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Show Journal of CliniCJll Neuro- ophthalmology 10( 1): 38- 40, 1990. © 1990 Raven Press, Ltd., New York Dissociated Vertical Deviation in a Patient with Duane's Retraction Syndrome Steve Rimmer, M. D., and Barrett Katz, M. D. Dissociated vertical deviation is a not uncommon strabismic syndrome characterized by upward deviation of an eye when occluded, with downward movement of the eye when occlusion is removed, Associated findings include latent nystagmus and horizontal strabismus. Duane's retraction syndrome is the clinical declaration of anomalous cranial nerve innervation characterized by a marked limitation or absence of abduction, variable limitation of adduction, narrowing of the palpebral fissure, and apparent globe retraction on attempted adduction. We report a patient with both dissociated vertical deviation and bilateral Duane's retraction syndrome, demonstrating that dissociated vertical deviation can occur with the anomalous neuroanatomic substrate present in Duane's retraction syndrome. Key Words: Duane's retraction syndrome-- Dissociated vertical deviation- Cranial nerves- StrabismusBrainstem anatomy- Eye movements. From the Neuro- Ophthalmology Unit, Pacific Presbyterian Medical Center, and The Smith- Kettlewell Eye Research Institute, San Francisco, California, U. S. A. Address correspondence and reprint requests to Barrett Katz, M. D., Pacific Presbyterian Medical Center and The SmithKettlewell Eye Research Institute, 2340 Clay Street, San Francisco, CA 94115, USA 38 Dissociated vertical deviation was first described in 1895 by Stevens ( 1) as an alternating vertical strabismus. Dissociated vertical deviation is characterized by an upward deviation of an eye when occluded ( or spontaneously during periods of inattention), with downward movement of the eye when occlusion is removed ( or when a refixation stimulus occurs). Associated findings may include latent nystagmus, horizontal strabismus ( usually an esodeviation), downward movement of the occluded eye when filters of increasing density are placed before the fixing eye ( Bielschowsky's phenomenon), inferior oblique overaction, deficient fusion, excyclotorsion with supraduction during occlusion, with incyclotorsion and infraduction after the occlusion is removed ( 2). The vertical dissociation may be related to inattention or visual deprivation. Though the occluded eye undergoes supraduction, both eyes move concomitantly in horizontal and vertical meridians. While several theories have been proposed to explain the cause of dissociated vertical deviation ( 3- 9), its etiology remains unknown. Duane's retraction syndrome is characterized by a marked limitation or absence of abduction, variable limitation of adduction, narrowing of the palpebral fissure and apparent globe retraction on attempted adduction, and vertical adventitious movements on adduction. Duane's syndrome is more common in women and on the left side, although bilateral cases occur regularly ( 10). Electromyographic and pathologic studies have shown that Duane's retraction syndrome is a neurogenic disorder in which the abducens nuclei and nerves are absent from the brainstem, and the lateral rectus muscle is innervated by branches of the oculomotor nerve ( 11,12). We describe a patient with both dissociated vertical deviation and Duane's retraction syndrome. To the best of our knowledge the association of DISSOCIATED VERTICAL DEVIATION IN DUANE'S RETRACTION SYNDROME 39 CASE REPORT DISCUSSION .. FIG. 2. Supraduction of the right eye under cover as the left eye fixates ( gaze directed to the right, minimiZing actions of the right inferior oblique). Several theories have been proposed to explain the etiology of dissociated vertical deviation. These include superior rectus hypofunction ( 3), " insufficient retinal stimulation causing elevation as a return to the position of absolute rest" ( 4), superior oblique hypofunction ( 5), anomalous excitations of subcortical vertical divergence centers ( 13), aberration of extraocular muscle tonus ( 6), bilateral paresis of the depressor muscles ( 7), and a defect of optomotor impulses from the lower nasal quadrants ( 8,9). Helveston ( 2) proposed that a center for vertical vergence may influence ocular motility, and that this center may be overridden by the relative visual experience of the two eyes. He suggested dissociated vertical deviation to be a sensory neurogenic disorder because