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

INTRODUCTION: 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. CASE REPORT: 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. CONCLUSIONS: 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.

Bilateral Choroidal Metastases from Endobronchial Carcinoid Treated with Somatostatin Analogues.

OBJECTIVE: To describe a patient with bilateral multifocal choroidal metastases from an endobronchial carcinoid treated with a somatostatin analogue. METHOD: A 60-year-old woman presenting with photopsia in the left eye underwent an extensive ophthalmic examination, including fluorescein angiography, OCT and ultrasound. RESULTS: Fundoscopy revealed a small retinal tear in the left eye, for which she received laser treatment. In addition, choroidal masses were detected in both eyes. Her medical history of a pneumectomy for a bronchial carcinoid six years earlier together with recent elevated chromogranin A blood levels prompted a diagnosis of choroidal metastases. Subsequently, a Gallium-68 DOTANOC positron emitting tomography/computer tomography scan revealed a spinal cord metastasis and mediastinal as well as mesenterial lymph node invasion. Systemic treatment with Sandostatin®, a somatostatin analogue was started. Up until two years after the initial presentation and treatment, these choroidal lesions remained stable without any signs of growth. CONCLUSION: Endobronchial carcinoid tumors have an indolent nature and long-term follow-up is recommended for early detection of metastases. Although treatment with somatostatin analogues rarely induces complete tumor regression, tumor stabilization and prevention of symptoms related to hormone secretion is achieved. This well-tolerated systemic treatment provides a worthy alternative treatment for choroidal metastasis compared to classic radiotherapy without any risk of radiation or laser-related visual loss.

The effects of macular ischemia on visual acuity in diabetic retinopathy.

PURPOSE: To investigate the impact of diabetic macular ischemia (DMI) on visual acuity (VA), through the analysis of novel fluorescein angiography (FA) parameters. METHODS: Data were retrospectively collected over a 6-month period. DMI severity was graded using Early Treatment Diabetic Retinopathy Study (ETDRS) protocols. Custom software was used to quantify areas of the foveal avascular zone (FAZ), and of capillary nonperfusion over the papillo-macular nerve fiber layer bundle, and temporal macula, and associations tested with VA. RESULTS: A total of 488 patients with type 2 diabetes mellitus and FAs of sufficient quality to allow detailed quantitative analyses were included. ETDRS-DMI SEVerity was graded as: none, 39.7%; questionable, 18.4%; mild, 25.2%; moderate, 11.0%; and severe, 5.6%. Median FAZ areas were 0.19 mm(2) (interquartile range [IQR], 0.13-0.25); 0.25 mm(2) (IQR, 0.18-0.32); 0.27 mm(2) (IQR, 0.19-0.38); 0.32 mm(2) (IQR, 0.25-0.54); and 0.78 mm(2) (IQR, 0.60-1.32), respectively, and were significantly different between all grades (P < 0.002), apart from "questionable" versus "mild" grades. Significant association of VA to FAZ area was observed only in the moderate (ß = 0.406, SE = 0.101, P = 0.001) and severe (ß = 0.299, SE = 0.108, P = 0.006) subgroups, but not in milder ETDRS-DMI grades. A strong association with VA was observed in cases with papillomacular ischemia (ß = 1.123, SE = 0.355, P = 0.005), independent of FAZ size or the presence of macular edema. CONCLUSIONS: Diabetic macular ischemia is associated with reduced VA in eyes with moderate to severe ETDRS-DMI grades of ischemia but preserved in milder grades. In addition, we describe the independent association of papillomacular nerve fiber bundle ischemia with reduced VA.