Keereman Vincent, Platteau Elise, Crevits Luc, Algoed Luc
Department of Neurology, Ghent University Hospital.
Departments of Ophthalmology.
Neurologist. 2018 Sep;23(5):157-159. doi: 10.1097/NRL.0000000000000192.
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.
外展神经麻痹性双眼核间性眼肌麻痹（WEBINO）是核间性眼肌麻痹的一种变异综合征，包括原在位外斜视、内收障碍、外展眼眼球震颤以及垂直注视诱发眼球震颤。尽管也有其他病因（如脑干缺血或脑积水）的报道，但它似乎最常与多发性硬化相关。
我们报告一例25岁女性患者，其出现亚急性进行性动眼神经功能障碍，在数天内发展为WEBINO。眼底检查最初在右眼发现视乳头水肿，随后左眼也出现。脑部磁共振成像正常。腰椎穿刺显示初压为38 cm H2O，无细胞增多且蛋白正常。由于影像学检查或广泛的生化检测未发现其他颅内高压病因，该患者接受乙酰唑胺治疗特发性颅内高压。尽管增加了乙酰唑胺剂量仍有进一步进展，故采取了更积极的治疗措施，我们的神经外科医生为患者进行了脑室腹腔分流术。临床随访显示在6周内动眼神经功能逐渐恢复正常且视乳头水肿消失。
据我们所知，这是首例关于WEBINO合并特发性颅内高压患者的病例描述。极高的初压、神经影像学无异常、视乳头水肿以及对脑室腹腔引流的反应支持了该诊断。
