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We visited a 57‐year‐old man with horizontal diplopia. His problems had started 5 years earlier with progressive gait disturbance followed by falling backward, low‐tone speech, and drooling. He was also suffering from binocular nonfluctuating diplopia since the previous year. Examination revealed bilateral exotropia on primary position with very slow saccadic movements. Vertical eye movements were severely limited. On horizontal movements, he had severe limited adduction of both eyes with nystagmus on the abducting eye. These abnormalities could be overcome by oculocephalic maneuver. The convergence was also impaired (Video S1). In addition, there was slowness of all movements and severe postural instability on the pull test (Video S2).
Which of the following is a better explanation of eye movement abnormalities in this progressive supranuclear palsy (PSP) patient?
Coincidental ocular myasthenia
Coincidental internuclear ophthalmoplegia as a result of a structural lesion
Wall‐eyed bilateral internuclear ophthalmoplegia (WEBINO) as an ocular finding in PSP
Answer
C. WEBINO as an ocular finding in PSP. Parkinsonism with recurrent backward falls, vertical supranuclear gaze palsy, and a typical humming‐bird sign on brain MRI (Supplementary Figure S1) are highly suggestive of PSP. Brain magnetic resonance imaging did not show any other structural lesion to explain the diplopia and abnormal eye movements. To rule out accompanying myasthenia, single‐fiber electromyography and serum levels of antiacethylcholine receptor and anti–muscle specific tyrosine kinase antibodies were checked, and all of them were normal. We concluded that diplopia and abnormal horizontal eye movements in this case are compatible with WEBINO.
WEBINO is a rare neurological syndrome characterized by exotropia in the primary gaze, limited adduction of the eyes, and convergence impairment. It is usually attributed to lesions of mesencephalic tegmentum involving medial longitudinal fasciculi or exaggerated excitation of both paramedian pontine reticular formation.1, 2, 3
In addition to supranuclear gaze palsy, other eye movement abnormalities reported in PSP include lid retraction, square wave jerks, lid opening apraxia, and blepharospasm and rarely internuclear ophthalmoplegia and WEBINO.1, 2, 3
Author Roles
(1) Research Project: A. Conception, B. Organization, C. Execution; (2) Manuscript Preparation: A. Writing of the First Draft, B. Review and Critique.
N.Y.: 1A, 2A
M.R.G.: 1C
A.B.: 2B
M.R.: 1A, 1B, 1C, 2B
Disclosures
Ethical Compliance Statement
The authors confirm that the approval of an institutional review board was not required for this work. We confirm that we have read the Journal's position on issues involved in ethical publication and affirm that this work is consistent with those guidelines. Informed consent was obtained from the patient.
Funding Sources and Conflict of Interest
The authors report no sources of funding and no conflicts of interest.
Financial Disclosures for the Previous 12 Months
The authors have nothing to disclose.
Supporting information
Supplementary Fig. 1. Sagittal T2 weighted magnetic resonance imaging (left side) revealing humming‐bird sign (red arrow) and axial T2 showing midbrain tegmental atrophy (right side). There is no other structural lesion explaining abnormal eye movements.
Video S1. Bilateral exotropia on primary position with severe limited adduction of both eyes and nystagmus on the abducting eye. Vertical eye movements are severely limited mostly downward. Convergence of both eyes is impaired. These abnormalities are overcome by oculocephalic maneuver.
Video S2. Severe postural instability on pull test.
Acknowledgments
The authors acknowledge the patient and his family members for consenting to participate in this study.
Relevant disclosures and conflicts of interest are listed at the end of this article.
References
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Associated Data
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Supplementary Materials
Supplementary Fig. 1. Sagittal T2 weighted magnetic resonance imaging (left side) revealing humming‐bird sign (red arrow) and axial T2 showing midbrain tegmental atrophy (right side). There is no other structural lesion explaining abnormal eye movements.
Video S1. Bilateral exotropia on primary position with severe limited adduction of both eyes and nystagmus on the abducting eye. Vertical eye movements are severely limited mostly downward. Convergence of both eyes is impaired. These abnormalities are overcome by oculocephalic maneuver.
Video S2. Severe postural instability on pull test.