Ocular tilt reaction, internuclear ophthalmoplegia,and torsional nystagmus following mitralcommissurotomyP. K. Pandey, MD,a Anupam Singh, MS,a Barun Kumar, MD,b Shagun Sood, MBBS,a

Sanjeev Kumar, MBBS,a Ekta Kumari, MBBS,a and Meenakshi Chandel, MBBSa

Ocular tilt reaction, a type of skew deviation, and unilateral internu-clear ophthalmoplegia with torsional nystagmus resulting from anischemic event may result from unilateral disruption of otolithicpathways in themedial longitudinal fasciculus. A subset of skew de-viations is known to simulate superior oblique palsy; however, nonehave been reported with a coexisting internuclear ophthalmoplegia.The present report documents the rare occurrence of an oculartilt reaction simulating a left superior oblique palsy from involve-ment of left medial longitudinal fasciculus at the level ofinterstitial nucleus of Cajal.

Case Report

A50-year-old man presented to the Guru NanakEye Centre, Maulana Azad Medical College,New Delhi, with a sudden onset of horizontal,

vertical, and torsional diplopia, inability to move his lefteye inward, and oscillopsia. Diplopia worsened in the rightgaze and was relieved in the supine position and left gaze.No other associated visual symptoms, vertigo, or ataxiawere reported.No treatment was offered and his symptomsdid not improve.

On examination at our clinic 4 months later, the patientwas alert and maintained head tilt to the right; on neuro-logical examination, no sensory or motor deficits werenoted. Unaided visual acuity was 20/20 in both eyes; visualacuity was undiminished on vertical head shaking. Ptosis orlid retraction was not observed. Pupils were normal in sizeand reactive to light and near. Visual field testing was un-remarkable.

The patient had an exotropia of 8D while fixing with theright eye and 14D while fixing with the left eye, with a lefthypertropia of 6D. A maximum exotropia of 30D was notedon dextroversion and a maximum left hypertropia of 20D

was noted on dextroelevation. A left hypertropia of 4D

was present on right head tilt and 12D was present on lefthead tilt, suggesting a possible left superior oblique palsy

Author affiliations: aGuru Nanak Eye Centre, MAMC, New Delhi, India; bDepartment ofCardiology, Dr. RML Hospital, PGIMER, New Delhi, IndiaSubmitted November 26, 2011.Revision accepted June 6, 2012.Correspondence: Anupam Singh, MS, Guru Nanak Eye Center, Maulana Azad Medical

College, New Delhi 110002, India (email: dr.anupamsingh@gmail.com).J AAPOS 2012;16:484-486.Copyright � 2012 by the American Association for Pediatric Ophthalmology and

Strabismus.1091-8531/$36.00http://dx.doi.org/10.1016/j.jaapos.2012.06.004

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(Figure 1). Moderate limitation of adduction in the lefteye was present with decreased saccadic, smooth pursuit,and vestibulo-ocular movements (Figure 1). Abductionnystagmus was present in the right eye and torsionalnystagmus was noted in both eyes (Video 1, available atjaapos.org). Convergence was not affected.

On double Maddox prism testing, 10� of extorsion waspresent on right gaze in the right eye and 12� of intorsionwas present in left eye. In the supine position vertical devi-ation was reduced to 2D and subjective conjugate torsionwas 4� excyclotorsion in the right eye and 6� incyclotorsionin the left eye. Fundus photography confirmed the tor-sional findings (Figure 2A, B). A left internuclear ophthal-moplegia with a right ocular tilt reaction simulating a leftsuperior oblique palsy with torsional nystagmus due to anischemic event at the level of left interstitial nucleus ofCajal involving medial longitudinal fasciculus was sus-pected. Magnetic resonance imaging confirmed an ische-mic lesion at the suspected location (Figure 2C, D).

Discussion

The interneurons from the abducens nucleus ascend in thecontralateral medial longitudinal fasciculus to reach themedial rectus subnucleus with otolithic projections fromthe vertical semicircular canals. A unilateral lesion in themedial longitudinal fasciculus results in an ipsilateral inter-nuclear ophthalmoplegia and sometimes an ocular tilt re-action. Asymmetrical damage to the otolithic pathwaysmediating the vertical vestibulo-ocular reflex may producethis incomitant ocular tilt reaction, which can simulate anoblique muscle palsy.1,2 Damage to fibers correspondingto the anterior semicircular canal may simulatecontralateral superior oblique palsy, whereas damage tothe fibers corresponding to the posterior canal mayproduce ipsilateral inferior oblique palsy.3 Diagnosis maybe difficult because patients with internuclear ophthalmo-plegia and ocular tilt reaction may not complain of hori-zontal or vertical diplopia. If present, diplopia may bepresent only in certain gaze positions.

Conjugate torsion (intorsion of the hypertropic eye andextorsion of the hypotropic eye) is a hallmark finding inocular tilt reaction, whereas only the hypertropic eye is ex-torted in superior oblique muscle palsies. An ameliorationof vertical deviation and subjective torsion in the supine po-sition, as seen in our patient, is another marker of ocular tiltreaction.4 Evaluation of torsion and change in vertical

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FIG 1. External photographs of a patient in the primary position betraying a right ocular tilt reaction and in nine cardinal gazes with head tilts to theleft and the right.

FIG 2. A, B, Fundus photographs showing extorsion of the right and intorsion of the left eye. C, D, Magnetic resonance imaging of the brain showingthe ischemic lesion (arrows) involving left medial longitudinal fasciculus and interstitial nucleus of Cajal.

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deviation and torsion in the supine position can help to dis-tinguish oblique muscle palsies from ocular tilt reactions.

In conclusion, when evaluating an internuclear ophthal-molpelgia with a coexisting vertical deviation, the possibil-ity of the ocular tilt reaction should be considered andshould guide the search for a lesion on neuroimaging atthe interstitial nucleus of Cajal.

Literature Search

PubMed, MEDLINE, and Google were searched us-ing the following terms: conjugate torsion, internuclearophthalmoplegia, ocular tilt reaction, skew deviation, supe-

rior oblique palsy, mitral valve commissurotomy, ballon mi-tral valvulotomy, and percutaneus transvenous mitralvalvulotomy.

References

1. Brodsky MC, Donahue SP, Vaphiades M, Brandt T. Skew deviationrevisited. Surv Ophthalmol 2006;51:105-28.

2. Keane JR. Ocular skew deviation. Arch Neurol 1975;32:185-90.3. Donahue SP, Lavin PJM, Hamed LM. Tonic ocular tilt reaction

simulating superior oblique palsy. Arch Ophthalmol 1999;117:347-52.

4. Parulekar MV, Dai S, Buncic JR, Wong AMF. Head position depen-dent changes in ocular torsion and vertical misalignment in skew devi-ation. Arch Ophthalmol 2008;126:899-905.

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