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Interpretations of Synkinetic Oculopalpebral Phenomena in Acquired Ophthalmoplegias*
INTERPRETATIONS O F SYNKINETIC OCULOPALPEBRAL P H E N O M E N A IN ACQUIRED O P H T H A L M O P L E G I A S * N. S. J A I N , D. O.
(OXON.)
Delhi, India INTRODUCTION
Several synkinetic oculopalpebral phe nomena are described in the literature in relation to ophthalmoplegias, congenital as well as acquired. Several neurologic ex planations have been offered to explain their occurrence. For instance, in an adduc tor palsy there is a Fuchs' phenomenon of lid retraction in attempted adduction. Gowers (1883) suggested the possibility of the over flow of involuntary impulses carried by one nerve from its usual channels into another (from the nerve to the medial rectus into that of the levator) giving rise to the phe nomenon. The view seems to find support from Fuchs' case (1895) of syphilis and Cord's case (1930) of trauma. Whether there is wandering of the regenerating axons or straying of nerve fibers from the sheath of one into that of the other (Bender, 1936) is not known. Duke-Elder says that the probable reason for paradoxic lid retraction of the ptotic lid on occlusion of the sound eye is that sen sory-motor connections become established at higher neural levels. The level at which crossing of the regenerating fibrils or im pulses takes place has, by and large, been a matter of conjecture only. Fuchs (1895) places this level in the central nervous sys tem and Bielschowsky (1932-35) thinks that it is in the periphery. Two cases of acquired ophthalmoplegia are described in this paper and it is hoped that the conclusions drawn therefrom may throw some light on this matter.
In the primary position there was a partial ptosis on the right side (fig. 1-A), the right palpebral aper ture was narrower than the left and the right eye was divergent. On covering the left eye retraction of the ptotic lid on the right side was elicited (fig. 1-B). The left eye was markedly abducted behind the occluder (fig. 1-C). On levoversion adduction of the right eye was restricted and on attempting it widen ing of the right palpebral aperture indicated the oc currence of Fuchs' phenomenon (fig. 1-C), the sound left eye being markedly abducted. On dextroversion the right palpebral aperture became narrower due to an increased ptosis while abducting the affected eye (fig. 1-D). Elevation of the right eye was absent (fig. 2-A) and the palpebral fissure became wider, thus eliciting what might be called a lid retraction on attempted elevation. Depression of the right eye was absent (fig. 2-B). Whereas the left lid drooped nor mally with the downward movement of the left eye, the right lid remained stationary, thus simulating the pseudo-Graefe phenomenon of lid retraction on in troduction (fig. 2-C). This phenomenon was more marked when the right eye attempted to look down and in (fig. 2-D) than down and out (fig. 2-E). The right pupil was dilated and fixed, there was traumatic optic atrophy and vision was reduced to counting fingers in the right eye. The father of the patient refused permission for any kind of surgery. CASE 2
A man, 22 years of age, came on September 5, 1959, with ptosis on the left side (fig. 3-A) and com plete palsy of the third cranial nerve, with a dilated and fixed left pupil. On the preceding day he had had pain in the left eye and headache, a mild pyrexia, obstruction to nasal breathing, nausea and vomiting. No definite cause for the palsy could be determined and all investigations (including complete blood ex amination, E.S.R., sérologie test for syphilis, urine and skiagraphy of the skull and the paranasal si nuses) were negative. He was given antibiotics parenterally. vitamins B-complex and C, and oral corticosteroids. Two weeks later signs of recovery were notice able and the following observations were made:
1. In primary position a. The ptosis was less in amount (fig. 3-B), the eyeball much less abducted than at the time of ad CASE REPORTS mission and the pupil had begun to react to light. CASE 1 b. On covering the sound right eye the left eye A young man, aged 19 years, sustained an injury moved slightly medially to assume fixation. The pal to the right side of the head on February 25, 1958. pebral aperture became wider and the ptosis was re duced in amount (fig. 3-C), eliciting the phenomenon * From the Department of Ophthalmology, Irwin of a paradoxic retraction of the ptotic lid on occlu sion of the sound eye. The occluded right eye, howHospital, New Delhi, India. 115
