Journal of Neuro-Ophthalmology最新文献

Background: Neuro-ophthalmology (NO) is a subspeciality of Ophthalmology, which represents more than an intersection of Neurology and Ophthalmology. The present report highlights the increasing importance of the subspeciality in Brazil and provides a unique retrospective study of the patient's clinical profile of a NO reference center.

Methods: Our study was retrospectively planned aiming to identify all neuro-ophthalmic cases of Instituto de Olhos of the Medical School Faculdade Ciências Médicas de Minas Gerais from August 2013 to March 2022.The first clinical diagnostic impression was selected from a predetermined list of 18 neuro-ophthalmologic conditions. Some NO conditions were eventually reclassified during the follow-up as the final clinical diagnosis impression. The concordance between the first and final clinical impressions was also investigated, as well as the patient's referral source.

Results: The sample comprised 903 patients from which 56.4% were female. The mean age was 51.48 ± 20.93 years. Males were more frequent in lower age groups <1 year (n = 3, 100%) and 1-9 years (n = 19/37, 51.4%). An external referral source represented 23.1%, and patients referred after basic ophthalmic consultation and from glaucoma service were 30.3% and 23.2%, respectively. The most encountered first clinical diagnostic impressions were isolated optic atrophy (13.1%), non-neuro-ophthalmic disease (11.7%), optic disc abnormalities (10.4%), ischemic optic neuropathies/retinal vascular occlusions (10.2%), and other visual field defects (9.0%). The kappa concordance coefficient among the first and final clinical diagnostic impressions was 0.53 (95% CI 0.48-0.59), indicating a moderate concordance level. The concordance among the most frequent diagnoses was lower in isolated optic atrophy (33%), other visual field defects (41%), and idiopathic optic neuritis (40%).

Conclusions: Due to the limited number of epidemiology studies in neuro-ophthalmology, we highlight the importance of a NO service in the public health system in Brazil. It may certainly contribute to better strategy plan assistance among professionals and health care managers. This report should seemingly stimulate other studies regarding the relevant and unique features of this subspeciality, which is undoubtedly increasing its importance among patients, and in the scientific community worldwide.

Abstract: An 84-year-old man with a genetically confirmed diagnosis of VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome treated with tocilizumab reported with a 3-day history of left eye pain, conjunctival chemosis and injection, and horizontal binocular diplopia. Examination revealed restriction of left eye ductions in all directions with mild unilateral proptosis. MRI of his brain and orbits demonstrated abnormal enhancement of the left posterior sclera and intraorbital optic nerve sheath, without involvement of the extraocular muscles, cavernous sinus, or optic nerve parenchyma. B-scan ultrasonography heralded a positive "T-sign." Empiric treatment with high-dose intravenous methylprednisolone led to rapid clinical improvement. Workup for alternative causes was unremarkable. Oral prednisone dose and tocilizumab ultimately resulted in complete resolution after 4 weeks. The final diagnosis was consistent with orbital inflammatory manifestations of VEXAS syndrome.

Background: Pseudotumor cerebri (PTC) syndrome is a disorder of increased intracranial pressure, most commonly affecting overweight women of childbearing age. Malignant PTC (MPTC) is a rare presentation that involves rapidly worsening vision, often necessitating surgical intervention to prevent permanent vision loss. The goal of this study was to determine whether radiographic findings of PTC are predictive of MPTC and the final visual outcome.

Methods: Charts of patients diagnosed with PTC based on the modified Dandy criteria were reviewed. Snellen best-corrected visual acuity (BCVA), automated perimetry mean deviation (APMD), and papilledema grade were documented from the initial and final follow-up examinations. The MRI images were read by 2 neuroradiologists blinded to the clinical diagnosis, and consensus findings were obtained. The patients were classified into malignant and nonmalignant PTC groups. Malignant was defined as BCVA ≤ 20/200 or APMD ≤ -7 dB, and acute onset of symptoms. The sensitivity and specificity of each MRI finding for detecting MPTC was determined. Generalized linear model with random effect and backward model selection was performed to identify which radiographic findings were predictive of final BCVA, APMD, and papilledema grade.

Results: Overall, 114 eyes (57 malignant/57 nonmalignant) were included. No MRI finding has both sensitivity and specificity for MPTC reaching 60%. The highest sensitivity (86%) was flattening of the posterior globes and unilateral or bilateral transverse sinus stenosis. The highest specificities were completely empty sella (86%), enhancement (84%), and diffusion restriction (81%) of the prelaminar optic nerves. The presence of vertical tortuosity of the optic nerves was predictive of better final BCVA ( P = 0.003) and better final APMD ( P = 0.011). While papilledema grade was higher in MPTC, no MRI finding was predictive of papilledema grade.

Conclusions: No single MRI finding has both high sensitivity and specificity for diagnosing MPTC. Vertical tortuosity of the optic nerves may be predictive of better final BCVA and APMD.