ORIGINAL CONTRIBUTION

Wall-Eyed Bilateral Internuclear Ophthalmoplegiain a Patient with

Progressive Supranuclear PalsyMunetaka Ushio, MD, Shinichi Iwasaki, MD, Yasuhiro Chihara, MD, and Toshihisa Murofushi, MD

Abstract: Wall-eyed bilateral internuclear ophthal-moplegia (WEBINO) is a rare disorder consisting ofa bilateral adduction deficit and primary gaze posi-tion exotropia. Associated with bilateral mediallongitudinal fasciculus lesions, it has been mostlyreported in patients with multiple sclerosis andbrainstem stroke. A 72-year-old man with character-istic clinical features of progressive supranuclearpalsy (PSP) later developed WEBINO. Brain MRIrevealed atrophy of the midbrain tegmentum. Caloricirrigation revealed intact horizontal eye movementsin both eyes. We believe this to be the first report ofWEBINO in PSP. The preservation of vestibulo-ocular horizontal eye movements supports the notionthat the WEBINO in this condition was caused bya supranuclear rather than a nuclear lesion and sug-gests the possibility that even in other causes ofWEBINO, the lesion is supranuclear and not in themedial rectus subnucleus as is often suggested.

(J Neuro-Ophthalmol 2008;28:93-96)

all-eyed bilateral internuclear ophthalmoplegia (WE-BINO) is a rare variation of internuclear ophthal-

moplegia associated with lesions of the medial longitudinalfasciculus (MLF) bilaterally (1). The distinguishing fea-tures of WEBINO are adduction deficits on lateral gaze toboth sides and exotropia in primary gaze position. The termis attributed to Lubow according to Hoyt and Daroff in1971 (2). Corresponding lesions of the MLF on both sideswere pathologically revealed later in an autopsy study (3).

Department of Otolaryngology (MU, SI, YC), Graduate School ofMedicine, University of Tokyo, Tokyo, Japan; and Department ofOtolaryngology (TM), Tokyo Postal Services Agency Hospital, Tokyo,Japan.

Address correspondence to Munetaka Ushio, MD, Department ofOtolaryngology, Graduate School of Medicine, University of Tokyo, 7-3-1Hongo, Bunkyo-ku, Tokyo 113-8655, Japan. E-mail: IZT01356@nifty.ne.jp

The most common causes of internuclear ophthalmoplegiaand its WEBINO variant are demyelination and stroke (1,3-5). Internuclear ophthalmoplegia has also been reportedrarely in patients with progressive supranuclear palsy (PSP)(6-8), but to the best of our knowledge, its WEBINOvariant has not been reported in PSP. We report such a case.

CASE REPORTA 72-year-old man was referred to our clinic with

complaints of diplopia and gait disturbance. He had notedmild dysarthria and resting tremor in the right upper extrem-ity for 11 years. Parkinson disease had been diagnosedearlier, and he had undergone levodopa therapy. However, ithad proved ineffective. For 7 years, he had noted a frozengait, mild disequilibrium, difficulty in writing, and mildrecent memory disturbance. Amantadine hydrochloridetherapy had been effective in alleviating the resting tremor.A disorder of postural reflexes, dysarthria, and dementiagradually developed thereafter. For 1 year, he had noteddiplopia on lateral gaze bilaterally. Bilateral internuclearophthalmoplegia with primary gaze position exotropiawas identified. Past medical and family histories werenoncontributory.

On our examination, the patient displayed milddementia (15/30 on Mini Mental State Examination).Visual acuity and fields were normal in both eyes. Pupilswere of normal size, equal in diameter, and normallyreactive to light. The magnitude of the exotropia of the lefteye (right eye fixating) in primary gaze position was 50prism diopters; the magnitude of the exotropia of the righteye (left eye fixating) was 55 prism diopters using theKrimsky test. The patient showed an adduction deficit onlateral gazing bilaterally. Vertical eye movements wereseverely limited. Convergence was absent (Fig. 1).

Distal and proximal wasting and weakness were notobserved in any of the four extremities. Although he dis-played a mask-like facies, frozen gait, and nuchal rigidity,he was able to stand and walk with aids. Deep tendonreflexes were normal except at the patella, where they were

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absent. Extensor plantar reflexes were seen bilaterally. Noloss of sensation was evident.

Brain MRI revealed atrophy of the midbrain teg-mentum (Fig. 2). The pons, cerebellum, and basal gangliashowed no evidence of atrophy.