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Fig. 1 (Jain). Case 1. (A) Primary position. (B) On occlusion of the sound eye. (C) Levoversion. (D) Dextroversion. ever, was markedly abducted behind the cover to an extent seen in Figure 4-D. c. An injection of 0.5 cc. of Novocaine was made in the left external rectus on September 21, 1959. Ten minutes later the ptosis was further reduced and the palpebral fissure became wider than before (fig. 3-D, compare with fig. 3-B). d. On September 25, 1959, an injection of 0.5 cc. of Novocaine was made in the left internal rectus. Ten minutes later the left eye had assumed a more abducted position and the ptosis was enhanced (fig. 3-E), making the palpebral aperture narrower. Now on covering the sound eye, the left palpebral aperture did not become as wide as it did before injection (fig.3-F,cf.fig.3-C). 2. In levoverted position a. Before the injection of Novocaine in the left external rectus, abduction of the left eye was normal (fig. 4-A). b. Ten minutes later the left eye failed to abduct and the left palpebral aperture became wider (fig. 4-B) than seen under any other circumstance (cf. fig. 3-B, C, D, F and fig. 4-D). By thus creating a temporary weakness of the overacting left external rectus, Fuchs' phenomenon of lid retraction on at tempted abduction of the left eye was artificially created. c. Ten minutes after the injection in the left in ternal rectus, the ptosis on the left side was enhanced on levoversion (fig. 4-C, cf. fig. 4-A). 3. In dextrovertcd position a. Before injection in the medial rectus an attempt at adduction of the left eye caused widening of the left palpebral fissure (fig. 4-D, cf. fig. 3-B in pri
mary position) eliciting Fuchs' phenomenon of lid retraction on attempted adduction. The width of the palpebral aperture now became almost equal to that seen when the right eye was occluded (cf. fig. 3-C) to elicit retraction of the ptotic lid. b. Ten minutes after Novocaine injection in the medial rectus, all attempts at adduction of the left eye were abolished, as was Fuchs' phenomenon of lid retraction on attempted adduction (fig. 4-E). 4. After full recovery When the patient was seen on November 11, 1959, recovery was complete in all positions of the globe (fig. 5-A in primary position ; fig. 5-B in levo version; fig. 5-C in dextroversion). Almost no treatment had been given during the preceding five weeks and yet the rapidity and completeness of the recovery were remarkable. DISCUSSION
From the study of the two cases of ac quired ophthalmoplegia herein described, where in the third cranial nerve showed a palsy, some observations appear to be signifi cant. ELEVATION OF PTOTIC LID ON OCCLUSION OF SOUND EYE
This was present in both the cases (figs. 1-B and 3-C). In each the affected eye was divergent in the primary position (figs. 1-A and 3-B). When the contralateral sound eye
Fig. 2 (Jain). Case 1. (A) Elevation. (B and C) Depression. (D) Right eye looking down and in. (E) Right eye looking down and out.
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Fig. 3 (Jain). Case 2. (A) Primary position at the time of admission. (B) Primary position two weeks later (ptosis less). (C) On occlusion of the sound eye, there was retraction of the ptotic lid. (D) After injection of Novocaine into the left external rectus. (E) After injection into the left internal rectus. (F) On covering sound eye, the left palpebral aperture did not become so wide as it did before injection. was covered the divergent affected eye moved medially to assume fixation, the sound eye showed an exaggerated secondary devia tion behind the occluder and, at the same time, the ptotic lid showed an elevation, making the palpebral aperture wider. This suggests that, though interpreted as lid re traction on occlusion of the sound eye, it was only lid elevation associated with attempted adduction. This same observation has been made in five cases reported by Jain (1959). It appears, therefore, that the entity of the so-called paradoxic lid retraction- on occlu sion of the sound eye is not independent but exists in association with Fuchs' phenome non of lid retraction on attempted adduction of the affected eye. In Yanes' case (1940) the occurrence of this phenomenon was seen when the sound eye was closed with the hand of the subject and it seems probable that the behavior of the covered eye (which may have been abducted) was not observed. If this was so, since much of the clinical data is lacking, Yanes' case cannot be conclusively accepted as one of lid retraction on occlusion of the sound eye but was, presumably, one of lid elevation associated with adduction.
Similar mistakes, due to lack of identical observation, appear to have been made by Jain (1957, Cases 2 and 3, Brit. J. Ophth.). This misinterpretation was demonstrated and the possibility of repeating identical mis takes was emphasized by Jain in 1959. It seems reasonable to assume that occlu sion of the sound eye did not have any direct influence on lid elevation. In forcing the affected eye to assume fixation the small degree of primary medial deviation of the paralyzed eye probably brought about two exaggerated secondary movements: (1) an increased secondary divergence of the con tralateral sound eye, and (2) an increased levator action to produce lid elevation, the levator functioning as an ipsolateral Syner gist. If this hypothesis is to be accepted, two conclusions seem apparent: The first is that the levator and the medial rectus have a direct synergic interrelation ship. Judging from the law of equal and re ciprocal innervation in cases of paresis, the response of a weak medial rectus would be poor but, to the same stimulus, that of the levator would be sufficiently normal to stimu-
Fig. 4 (Jain). Case 2. (A) Normal abduction, L.E. (B) After Novocaine injection into the left ex ternal rectus. (C) After Novocaine injection into the left internal rectus. (D) Lid retraction on at tempted adduction of the left eye. (E) Phenomenon is abolished after the Novocaine injection into the left medial rectus.
