from minute to minute, as is not uncommon in central cases. This is naturally explained by the nerves being little more than conductors, acting when isolated from the body, while the centres discharge and generate force. As a result of this it is not rare to see a slight 21 paresis grow during the course of an examination into a paralysis, and new paresis develop. From the same cause the paresis is apt to show itself at night or even- ing and not in the mornm". Hut the above characteristics are of use in only a small part of the cases : in the majority we must rely on the general symptoms and etiology for our diag- nosis. There are a number of diseases which from their importance require consideration. In tabes, as has long been known, there is frequently a history of transitory diplopia during the prodromal period. A patient sees double on and off, just for a moment, particularly at night. The time is fixed partly by the causes already referred to, and partly bv the fact that lamps afford a close test of the eyes. When the eyes are tested no signs of paralysis are detected. In another group, usually more advanced, a positive paralysis exists, an external rectus is weak, convergence is poor, or they see double in the upper stories of the houses as they pass along the street. These paralyses may in their turn pass off in a few days, give place to others, or remain fixed. The origin of these fugacious paralyses is not known, but their significance when combined with previous syphilitic infection is gravely suggestive of tabes to follow. The more fixed paraly- ses are apparently due to a mixture of causes, the most common being a thickening of the ependyma and an extension of the process into the nuclei and fibre tracts ; another is syphilitic infiltration about the roots of the nerves before they are combined into a compact bundle. Endarteritis and its results do not apparently play an important part in these early paralyses. The latter ami general paralyses of the eye occur- ring in tabes are usually classed as cases of ophthal- moplegia. These are characterized by their irregular distributions, extent and persistency. Paralysis of the eye muscles is by no means rare in 22 cases of multiple sclerosis. Upthoff 157 found seventeen cases in a hundred. These cases are classed as follows: Two of double sixth paralysis, four of single sixth, three of third nerve, all partial; two of lateral con- jugate, one of up conjugate, three of convergence, and two of ophthalmoplegia externa. It will be noticed that the paralyses are all limited, do not tend to in- clude the whole of the third nerve, bat only alfect one or two muscles, as an associated motion. They tend to be more closely limited than in tabes. Tumors involving the corpora quadrigeroina, the pineal or pituitary gland are very apt to cause paraly- sis in the branches of the third, together with a pecu- liar form of ataxia and impairment of sight, but there is nothing in the paralysis itself to indicate the nature of the lesion, except perhaps in cases of paralysis of up-and down motion. The diagnosis of the nature of the lesion must be based on the general symptoms of tumor and the fact that other lesions are rare in this locality. In a summary of 29 cases Christ17 found paralysis of the third nerve in 22, of the fourth in six, and of the sixth in nine cases. The so-called cases of ophthalmoplegia externa re- quire notice. In 1879 Hutchinson 64 called attention to a group of cases in which a progressive fairly sym- metrical paralysis of the muscles of the e-balls formed a predominant symptom among a group of scattered bulbar paralyses and general cerebral symptoms. Since then the limits of this group have been extended so as to cover all general eye paralyses apparently of central origin and thus any value which may have at- tached to the name has been lost. To-day a diagnosis of ophthalmoplegia is about as significant as one of stomach ache. The twenty odd cases in which there are fairly good autopsy reports, some being monuments of labor, skill ami knowledge, show a great variety of processes. 23 A few groups, however, can be separated out, with a fair decree of accuracy : First, there are the cases of polio-encephalitis of Wernicke, represented by Gavet's case,40 Wernicke’s187 three cases, and Thomsen’s151 two cases. In these cases there are but little paralysis except of the eyes, but in all mental disturbance, active delirium or somno- lence, a staggering ataxic gait, tremor and the general signs of severe brain disease. At the autopsy a marked injection of the vessels, and numerous miliary haemor- rhages throughout the central gray matter of the third ventricle, aqueduct, and fourth ventricle have been the principal trouble. The process has also iu some cases been diffused through the whole of this region. In others, more or less extensive degeneration of the nu- clei has also been present. In Hutchinson’s caseM the process is given as a nuclear degeneration, the same as in muscular atrophy, a pathology with which the symptoms well accorded. Some of the cases of