Journal of Pediatric Ophthalmology and Strabismus

Abnormal Eye Movements in Rubella Syndrome

Joseph H Goldstein, MD; Mohinder K Gupta, MD

Abstract

The clinical findings in congenital rubella have been well described in several reviews.K1 Eye findings include disturbance of the cornea, iris, lens, retina, as well as glaucoma, strabismus, and nystagmus.

Nystagmus is a not infrequent finding. In one series, it was found in 13 percent of cases.' It was described as pendular or searching. Latent nystagmus and jerk type were also noted.

It is the purpose of this report to call attention to asymmetric nystagmus and ocular flutter, as two other types of abnormal eye movements in congenital rubella.

CASE REPORT

A 4-1/2 year-old Black male child was first examined on October 24, 1975. His parents had noted quivering movements of the left eye and progressive whitening of the left pupil since early childhood.

His mother experienced a febrile illness accompanied by morbilliform rash lasting three days during the first trimester of pregnancy. Birth weight was normal. Physical and mental development progressed normally. There was no family history of ocular or neurological disease.

The general physical examination and detailed examination of the cardiovascular system, respiratory system, gastrointestinal, and genitourinary system revealed no abnormality. Examination of the nervous system revealed normal intellect, speech, orientation to three spheres, and intact cranial nerves. Sensory and motor systems were normal. Examination of the cerebellar system including finger to nose test, repeated alternating movements, walking on toes and heels, and ability to stand erect with feet together with eyes open and eyes closed was normal.

Visual acuity was 20/20 with line letters in the right eye and hand movements at one foot in the left eye. The left eye showed rapid large amplitude horizontal pendular nystagmus in primary position changing to jerk nystagmus in extreme positions of gaze with almost no movement in the right eye (Fig. 1 ). Associated with asymmetric nystagmus there was noted a short burst of horizontal oscillations of the eye during fixation in the primary position (Fig.2). No head nodding was noted. Both corneas were clear and measured 12 mm X 11.5 mm in the right eye and 10 mm X 9 mm in the left eye. The anterior chamber and iris were normal. The pupils were round, regular and equal and reacted normally to light on direct and consensual stimulation. The left lens was found to be totally cataractous while that of the right eye was normal. Ophthalmoscopy of the right eye showed the disc, blood vessels, macula and general fundus to be normal. The presence of a mature cataract prevented visualization of the left fundus. The anterior-posterior diameter of the eye was 27 mm in each eye determined by A-scan ultrasonography. Serum exa mination for viral titres revealed. Herpes Hominis titres 8, Rubella Hi-32, Cytomegalovirus less than 14.

DISCUSSION

Asymmetric nystagmus is an infrequent finding. It may be associated with certain well defined entities such as spasmus nutans, latent nystagmus, internuclar ophthalmoplegia and may signify "a lesion of the brain stem.''4 Nathanson5 described three cases of acquired monocular nystagmus, two of whom had disseminated sclerosis and one a brain steam disorder. Donin6 reviewed the causes of monocular nystagmus in children and reported a case with astrocytoma. No involvement of the brain stem or cerebellum was noted. These cases represented monocular nystagmus rather than asymmetric nystagmus.

The present case showed periods of marked asymmetric nystagmus with almost no movement in the fixing eye and rapid large amplitude pendular nystagmus in the affected eye.

The second unusual finding is that of ocular flutter. Flutter*4'6 is a rapid eye movement occurring during fixation or during saccadic movement. Movements are 5 to 10 degrees with a rate of 10 cycles per second.…

The clinical findings in congenital rubella have been well described in several reviews.K1 Eye findings include disturbance of the cornea, iris, lens, retina, as well as glaucoma, strabismus, and nystagmus.

Nystagmus is a not infrequent finding. In one series, it was found in 13 percent of cases.' It was described as pendular or searching. Latent nystagmus and jerk type were also noted.

It is the purpose of this report to call attention to asymmetric nystagmus and ocular flutter, as two other types of abnormal eye movements in congenital rubella.

CASE REPORT

A 4-1/2 year-old Black male child was first examined on October 24, 1975. His parents had noted quivering movements of the left eye and progressive whitening of the left pupil since early childhood.

His mother experienced a febrile illness accompanied by morbilliform rash lasting three days during the first trimester of pregnancy. Birth weight was normal. Physical and mental development progressed normally. There was no family history of ocular or neurological disease.

The general physical examination and detailed examination of the cardiovascular system, respiratory system, gastrointestinal, and genitourinary system revealed no abnormality. Examination of the nervous system revealed normal intellect, speech, orientation to three spheres, and intact cranial nerves. Sensory and motor systems were normal. Examination of the cerebellar system including finger to nose test, repeated alternating movements, walking on toes and heels, and ability to stand erect with feet together with eyes open and eyes closed was normal.