Spontaneous, positional, and positioning nystagmuswere not seen with an infrared charge-coupled device(CCD) camera. On electronystagmography (AC-coupled;time constant of original recording = 3 seconds, time con-stant of eye velocity = 30 milliseconds, upper frequencylimit =10 Hz), he showed smooth pursuit in the horizontalplane. Precise calibration was precluded by the ophthal-moplegia, but an estimate of ductional amplitudes could beattained by clinical observation. Neither eye was able toadduct past the midline with saccades or smooth pursuit,but oculocephalic maneuvers during attempted fixation ofan object in light elicited a full range of horizontal eyemovements (Fig. 3A). On lateral gaze, dissociated nys-tagmus on abduction was observed bilaterally (Fig. 3B).Although vertical eye movements, including saccadesand smooth pursuit, were severely limited, oculocephalicmaneuvers during attempted fixation of an object in light

elicited a full range of vertical eye movements (Fig. 3C). Oncaloric tests using ice water, each eye adducted andabducted to the full horizontal range contralateral to the

FIG. 2. T2 sagittal brain MRI reveals atrophy of midbraintegmentum (arrow).

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Wall-Eyed Bilateral Internuclear Ophthalmoplegia J Neuro-Ophthalmol, Vol. 28, No. 2, 2008

pursuit oculo-cephalic maneuver

right eye~\A

- J .U~ J \L J kV

left eye

100 p. V

1100 ^ V

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100 (U. V

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right eye

r

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FIG. 3. Eye movement recordings. Horizontal eye move-ments were recorded from each eye. Vertical eye movementswere recorded from the left eye. The upper traces indicatethe eye movement and the lower traces indicate eye velocity,respectively. Dashed lines (A, C) indicate the range ofoculocephalic eye movements. A. Horizontal pursuit isrelatively smooth; monocular nystagmus is present inabduction bilaterally. Although adduction deficits are presentbilaterally, oculocephalic maneuvers during attemptedfixation of an object in light elicit a full range of eyemovements. B. Horizontal saccades. Nystagmus is present onabduction bilaterally (arrows). C. Vertical eye movements.Although vertical pursuit movements are severely reduced,oculocephalic maneuvers during attempted fixation of anobject in light elicit a full range of movements.

in response to clicks at 95 dB normal hearing level (9),showed normal responses on both sides. Normal ocularcounter-rolling was observed under the infrared CCDcamera. Other cranial nerves functioned normally byclinical examination.

On the basis of these findings, PSP with WEBINOwas diagnosed. Gait disturbance and ophthalmoplegia havedisplayed gradual deterioration despite medication.

D I S C U S S I O NOur patient displayed the typical features of PSP and

was distinctive only in having a WEBINO, which has notbeen previously reported in this condition. As the lesions ofPSP characteristically involve the pontine and midbrain teg-mentum and those of WEBINO involve the same regions(1,10), the appearance of WEBINO in PSP is not sur-prising. Brain MRI for this patient did reveal atrophicchanges in the midbrain tegmentum, a finding previouslydescribed in WEBINO (11).

In this patient, horizontal and vertical eye move-ments, including saccades and smooth pursuit, werelimited, whereas oculocephalic and caloric maneuverselicited full range of movements. These findings suggestthat the vestibulo-ocular reflex is intact and that theophthalmoplegia in this patient probably originated froma supranuclear lesion (12). McGettrick et al (1) havesuggested that an oculomotor nucleus lesion accounts forthe exotropia in patients with WEBINO, but Gonyea (3)reported that the oculomotor nucleus was not involved. Thefindings in our patient favor Gonyea's observation.

This patient also showed loss of convergence, but thisfinding does not necessarily support the postulate that theexotropia of WEBINO is due to an oculomotor nuclearlesion (14). Impaired convergence is common in olderadults. In our patient, the cause of the convergence disorderis unclear.

We believe that the WEBINO in this patient wascaused by extension of the PSP lesions into the midbraintegmentum. The preservation of vestibulo-ocular move-ments supports the notion that the WEBINO was caused bya supranuclear rather than a nuclear lesion and suggests thatWEBINO in demyehnation and stroke may also be ofsupranuclear origin.

AcknowledgmentWe thank Ms. Masako Nakamura, BS, for technical

assistance.

stimulated side, and no quick phases of nystagmus wereobserved. Vestibular evoked myogenic potentials (VEMPs),which were recorded from the sternocleidomastoid muscles

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