diphtheritic paralysis also appear to partake of the same nature though peripheral trou- ble is present. These cases are slower than those of Wernicke’s group, and lack the high degree of vascu- lar change and acute symptoms, but seem to be allied to them by a certain amount of vascular change and the nuclear degeneration. A second class is represented bv the cases of Duboys,2* Eiseulohr,2* and Bristowe,12 in which careful microscopic examination gave negative results. In character they all differ: the first was a recurrent trouble, the second like a bulbar case, while iu the third there were signs of Graves’ disease and much suggesting hysteria. The rest of the cases depend on all sorts of gross lesions, as Etter’s81 with myelitis, Sutter’s161 with tumor, Buzzard’s18 with syphilitic endarteritis (?), while the changes occurring iu company with tabes, 24 multiple sclerosis, and general paralysis make up the rest. Dufour 24 has summarized the mass of reported cases of ophthalmoplegia exterior or nuclear palsies, from which the following figures are taken. Males 122, females 41. Before the fifteenth year, 23; in the next fifteen 35; and from thence to the sixty-ninth year 67 cases. Total, 125. Of 183 cases 31 had previous cerebrospinal disease, 74 syphilis, diphtheria, diabetes, or the like, and 78 were in health. Disease at the base of the skull often causes more or less paralysis of the eyes, as in tubercular menin- gitis and the so-called syphilitic meningitis. As a rule iu the first any eye paralysis is of late date, and accom- panied by affection of other nerves in a way suggest- ing a disease of the membranes, as well as by general constitutional symptoms. But this is no law, as the following case of Seitz135 proves. A man of forty years went to bed well and woke up in the morning with a complete paralysis of the right third nerve. He later developed the full signs of tubercular meningitis from which he died. The autopsy showed nothing re- markable to explain the early eye paralysis. Syphili- tic disease of the membranes is usually in reality a more or less diffuse gummatous or round cell growth, springing from the dura, and often lying between the dura and the skull. By creeping along the base of the skull and crowding the foramina such growths, though of small mass, can produce great mischief. It is equally true in these cases, as in others of syphilitic origin, that fever is conspicuous by its absence, while nocturnal pain and insomnia predominate. Uuverricht’s case 158 is a type of how a small amount of tumor can produce much mischief. In the other case the disease at the surface of the base is combined with central trouble depending on disease of the vessels or the neu- roglia, as iu the case carefully studied by Siemerling.140 25 Other tumors in the some region produce the same effects, but tend by pressure to cause paralyses of the body as well as optic neuritis. They also tend to a unilateral distribution, to picked paralysis of the fifth nerve, with neuralgia, as well as to involve the nerves in series as they run along the base of the skull. There are still but few cases of recurrent paralysis of a third nerve with autopsy reported. (Richter,120 Weiss,163 Fiedler 84 two.) in all of these, in spite of the various theories advanced, some form of local dis- ease of the nerve has been found as tubercle, lihroma, meningitis. These cases have not been true recurrent paralysis, since there has been a certain amount of paresis between the spells. They are cases of paresis of the third nerve with recurrent exacerbations. The spells are apt to he accompanied or preceded by pain, vomiting, und confusion. Until lately there has been more or less feeling that the eyes were exempt in cases of multiple neuritis, a position exploded bv the cases of Pal,107 as well as by that of Meyer.84 The last case is of special interest, as it partook of the character of an ophthalmoplegia. A man forty-two years, old suffering from chronic bronchitis and bronchiectasis developed a paralysis of all the muscles of the eye-halls and the levators of the lids, but with no paralysis of the pupils. Anaesthesia of the cornea, paraesthesia of the back and limbs, and dysphagia soon developed, shortly after which death occurred- A careful microscopic examination failed to show any disease of the brain, but did demonstrate an extensive multiple neuritis. The third, fourth, and sixth nerves were degenerated, while the facials, hyo- glossals, glossopharyngeals aiid many spinal nerves were partially degenerated. In disease of the orbit the paralysis may be general, according to accepted doctrine, or localized in certain branches of the third nerve ; hut our knowledge of the subject is very meagre. The foramina themselves offer opportunity for trouble by the growth of exosto- ses, while fracture is not a rare cause of trouble. With so-called rheumatic paralyses of the eye muscles, so commonly referred to, I have had little or no experi- ence. Of some fifty or sixty cases of which I have records, there is but one which can fairly be considered as