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Fig. S (Jain). Appearance of patient on recovery. (A) Primary position. (B) Levoversion. (C) Dextroversion.
late lid elevation. Since the intensity of the stimulation has to be far in excess of the response expected from the weak medial rectus, the proportionate response obtained from the levator through an excessive syn ergic stimulation would give rise to an ap parent lid retraction. The second conclusion is that the entity of paradoxic lid retraction on occlusion of the sound eye appears to be of doubtful clinical existence and significance. These observations emphasize the need for a more careful examination of the be havior of the sound eye behind the occluder before any conclusions can be drawn as to the nature of the palpebral elevation. That the behavior of the ptotic lid is governed by the medial rectus will be further elucidated in the lines that follow. FUCHS'
P H E N O M E N O N O F LID RETRACTION
ON SIDE-TO-SIDE MOVEMENTS
In both the cases described herein Fuchs' phenomenon was observed when adduction was attempted by the paresed medial rectus (fig. 1-Candfig. 4-D). Following an injection of Novocaine into the left medial rectus in Case 2, the exces sive nervous effort originating in the para lyzed muscle was temporarily abolished, and so was Fuchs' phenomenon of lid retraction on attempted adduction (fig. 4-E, cf. fig. 4-D). This further supports the hypothesis of a direct synergistic relationship between the ipsolateral medial rectus and the levator. The Novocaine injection rendered the contracted external rectus even stronger and made the eye more divergent, while ptosis was en
hanced (fig 3-E). This suggests an inverse relationship between the external rectus and the levator. A similar observation has been made by Jain (1959). The magnitude of the phenomenon of lid elevation was also re duced on occlusion (fig 3-F, cf. fig. 3-C). When an injection of Novocaine was made to weaken the stronger external rectus in Case 2, a better adductive effort by the paralyzed medial rectus was obtained. This gave rise to an automatic elevation of the ptosed eyelid and the palpebral fissure be came wider than before (fig. 3-D, cf. fig. 3-B). Abduction of the left eye became markedly restricted and the palpebral aper ture became so much wider (fig. 4-B) as to simulate the phenomenon of lid retraction on attempted abduction in a temporarily and artificially created external rectus palsy in a case in which there was pathologic paresis of the third cranial nerve. This interesting ob servation appears not to have been previ ously recorded in the literature. After full recovery of the patient, the visual axes became parallel in all directions of gaze (fig. 5-A, B and C). This suggests that restoration of parallelism of the vis ual axes of the two eyes with time and treatment, whether conservative or surgical, can be expected to restore the normal be havior of the eyelids. This has been sug gested and elucidated by Jain (1959). SYNKINETIC
PHENOMENON
IN
ANOMALIES
OF VERTICAL MOVEMENTS
The first case presents an example of this phenomenon : a. With elevation. Where the superior rec tus is paralyzed and supraduction absent, as is often seen in congenital cases, the levator is also affected and there is an associated ptosis. In Case 1, however, with an absence of supraduction, instead of there being a ptosis, the lid remained elevated (fig. 2-A). This suggests (1) that, while the nerve to the superior rectus was involved, the nerve subserving the levator had escaped damage, and ( 2 ) , therefore that the site of lesion
OCULOPALPEBRAL PHENOMENA
must be infranuclear because in a nuclear lesion both the levator and the superior rec tus would be expected to become affected simultaneously, both being embryologically and neurophysiologically akin. b. With depression. In normal infraduction, the upper eyelid proportionately droops down. Should the lid fail to follow the down ward ocular movement and remain elevated, the phenomenon is known as pseudo-Graefe's phenomenon on downward gaze. In this case the right eyeball did not move downward (fig. 2-C), nor did the eyelid. Therefore, strictly speaking, it cannot be labelled as a true pseudo-Graefe phenomenon of lid re traction. The reason probably lies in the in ability of the eye to move downward and hence the absence of an attempt at a recipro cal droop of the ipsolateral lid. Being more marked in the movement down and in than down and out (fig 2-D and E ) , the influence of the medial rectus on widening of the palpebral fissure is apparent. All the circumstances wherein the lid continued to remain elevated (adduction, elevation, depression and on occlusion of the sound eye, figs. 1-C, 2-A, 2-C and 1-B, re spectively) suggest that the ptosis was pseudo- and not true in nature, and that at the time these phenomena were present the levator was functioning so normally as to be capable of keeping the lid elevated. Valuable information could be expected regarding the behavior of the ptotic lid in Case 1 had consent been obtained to study the effect of Novocaine injection to abolish the nervous impulses originating in the su perior rectus and passive downward traction had been obtained. Such cases are extremely rare and rarer still is the opportunity for a detailed study. SITE AND CAUSE OF LESION
