Wall-eyed Bilateral Internuclear Ophthalmoplegia (WEBINO) in a Patient With Idiopathic Intracranial Hypertension

Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) is a variant syndrome of internuclear ophthalmoplegia, consisting of primary gaze exotropia, adduction impairment, nystagmus of the abducting eye, and vertical gaze-evoked nystagmus. It seems to be most frequently associated with multiple sclerosis, although other etiologies such as brainstem ischemia or hydrocephalus have also been described.

We report the case of a 25-year-old woman who presented with subacute progressive oculomotor disturbances, resulting in the development of a WEBINO over a few days. Fundoscopy showed papilledema first in the right and afterwards also in the left eye. Brain magnetic resonance imaging was normal. Lumbar puncture demonstrated an opening pressure of 38 cm H2O, without pleiocytosis and with normal protein. As no other cause of intracranial hypertension could be identified by imaging or extensive biochemical testing, the patient was treated with acetazolamide for idiopathic intracranial hypertension. As there was further progression despite increase of acetazolamide dosing, more aggressive therapy was pursued, and a ventriculoperitoneal shunt was placed by our neurosurgeons. Clinical follow-up showed progressive recovery of normal oculomotor function and disappearance of papilledema over the course of 6 weeks.

To our knowledge this is the first case description of a patient with WEBINO and idiopathic intracranial hypertension. The diagnosis is supported by the very high opening pressure, the absence of neuroimaging abnormalities, the papilledema, and the response to ventriculoperitoneal drainage.