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Show Journal of Ncitro- Ophthalmotogy 16( 3): 196- 198, 1996. © 1996 Lippincott- Ravcn Publishers, Philadelphia Convergence Nystagmus Associated With Spasmus Nutans Guy G. Massry, M. D., Jeffrey N. Bloom, M D., and Oscar A. Cruz, M. D. Spasmus nutans ( Greek: nodding spasm) is classically described as a benign clinical condition of infancy consisting of head nodding, torticollis, and nystagmus ( 1- 3). The nystagmus may be unilateral or bilateral, and is often asymmetric ( 3), of high frequency and low amplitude, and usually horizontal, although vertical and torsional forms have been described ( 4). We present a case of spasmus nutans with convergence nystagmus, a rare clinical entity seen in few ophthalmic conditions. CASE REPORT A 6- month- old Black infant was referred to the pediatric ophthalmology clinic with a 1- month history of head nodding and nystagmus. He was otherwise healthy, with no signs or symptoms of neurologic disease. He was not taking medication nor was there a family history of nystagmus. Ocular examination revealed central fixation of both eyes. Pupils measured 4 mm with no afferent pupillary defect or light- near dissociation. External examination revealed no torticollis. Motility evaluation demonstrated full versions and ductions, including normal elevation ( Fig. 1), and no evidence of strabismus. In primary gaze, at both distance and near fixation, there was a symmetric convergence nystagmus of high frequency and low amplitude that became conjugate in lateral gaze. There was no associated globe retraction, and optokinetic nystagmus testing was normal. Cyclople-gic refraction was - 3.50 + 1.00 x 180 OU, and Manuscript received November 30, 1994; accepted October 20, 1995. From the Department of Ophthalmology, Cardinal Glennon Children's Hospital, St. Louis University School of Medicine, St. Louis, Missouri, U. S. A. Address correspondence and reprint requests to Dr. Jeffrey N. Bloom, Department of Ophthalmology, Carle Clinic, 602 West University Avenue, Urbana, IL 61801, U. S. A. funduscopic examination revealed normal discs, maculae, vessels, and periphery OU. Magnetic resonance imaging ( MRI) of the brain, with contrast, was performed within 1 week of the child's presentation and was normal. The patient was followed closely over the next 2 years. Highlights of the follow- up examinations were: At 8 months of age, the head nodding resolved, but the patient now manifested a 35° left head turn ( Fig. 2). At 24 months of age, the torticollis began to alternate between a left and right head turn of equal magnitude ( again, 35°). Also at this time, the patient began to manifest a 15 prism diopter esotropia, and the frequency of the nystagmus began to decrease. There was no change in the amplitude or convergent nature of the nystagmus. Finally, at 28 months of age, the torticollis and nystagmus completely abated. Results of the remainder of the examination were unchanged from those of the initial examination at 6 months of age. All clinical findings described above were documented on videotape. DISCUSSION Spasmus nutans was first described by Raudnitz in 1897 ( 5) and to this day, its pathogenesis remains unknown. Onset is usually between ages 3- 18 months and the condition frequently spontaneously resolves by 3 years of age ( 6). Manifestations of spasmus nutans may mimic more serious conditions of the anterior visual pathway, such as optic nerve and chiasmal glioma ( 7- 13), and neu-roimaging may be required to determine the diagnosis. As previously mentioned, the nystagmus of spasmus nutans has been described as horizontal, vertical, or torsional ( 4). Convergence nystagmus is a rare clinical entity that occurs in the pretectal syndrome and Whipple's disease. The pretectal syndrome is character- 196 CONVERGENCE NYSTAGMUS AND SPASMUS NUTANS 197 FIG. 1. Despite the presence of convergence nystagmus, the patient demonstrated normal elevation of both eyes. ized by decreased upgaze, light- near dissociation, convergence retraction nystagmus, and lid retraction ( 14). Whipple's disease is an adult- onset multisystem disorder. Manifestations of this illness include diarrhea, arthritis, anemia, malaise, dementia, uveitis, and ophthalmoparesis ( 15). Convergence nystagmus has been noted in three cases of Whipple's disease associated with co-contraction of the masticatory muscles ( oculomas-ticatory myorhythmia) ( 16). The child described in our report developed nystagmus and head nodding at 5 months of age and torticollis at 8 months of age, had a normal MR1, and demonstrated resolution of all clinical findings by 28 months of age. This constellation of FIG. 2. Patient demonstrating 35° left head turn. ( Reproduced with parental permission) findings suggests the diagnosis of spasmus nutans. Although our patient did not concurrently manifest the classic triad of spasmus nutans, the coincidence of signs is not necessary for this diagnosis ( 4). The unique feature of this case is that the child presented with convergence nystagmus. In 1986, Chrousos et al. ( 17) reported on two children, both of whom had spasmus nutans and both of whom demonstrated near- evoked nystagmus documented by clinical observation and electro-oculographic recording. The authors suggested that in one child, the nystagmus was induced by fusional convergence ( retinal disparity), and that in the other child, by convergence- accommodation ( blurred image). In both children, the nystagmus was only present upon testing fixation at = s0.5 m and was absent at distance. Additionally, in neither case was the nystagmus convergent in nature. Our case represents a different clinical entity- convergence nystagmus, irrespective of the act of converging or accommodating, that is present and unchanged at distance and near. Since our patient neither manifested signs of the pretectal syndrome nor fit the clinical description of a patient with Whipple's disease, we had initially considered the diagnosis of spasmus nutans. However, it was not until the nystagmus resolved that we were certain of this diagnosis. To our knowledge, there are no previously published cases of spasmus nutans with convergence nystagmus other than an anecdotal reference by Dell'Osso, Daroff, and Troost ( 18). We believe that convergence nystagmus can occur with spasmus nutans, and, thus spasmus nutans should be considered in the differential diagnosis of convergence nystagmus in a child. Acknowledgment: Supported in part by the Knights Templar Eye Foundation, Inc. ( J. N. B.) and an Unrestricted Grant from Research to Prevent Blindness, Inc., New York. 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Schwartz MA, Selhort JB, Ochs AL, et al. Oculomasticatory myorhythmia: a unique movement disorder occurring in Whipple's disease. Ann Neurol 1986; 20: 677- 83. 17. Chrousos GA, Matsuo V, Ballen AE, Cogan DG. Near-evoked nystagmus in spasmus nutans. / Pediatr Ophthalmol Strabismus 1986; 23: 141- 3. 18. Dell'Osso LF, Daroff RB, Troost BT. Nystagmus and saccadic intrusions and oscillations. In: Tasman W, Jeager EA, eds. Duane's clinical ophthalmology. Rev ed, vol. 2. Philadelphia: J. B. Lippincott, 1995: 1- 30.' / Neuw- Ophthnlmol, Vol. 16, No. .1, J996 |