Visual acuity was 20/20 with line letters in the right eye and hand movements at one foot in the left eye. The left eye showed rapid large amplitude horizontal pendular nystagmus in primary position changing to jerk nystagmus in extreme positions of gaze with almost no movement in the right eye (Fig. 1 ). Associated with asymmetric nystagmus there was noted a short burst of horizontal oscillations of the eye during fixation in the primary position (Fig.2). No head nodding was noted. Both corneas were clear and measured 12 mm X 11.5 mm in the right eye and 10 mm X 9 mm in the left eye. The anterior chamber and iris were normal. The pupils were round, regular and equal and reacted normally to light on direct and consensual stimulation. The left lens was found to be totally cataractous while that of the right eye was normal. Ophthalmoscopy of the right eye showed the disc, blood vessels, macula and general fundus to be normal. The presence of a mature cataract prevented visualization of the left fundus. The anterior-posterior diameter of the eye was 27 mm in each eye determined by A-scan ultrasonography. Serum exa mination for viral titres revealed. Herpes Hominis titres 8, Rubella Hi-32, Cytomegalovirus less than 14.

Fig. 1. Top OS. bottom O.O.-E/ectroocufography showing asymmetric nystagmus.

Fig. 1. Top OS. bottom O.O.-E/ectroocufography showing asymmetric nystagmus.

Fig. 2. Top OS. bottom O.D.-Electrooculography demonstrating ocular flutter.

Fig. 2. Top OS. bottom O.D.-Electrooculography demonstrating ocular flutter.

DISCUSSION

Asymmetric nystagmus is an infrequent finding. It may be associated with certain well defined entities such as spasmus nutans, latent nystagmus, internuclar ophthalmoplegia and may signify "a lesion of the brain stem.''4 Nathanson5 described three cases of acquired monocular nystagmus, two of whom had disseminated sclerosis and one a brain steam disorder. Donin6 reviewed the causes of monocular nystagmus in children and reported a case with astrocytoma. No involvement of the brain stem or cerebellum was noted. These cases represented monocular nystagmus rather than asymmetric nystagmus.

The present case showed periods of marked asymmetric nystagmus with almost no movement in the fixing eye and rapid large amplitude pendular nystagmus in the affected eye.

The second unusual finding is that of ocular flutter. Flutter*4'6 is a rapid eye movement occurring during fixation or during saccadic movement. Movements are 5 to 10 degrees with a rate of 10 cycles per second. According to Duke-Elder3 it is "typical of cerebellar disease." Ina series of cases with ocular flutter, Cogan7 noted that cerebellar signs were "outstanding and that one may justifiably infer that the eye signs were also due to the cerebellar disease."

In both cases of ocular flutter reported by Goldberg and Jampel,8 cerebellar findings predominated. One case had progressive cerebellar degenerative disease and the other multiple sclerosis with cerebellar signs.

It is quite apparent that the presence of ocular flutter is almost invariably indicative of cerebellar or cerebellar pathway disease. No clinical indication of cerebellar involvement was found in the case presented. Since it is easy to overlook flutter in patients with nystagmus, a more careful search for both flutter and other cerebellar signs would seem indicated. Pathological studies of the nervous system in infants with congenital rubella syndrome by Rorke9 indicated some abnormality of cerebral blood vessels, but no specific cerebellar disease.

This case would seem to be the first indication of cerebellar pathway disease in rubella.

SUMMARY

A patient with a characteristic picture of rubella eye disease is presented. Its interest lies in the associated finding of asymmetric nystagmus and ocular flutter. The presence of flutter would indicate cerebellar or cerebellar pathway disease, a previously unassociated finding.

REFERENCES

1. Wolff SM: The ocular manifestation of congenital rubella. Trans Amer Ophthal Soc 70:577-614, 1972,

2. Wolff SM: The ocular manifestation of congenital rubella. J Ped Ophthal 10:101-141, 1973.

3. Duke-Elder S (ed): System of ophthalmology. In Neuro-ophthalmology, Vol. XII, St. Louis, CV Mosby Co,, 1971, p 851.

4. Walsh FB, Hoyt WF: Clinical Neuro-ophthalmology, Baltimore, Williams & Wilkin Co., Vol III, p 2212, 1969.

5. Nathanson M, Bergman PS, Bender MB: Monocular nystagmus. Amer J Ophthal 40:685-692, 1955.

6. Donin JF; Acquired monucular nystagmus in children. Cañad J Ophthal 2:212-215, 1967.

7. Cogan DG: Ocular Dysmetria: Flutter like oscillation of the eyes and opsoclonus. Arch Ophthal 51:318-335, 1954.

8. Goldberg RT. Jampel RS: Flutter like oscillations of the eyes in cerebellar disease. Amer J Ophthal 55:1229-1233, 1963.

9. Rorke LB: Nervous system lesions in the congenital rubella syndrome. Arch Otolaryng 98:249-251. 1973.

10.3928/0191-3913-19770901-06

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