The remarkable variance in the palpebral behavior in Case 2 under artificially altered amplitude of function of the paralyzed and contracted horizontal muscles and restora tion of absolute normality following recovery
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throw considerable doubt on the validity of the views of the various authors mentioned in the introduction. It is questionable that establishment of aberrant neurologic inter connections could on the one hand be ex pected to give rise to synkinetic oculopalpebral phenomena under pathologically paretic conditions and, on the other, to disappear when a balance was struck in the amplitude of action of the synergists and the antago nists. All these observations suggest an in franuclear etiology and the obviously incomitant nature of the strabismus rules out the supranuclear dissociations as a likely cause since the disturbance in the motility of the two eyes is neither equal nor simultane ous. T I M E OF ONSET OF SYNKINETIC PHENOMENA AND THE SIGNIFICANCE OF PTOSIS
The generally accepted view regarding the time when such phenomena become manifest is that they appear in palsies of old standing when a state of recovery sets in. In Case 2 the phenomenon was noticeable 15 days from the onset of ptosis, a period which can hardly be considered long enough to allow for recovery, and disappeared com pletely in four months. This supports an identical observation made by Jain (1959) who, offering a likely explanation, said that in the most initial and acute phase of the ophthalmoplegia the clinical picture becomes complex due to multiple factors. When in the course of recovery, the pressing factors secondarily affecting the nerve to the levator are withdrawn, leaving behind the primary basic anomaly of more lasting nature, such as the adductor palsy, these phenomena be come manifest. Bender (1936) says that lid retraction may occur after ptosis has recovered and voluntary lid closure is possible. Duke-Elder (1952) says that it occurs preferentially when paresis of the lid is less marked than that of the oculomotor muscles simultane ously called into play. Lid elevation, on at tempting to restore a greater parallelism of
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the visual axes by injecting Novocaine into the external rectus, suggests that the ptosis was pseudo—and not true in nature and that the medial rectus continued to remain paralyzed for a longer period than the levator. It follows that, in neurologic lesions wherein synkinetic phenomena are elicited, either the levator escapes being paralyzed or is first to recover. Recovery from ptosis in advance of recovery of the medial rectus appears then to govern the time of occur rence of such phenomena. SUMMARY
1. A detailed clinical study has been made of two cases of synkinetic oculopalpebral phenomena in acquired third nerve palsy. Case 1 with a posttraumatic affection of the horizontal as well as the vertical movements of the eyes presents extremely rare findings. 2. By artificially altering the associated behavior of the lids and the eyes in Case 2, the theories of some authors on the etiology
and the site of the lesion are disputed. 3. The infranuclear etiology is supported. 4. The independent clinicopathologic value of the phenomenon of lid retraction on oc clusion of the sound eye is doubted and its association with Fuchs' phenomenon of lid retraction on attempted adduction is demon strated. 5. Mistakes in interpretation of the signs by some authors are pointed out. 6. It has been suggested that lid retraction associated with third-nerve palsy signifies an exaggerated secondary movement of the levator, behaving like an ipsolateral synergist of the medial rectus, in response to the re stricted primary medial deviation of the paralyzed eye. Kucha Mahajani, Chandni Chowk. ACKNOWLEDGMENT
Dr. H. Mohan was kind enough to refer the first case for my detailed examination, interpretations and opinion. I thank him for allowing me to use the material for discussion.
REFERENCES
Bender, M. B.: The nerve supply to the orbicularis muscle and the physiology of movements of the upper eyelid. Arch. Ophth., 15:21-30 (Jan.) 1936. : Abnormal associated movements of the eyelid (pseudo-Graefe sign). Arch. Neurol. & Psychiat, 35:403-404 (Feb.) 1936. Bielschowsky, A.: Die Motilitätsstörungen der Augen. Graefe-Saemisch Handb. Augenh., II, 8:191, 1932. : Ibid., Nachtrag I, 196, 394, 1932. : Lectures on motor anomalies of the eyes: II. Paralysis of individual eye muscles. Arch. Ophth., 13:33-59 (Jan.) 1935. Cords, R. : Störungen der Bewegungen der Lider bei Seitenwendung der Augen. Kurzes Handb. Ophth., 3:626-627,1930. Duke-Elder, W. S.: Textbook of Ophthalmology. London, Kimpton, 1952, v. 5. Fuchs, E. : Association von Lidbewegung mit seitlichen Bewegungen des Auges. Beitr. Augenh., 2:1230, 1895. Gowers, W. R. : Congenital ptosis with peculiar associated movements of the affected lid. Trans. Ophth. Soc. U. Kingdom, 3:286-287, 1883. Jain, N. S.: Synkinetic lid retraction. Brit. J. Ophth., 41:247-253 (Apr.) 1957. : Aetiology of synkinetic oculo-palpebral movements in congenital and acquired ophthalmoplogias. J. All-India Ophth. Soc, 7:1-36 (April) 1959. Yanes, T. R.: Paradoxic monocular ptosis. Arch. Ophth., 23:1169-1172 (June) 1940.