he recognized it to occur in some normals, after prolonged occlusion. Duane's retraction syndrome is now recognized to be the clinical expression of anomalous extraocular muscle innervation. The lateral rectus muscle is ( presumably) innervated by fibers from the inferior branch of the oculomotor nerve ( the same ramus that innervates the inferior oblique) ( 11,12). Patients with Duane's syndrome may exhibit other brainstem dysfunction ( 14), as well as congenital defects involving ocular, skeletal, and neural structures ( 15- 27). Jay and Hoyt ( 14) found abnormal brainstem auditory- evoked potentials in nine of 14 patients with Duane's syndrome. The specific abnormality was a delay in wave III ( thought to be generated in the superior olivary complex of the pons). In a study of 186 patients with Duane's retraction syndrome, Pfaffenbach et al. ( 19) found neurologic abnormalities that included hearing loss, seizures, generalized hypotonia, congenital facial palsy, and microcephaly. Maruo et al. ( 23) found a significant number of patients with Duane's retraction syndrome to have the gustolacrimal reflex ( tear production while masticating). Isenberg and Blechman ( 26) described a patient - .. f. 6 t B This 18- year- old right- handed woman first presented in the spring of 1984, when she was referred because of a recognized ocular motility disturbance. Family members noted problems with her eye movements that had been present since birth. When first examined by us ( at the age of 18) she was noted to have normal central vision, normal color vision, and normal pupillary function, bilaterally. Her external examination demonstrated narrowing of each palpebral fissure on attempted adduction ( Fig. 1), a small angle V- pattern esotropia, and minimal bilateral overaction of the inferior oblique muscles with upshooting of the adducted eye. The patient preferred to fixate with her right eye. Cover- uncover testing revealed a small angle left hypertropia in primary position. There was decreased abduction bilaterally, with each eye just crossing midline ( Fig. 1). Alternate cover testing revealed that each eye would elevate in abduction under cover, though the right would elevate more than the left ( Fig. 2). As each eye would elevate, it would also extort. A vertical seesaw effect was observed during alternate cover testing, that is, the covered eye moved up as the uncovered eye moved down to fixate. A latent nystagmus was present with fast phase toward the uncovered eye. Her examination was otherwise unremarkable, both ophthalmologically and neurologically; cycloplegic refraction revealed her to be 0.75 diopters hyperopic, bilaterally. these two syndromes has not been previously recorded. FIG. 1. Decreased abduction with associated narrowing of contralateral palpebral fissures and apparent enophthalmos of the adducting eye in gaze right ( A) and gaze left ( B). JClin Neuro- ophthalmol, Vol. 10, No. 1, 1990 40 S. RIMMER AND B. KATZ with Duane's retraction syndrome and Marcus Gunn jaw winking. If dissociated vertical deviation is a neurogenic phenomenon, it is interesting to note its occurrence with the anomalous neuroanatomic substrate present in Duane's retraction syndrome. Certainly, most patients with dissociated vertical deviation do not have coexistent Duane's retraction syndrome, yet the concurrence of both syndromes implies that a clinically common strabismic entity can occur with differing underlying neuroanatomy. Acknowledgment: We acknowledge the support of The Pacific Vision Foundation and Research to Prevent Blindness Inc., New York, which made this work possible. ( Grants to BK.) REFERENCES 1. Stevens GT. Du strabisme vertical altemant et des deviations symetriques moins prononcees que Ie strabisme. Ann Ocul 1895; 113: 225- 32. 2. Helveston EM. Dissociated vertical deviation: a clinical and laboratory study. Trans Am Ophthalmol Soc 1980; 78: 73~ 79. 3. White JW. Paralysis of the superior rectus muscle. Trans Amer Ophthalmol Soc 1933; 31: 551- 85. 4. Chavasse B. In: Worth's Squint or The binocular rtiflexes and the treatment of strabismus, 7th ed. Philadelphia: P Blackiston's Son and Co., 1939: 240- 8. 5. Verhoeff F. Occlusion hypertropia. Arch Ophthalmol 1941; 25: 78~ 95. 6. Posner A. Noncomitant hyperphorias considered as aberrations of the postural tonus of the muscular apparatus. Am J OphthalmoI1944; 27: 1275- 9. 7. Scobee RG. The oculorotary muscles. 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