belonging to this group if it exist. This case was seen but once, and was so classed from lack of any in- dications. It goes without saying that the probabilities of syphilis were great. The only autopsy made in the allied facial trouble (Minkowski90) revealed a general degeneration of the whole nerve, and no signs of rheu- matism. Had other nerves been affected, the case would have been one of multiple neuritis. There are a few other factors which require mention from the danger of their being overlooked, as congeni- tal imperfections of motion due to paralysis, as in the cases of conjugate paralysis reported by Giafe46and others, to imperfect insertion of the muscles or, to dis- ease in the muscles and tissue of the orbit. Of the latter I have seen one case (following influenza?) in which there was much limitation of motion of the eye- balls, intense pain, deep tenderness, fever, pupils not affected, which recovered in ten days. Ptosis, though not due to paralysis of a muscle of an eye-ball, is so closely connected with these as to re- quire notice. The lid is raised by two muscles, the levator palpebrae supplied by the third nerve .and un- striped fibres supplied by sympathetic fibres. Paraly- sis of the latter is not so very rare, judging from the slight dropping of the lid during a spell of hemicrania, and in tabetics. A slight congenital drooping of the upper lid is also by no means uncommon. This state is of importance chiefly from the possibility of its being mistaken for a true ptosis. The obverse con- 26 27 dition is seen in the failure of the lid to follow the pupil down and the wide palpebral fissure of Graves’ disease. True ptosis is a different affair and is a sign of im- portance as a danger signal rather than as a factor in diagnosis. It is commonly present in the early stages of hemiplegia from any cause, and has much the same significance as conjugate deviation of the eyes. Some writers have endeavored to localize a special centre for the lid in the temporal region, hut have failed to make out a strong case. Barring the cerebral cases, ptosis occurs with paralysis of the third nerve, from almost any cause, and is often the first sign. A patient wakes up with slight drooping of one lid, and in the course of a few hours to mouths the other {tarts sup- plied by the third nerve are involved. Either the nerve to the lid is more exposed to disease, or owing to its position and constant work defects in the muscle are more easily recognized. However this may he, a picked paralysis of one muscle supplied by the third nerve is very rare and quite surely will be followed by others. The danger is that the physician may over- look or slight the paralysis and thus receive a disagree- able surprise. No reference has been made to treatment, since, with 60 many different processes involved it would open up a wide field. The process causing the paraly- sis is the object to be aimed at, not the paralysis. M v own feeling is that where the cause is not apparent, an eye paralysis is very apt to be of syphilitic origin, and as such to suggest antisyphilitic treatment. 'There is little pathological evidence at hand, but this view seemed in accord with the growing sentiment of those who have paid most attention to the subject. Un- fortunately it does not follow that because an eye pa- ralysis has a syphilitic basis, treatment will do any good. 28 To sum up: (1) All cases of lateral conjugate paralysis are of central origin. (2) When the paralysis is on the same side as other paralyses the lesion is on the opposite side of the brain. Such paralyses as a rule are transitory and follow al- most any sudden lesion, and often only show them- selves as a prevailing position of the eye, and not as a true paralysis or even paresis. (3) When the paralysis is crossed with the paraly- ses below, the lesion is in the pons-medulla region. The above three are equally true of spasms. (4) A gradual development of conjugate paralysis clearly points to the region of the sixth nucleus of the same side as affected. (5) Paralysis of up or down motions or both mo- tions indicate disease in the region of the corpora quadrigemina, but may be due to disease in the third nerves proper, at the point of exit. (6) Reasoning from analogy, paralysis of convergence points to disease in the central gray below the aque- duct, but as yet autopsies are lacking. (7) Picked paralysis of parts of a third nerve strongly suggests central disease, but is not proof of it. (8) A majority of the cases of eye paralysis' occur in the syphilitic. (9) A paralysis which changes rapidly, quickly showing fatigue, is probably central in origin. (10) Transitory paralysis in the syphilitic is strongly suggestive of future tapes. (11) An eye paralysis, however simple it may seem, is always a just cause for suspicion of trouble to come, and demands a prompt and thorough examination of the patient. (12) There is no evidence that there is any form of connection between the sixth nucleus and the third, except iu the cerebrum. 29 1. 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