(OXON.)
Delhi, India INTRODUCTION
Several synkinetic oculopalpebral phe nomena are described in the literature in relation to ophthalmoplegias, congenital as well as acquired. Several neurologic ex planations have been offered to explain their occurrence. For instance, in an adduc tor palsy there is a Fuchs' phenomenon of lid retraction in attempted adduction. Gowers (1883) suggested the possibility of the over flow of involuntary impulses carried by one nerve from its usual channels into another (from the nerve to the medial rectus into that of the levator) giving rise to the phe nomenon. The view seems to find support from Fuchs' case (1895) of syphilis and Cord's case (1930) of trauma. Whether there is wandering of the regenerating axons or straying of nerve fibers from the sheath of one into that of the other (Bender, 1936) is not known. Duke-Elder says that the probable reason for paradoxic lid retraction of the ptotic lid on occlusion of the sound eye is that sen sory-motor connections become established at higher neural levels. The level at which crossing of the regenerating fibrils or im pulses takes place has, by and large, been a matter of conjecture only. Fuchs (1895) places this level in the central nervous sys tem and Bielschowsky (1932-35) thinks that it is in the periphery. Two cases of acquired ophthalmoplegia are described in this paper and it is hoped that the conclusions drawn therefrom may throw some light on this matter.
In the primary position there was a partial ptosis on the right side (fig. 1-A), the right palpebral aper ture was narrower than the left and the right eye was divergent. On covering the left eye retraction of the ptotic lid on the right side was elicited (fig. 1-B). The left eye was markedly abducted behind the occluder (fig. 1-C). On levoversion adduction of the right eye was restricted and on attempting it widen ing of the right palpebral aperture indicated the oc currence of Fuchs' phenomenon (fig. 1-C), the sound left eye being markedly abducted. On dextroversion the right palpebral aperture became narrower due to an increased ptosis while abducting the affected eye (fig. 1-D). Elevation of the right eye was absent (fig. 2-A) and the palpebral fissure became wider, thus eliciting what might be called a lid retraction on attempted elevation. Depression of the right eye was absent (fig. 2-B). Whereas the left lid drooped nor mally with the downward movement of the left eye, the right lid remained stationary, thus simulating the pseudo-Graefe phenomenon of lid retraction on in troduction (fig. 2-C). This phenomenon was more marked when the right eye attempted to look down and in (fig. 2-D) than down and out (fig. 2-E). The right pupil was dilated and fixed, there was traumatic optic atrophy and vision was reduced to counting fingers in the right eye. The father of the patient refused permission for any kind of surgery. CASE 2
A man, 22 years of age, came on September 5, 1959, with ptosis on the left side (fig. 3-A) and com plete palsy of the third cranial nerve, with a dilated and fixed left pupil. On the preceding day he had had pain in the left eye and headache, a mild pyrexia, obstruction to nasal breathing, nausea and vomiting. No definite cause for the palsy could be determined and all investigations (including complete blood ex amination, E.S.R., sérologie test for syphilis, urine and skiagraphy of the skull and the paranasal si nuses) were negative. He was given antibiotics parenterally. vitamins B-complex and C, and oral corticosteroids. Two weeks later signs of recovery were notice able and the following observations were made:
1. In primary position a. The ptosis was less in amount (fig. 3-B), the eyeball much less abducted than at the time of ad CASE REPORTS mission and the pupil had begun to react to light. CASE 1 b. On covering the sound right eye the left eye A young man, aged 19 years, sustained an injury moved slightly medially to assume fixation. The pal to the right side of the head on February 25, 1958. pebral aperture became wider and the ptosis was re duced in amount (fig. 3-C), eliciting the phenomenon * From the Department of Ophthalmology, Irwin of a paradoxic retraction of the ptotic lid on occlu sion of the sound eye. The occluded right eye, howHospital, New Delhi, India. 115
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Fig. 1 (Jain). Case 1. (A) Primary position. (B) On occlusion of the sound eye. (C) Levoversion. (D) Dextroversion. ever, was markedly abducted behind the cover to an extent seen in Figure 4-D. c. An injection of 0.5 cc. of Novocaine was made in the left external rectus on September 21, 1959. Ten minutes later the ptosis was further reduced and the palpebral fissure became wider than before (fig. 3-D, compare with fig. 3-B). d. On September 25, 1959, an injection of 0.5 cc. of Novocaine was made in the left internal rectus. Ten minutes later the left eye had assumed a more abducted position and the ptosis was enhanced (fig. 3-E), making the palpebral aperture narrower. Now on covering the sound eye, the left palpebral aperture did not become as wide as it did before injection (fig.3-F,cf.fig.3-C). 2. In levoverted position a. Before the injection of Novocaine in the left external rectus, abduction of the left eye was normal (fig. 4-A). b. Ten minutes later the left eye failed to abduct and the left palpebral aperture became wider (fig. 4-B) than seen under any other circumstance (cf. fig. 3-B, C, D, F and fig. 4-D). By thus creating a temporary weakness of the overacting left external rectus, Fuchs' phenomenon of lid retraction on at tempted abduction of the left eye was artificially created. c. Ten minutes after the injection in the left in ternal rectus, the ptosis on the left side was enhanced on levoversion (fig. 4-C, cf. fig. 4-A). 3. In dextrovertcd position a. Before injection in the medial rectus an attempt at adduction of the left eye caused widening of the left palpebral fissure (fig. 4-D, cf. fig. 3-B in pri
mary position) eliciting Fuchs' phenomenon of lid retraction on attempted adduction. The width of the palpebral aperture now became almost equal to that seen when the right eye was occluded (cf. fig. 3-C) to elicit retraction of the ptotic lid. b. Ten minutes after Novocaine injection in the medial rectus, all attempts at adduction of the left eye were abolished, as was Fuchs' phenomenon of lid retraction on attempted adduction (fig. 4-E). 4. After full recovery When the patient was seen on November 11, 1959, recovery was complete in all positions of the globe (fig. 5-A in primary position ; fig. 5-B in levo version; fig. 5-C in dextroversion). Almost no treatment had been given during the preceding five weeks and yet the rapidity and completeness of the recovery were remarkable. DISCUSSION
From the study of the two cases of ac quired ophthalmoplegia herein described, where in the third cranial nerve showed a palsy, some observations appear to be signifi cant. ELEVATION OF PTOTIC LID ON OCCLUSION OF SOUND EYE
This was present in both the cases (figs. 1-B and 3-C). In each the affected eye was divergent in the primary position (figs. 1-A and 3-B). When the contralateral sound eye
Fig. 2 (Jain). Case 1. (A) Elevation. (B and C) Depression. (D) Right eye looking down and in. (E) Right eye looking down and out.
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Fig. 3 (Jain). Case 2. (A) Primary position at the time of admission. (B) Primary position two weeks later (ptosis less). (C) On occlusion of the sound eye, there was retraction of the ptotic lid. (D) After injection of Novocaine into the left external rectus. (E) After injection into the left internal rectus. (F) On covering sound eye, the left palpebral aperture did not become so wide as it did before injection. was covered the divergent affected eye moved medially to assume fixation, the sound eye showed an exaggerated secondary devia tion behind the occluder and, at the same time, the ptotic lid showed an elevation, making the palpebral aperture wider. This suggests that, though interpreted as lid re traction on occlusion of the sound eye, it was only lid elevation associated with attempted adduction. This same observation has been made in five cases reported by Jain (1959). It appears, therefore, that the entity of the so-called paradoxic lid retraction- on occlu sion of the sound eye is not independent but exists in association with Fuchs' phenome non of lid retraction on attempted adduction of the affected eye. In Yanes' case (1940) the occurrence of this phenomenon was seen when the sound eye was closed with the hand of the subject and it seems probable that the behavior of the covered eye (which may have been abducted) was not observed. If this was so, since much of the clinical data is lacking, Yanes' case cannot be conclusively accepted as one of lid retraction on occlusion of the sound eye but was, presumably, one of lid elevation associated with adduction.
Similar mistakes, due to lack of identical observation, appear to have been made by Jain (1957, Cases 2 and 3, Brit. J. Ophth.). This misinterpretation was demonstrated and the possibility of repeating identical mis takes was emphasized by Jain in 1959. It seems reasonable to assume that occlu sion of the sound eye did not have any direct influence on lid elevation. In forcing the affected eye to assume fixation the small degree of primary medial deviation of the paralyzed eye probably brought about two exaggerated secondary movements: (1) an increased secondary divergence of the con tralateral sound eye, and (2) an increased levator action to produce lid elevation, the levator functioning as an ipsolateral Syner gist. If this hypothesis is to be accepted, two conclusions seem apparent: The first is that the levator and the medial rectus have a direct synergic interrelation ship. Judging from the law of equal and re ciprocal innervation in cases of paresis, the response of a weak medial rectus would be poor but, to the same stimulus, that of the levator would be sufficiently normal to stimu-
Fig. 4 (Jain). Case 2. (A) Normal abduction, L.E. (B) After Novocaine injection into the left ex ternal rectus. (C) After Novocaine injection into the left internal rectus. (D) Lid retraction on at tempted adduction of the left eye. (E) Phenomenon is abolished after the Novocaine injection into the left medial rectus.
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Fig. S (Jain). Appearance of patient on recovery. (A) Primary position. (B) Levoversion. (C) Dextroversion.
late lid elevation. Since the intensity of the stimulation has to be far in excess of the response expected from the weak medial rectus, the proportionate response obtained from the levator through an excessive syn ergic stimulation would give rise to an ap parent lid retraction. The second conclusion is that the entity of paradoxic lid retraction on occlusion of the sound eye appears to be of doubtful clinical existence and significance. These observations emphasize the need for a more careful examination of the be havior of the sound eye behind the occluder before any conclusions can be drawn as to the nature of the palpebral elevation. That the behavior of the ptotic lid is governed by the medial rectus will be further elucidated in the lines that follow. FUCHS'
P H E N O M E N O N O F LID RETRACTION
ON SIDE-TO-SIDE MOVEMENTS
In both the cases described herein Fuchs' phenomenon was observed when adduction was attempted by the paresed medial rectus (fig. 1-Candfig. 4-D). Following an injection of Novocaine into the left medial rectus in Case 2, the exces sive nervous effort originating in the para lyzed muscle was temporarily abolished, and so was Fuchs' phenomenon of lid retraction on attempted adduction (fig. 4-E, cf. fig. 4-D). This further supports the hypothesis of a direct synergistic relationship between the ipsolateral medial rectus and the levator. The Novocaine injection rendered the contracted external rectus even stronger and made the eye more divergent, while ptosis was en
hanced (fig 3-E). This suggests an inverse relationship between the external rectus and the levator. A similar observation has been made by Jain (1959). The magnitude of the phenomenon of lid elevation was also re duced on occlusion (fig 3-F, cf. fig. 3-C). When an injection of Novocaine was made to weaken the stronger external rectus in Case 2, a better adductive effort by the paralyzed medial rectus was obtained. This gave rise to an automatic elevation of the ptosed eyelid and the palpebral fissure be came wider than before (fig. 3-D, cf. fig. 3-B). Abduction of the left eye became markedly restricted and the palpebral aper ture became so much wider (fig. 4-B) as to simulate the phenomenon of lid retraction on attempted abduction in a temporarily and artificially created external rectus palsy in a case in which there was pathologic paresis of the third cranial nerve. This interesting ob servation appears not to have been previ ously recorded in the literature. After full recovery of the patient, the visual axes became parallel in all directions of gaze (fig. 5-A, B and C). This suggests that restoration of parallelism of the vis ual axes of the two eyes with time and treatment, whether conservative or surgical, can be expected to restore the normal be havior of the eyelids. This has been sug gested and elucidated by Jain (1959). SYNKINETIC
PHENOMENON
IN
ANOMALIES
OF VERTICAL MOVEMENTS
The first case presents an example of this phenomenon : a. With elevation. Where the superior rec tus is paralyzed and supraduction absent, as is often seen in congenital cases, the levator is also affected and there is an associated ptosis. In Case 1, however, with an absence of supraduction, instead of there being a ptosis, the lid remained elevated (fig. 2-A). This suggests (1) that, while the nerve to the superior rectus was involved, the nerve subserving the levator had escaped damage, and ( 2 ) , therefore that the site of lesion
OCULOPALPEBRAL PHENOMENA
must be infranuclear because in a nuclear lesion both the levator and the superior rec tus would be expected to become affected simultaneously, both being embryologically and neurophysiologically akin. b. With depression. In normal infraduction, the upper eyelid proportionately droops down. Should the lid fail to follow the down ward ocular movement and remain elevated, the phenomenon is known as pseudo-Graefe's phenomenon on downward gaze. In this case the right eyeball did not move downward (fig. 2-C), nor did the eyelid. Therefore, strictly speaking, it cannot be labelled as a true pseudo-Graefe phenomenon of lid re traction. The reason probably lies in the in ability of the eye to move downward and hence the absence of an attempt at a recipro cal droop of the ipsolateral lid. Being more marked in the movement down and in than down and out (fig 2-D and E ) , the influence of the medial rectus on widening of the palpebral fissure is apparent. All the circumstances wherein the lid continued to remain elevated (adduction, elevation, depression and on occlusion of the sound eye, figs. 1-C, 2-A, 2-C and 1-B, re spectively) suggest that the ptosis was pseudo- and not true in nature, and that at the time these phenomena were present the levator was functioning so normally as to be capable of keeping the lid elevated. Valuable information could be expected regarding the behavior of the ptotic lid in Case 1 had consent been obtained to study the effect of Novocaine injection to abolish the nervous impulses originating in the su perior rectus and passive downward traction had been obtained. Such cases are extremely rare and rarer still is the opportunity for a detailed study. SITE AND CAUSE OF LESION
The remarkable variance in the palpebral behavior in Case 2 under artificially altered amplitude of function of the paralyzed and contracted horizontal muscles and restora tion of absolute normality following recovery
119
throw considerable doubt on the validity of the views of the various authors mentioned in the introduction. It is questionable that establishment of aberrant neurologic inter connections could on the one hand be ex pected to give rise to synkinetic oculopalpebral phenomena under pathologically paretic conditions and, on the other, to disappear when a balance was struck in the amplitude of action of the synergists and the antago nists. All these observations suggest an in franuclear etiology and the obviously incomitant nature of the strabismus rules out the supranuclear dissociations as a likely cause since the disturbance in the motility of the two eyes is neither equal nor simultane ous. T I M E OF ONSET OF SYNKINETIC PHENOMENA AND THE SIGNIFICANCE OF PTOSIS
The generally accepted view regarding the time when such phenomena become manifest is that they appear in palsies of old standing when a state of recovery sets in. In Case 2 the phenomenon was noticeable 15 days from the onset of ptosis, a period which can hardly be considered long enough to allow for recovery, and disappeared com pletely in four months. This supports an identical observation made by Jain (1959) who, offering a likely explanation, said that in the most initial and acute phase of the ophthalmoplegia the clinical picture becomes complex due to multiple factors. When in the course of recovery, the pressing factors secondarily affecting the nerve to the levator are withdrawn, leaving behind the primary basic anomaly of more lasting nature, such as the adductor palsy, these phenomena be come manifest. Bender (1936) says that lid retraction may occur after ptosis has recovered and voluntary lid closure is possible. Duke-Elder (1952) says that it occurs preferentially when paresis of the lid is less marked than that of the oculomotor muscles simultane ously called into play. Lid elevation, on at tempting to restore a greater parallelism of
120
N. S. JAIN
the visual axes by injecting Novocaine into the external rectus, suggests that the ptosis was pseudo—and not true in nature and that the medial rectus continued to remain paralyzed for a longer period than the levator. It follows that, in neurologic lesions wherein synkinetic phenomena are elicited, either the levator escapes being paralyzed or is first to recover. Recovery from ptosis in advance of recovery of the medial rectus appears then to govern the time of occur rence of such phenomena. SUMMARY
1. A detailed clinical study has been made of two cases of synkinetic oculopalpebral phenomena in acquired third nerve palsy. Case 1 with a posttraumatic affection of the horizontal as well as the vertical movements of the eyes presents extremely rare findings. 2. By artificially altering the associated behavior of the lids and the eyes in Case 2, the theories of some authors on the etiology
and the site of the lesion are disputed. 3. The infranuclear etiology is supported. 4. The independent clinicopathologic value of the phenomenon of lid retraction on oc clusion of the sound eye is doubted and its association with Fuchs' phenomenon of lid retraction on attempted adduction is demon strated. 5. Mistakes in interpretation of the signs by some authors are pointed out. 6. It has been suggested that lid retraction associated with third-nerve palsy signifies an exaggerated secondary movement of the levator, behaving like an ipsolateral synergist of the medial rectus, in response to the re stricted primary medial deviation of the paralyzed eye. Kucha Mahajani, Chandni Chowk. ACKNOWLEDGMENT
Dr. H. Mohan was kind enough to refer the first case for my detailed examination, interpretations and opinion. I thank him for allowing me to use the material for discussion.
REFERENCES
Bender, M. B.: The nerve supply to the orbicularis muscle and the physiology of movements of the upper eyelid. Arch. Ophth., 15:21-30 (Jan.) 1936. : Abnormal associated movements of the eyelid (pseudo-Graefe sign). Arch. Neurol. & Psychiat, 35:403-404 (Feb.) 1936. Bielschowsky, A.: Die Motilitätsstörungen der Augen. Graefe-Saemisch Handb. Augenh., II, 8:191, 1932. : Ibid., Nachtrag I, 196, 394, 1932. : Lectures on motor anomalies of the eyes: II. Paralysis of individual eye muscles. Arch. Ophth., 13:33-59 (Jan.) 1935. Cords, R. : Störungen der Bewegungen der Lider bei Seitenwendung der Augen. Kurzes Handb. Ophth., 3:626-627,1930. Duke-Elder, W. S.: Textbook of Ophthalmology. London, Kimpton, 1952, v. 5. Fuchs, E. : Association von Lidbewegung mit seitlichen Bewegungen des Auges. Beitr. Augenh., 2:1230, 1895. Gowers, W. R. : Congenital ptosis with peculiar associated movements of the affected lid. Trans. Ophth. Soc. U. Kingdom, 3:286-287, 1883. Jain, N. S.: Synkinetic lid retraction. Brit. J. Ophth., 41:247-253 (Apr.) 1957. : Aetiology of synkinetic oculo-palpebral movements in congenital and acquired ophthalmoplogias. J. All-India Ophth. Soc, 7:1-36 (April) 1959. Yanes, T. R.: Paradoxic monocular ptosis. Arch. Ophth., 23:1169-1172 (June) 1940.