Journal of Neuro-Ophthalmology最新文献

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.

Background: Alexia without agraphia (AWA) is an acquired reading disturbance associated with left posterior cerebral artery (PCA) infarction. Based on autopsy and neuroimaging, there are two explanations for its pathogenesis: a visual cortex-language cortex disconnection and a visual word-form agnosia. Our goal was to discover if more refined brain imaging in a case-control study would provide further imaging support for either of these hypotheses.

Methods: A neuroradiologist masked to the presence of AWA reviewed diffusion-weighted and fluid-attenuated inversion recovery brain MRIs in patients who had left PCA infarctions with AWA (9 patients) or without AWA (18 patients) to characterize lesions in the splenium, left forceps major, and left fusiform gyrus.

Results: Patients with AWA had various combinations of lesions in the splenium, left forceps major, and left fusiform gyrus. One of these regions was involved in 3 patients, 2 were involved in 3 patients, and 3 were involved in 3 patients. Most (89%) patients without AWA lacked involvement of any of these 3 regions.

Conclusions: Our data provide imaging evidence for the two existing hypotheses of AWA. Involvement of any of these lesions on MRI imaging should encourage clinicians to obtain further neuropsychological testing.

Background: The differentiation of Horner syndrome from physiological anisocoria is important yet clinically challenging. We investigated the diagnostic accuracy of pupillometry to discriminate Horner syndrome from physiological anisocoria compared to pharmacological testing with the alpha-2-agonist apraclonidine, which is considered the current gold standard.

Methods: Forty-four adult patients, mostly referred to our neuro-ophthalmology service for evaluation of anisocoria, were included. Automated binocular pupillometry was performed under standardized light conditions before and >30 minutes after instillation of 1% apraclonidine eye drops. A positive apraclonidine test indicating unilateral Horner syndrome was defined as an increase of pupil size in the smaller pupil and decrease of size in the larger pupil. Receiver operator characteristic curves were calculated to find the best pupillometric parameter discriminating Horner syndrome from physiological anisocoria.

Results: We found that the parameters measuring the pupillary dilation lag using pupillometry could reliably discriminate Horner syndrome from physiological anisocoria compared to pharmacological testing. Calculating the change of anisocoria at 3-4 seconds after light-off relative to the anisocoria at the end of the light-on period (Δ3-4) may be most suitable to rule out Horner syndrome reaching a sensitivity of 95% and specificity of 68% using a cutoff of 0.35 mm.

Conclusions: Our results indicate that pupillometry is a robust tool to measure the dilation lag in Horner syndrome and, therefore, to distinguish pathological from physiological anisocoria obviating pharmacological testing. The high sensitivity of the test will allow to identify the patients with Horner syndrome requiring further investigation.

Background: OpenAI, the owner of ChatGPT, publicly released the GPT-4 Vision in September 2023. This multimedia chatbot has the capability to receive and analyze various images presented to it by the user. We assessed the accuracy of its interpretation of 2 of the images commonly used in neuro-ophthalmology, namely Hess screen and automated visual field images.

Methods: We separately uploaded typical images of 5 abnormal Hess screen charts related to third, fourth, and sixth cranial nerve palsy, Brown syndrome, and inferior orbital wall fracture with entrapment of the inferior rectus muscle. Likewise, 5 classic images of automated visual field grayscale maps related to lesions of the optic nerve, the chiasma, the optic tract, the optic radiations, and the occipital lobe were presented. The chatbot was instructed to select the best option among the 5 choices presented in each question.

Results: The GPT-4 Vision was able to select the right choice in 2/5 questions on Hess screens and 3/5 of the visual field questions. Despite selection of the correct option, qualitative evaluation of GPT-4 responses revealed flawed analysis of certain aspects of some image findings, such as the side of involvement or the misinterpretation of the physiologic blind spot as a central scotoma.

Conclusions: The performance of GPT-4 Vision in the interpretation of abnormalities of Hess screen and visual field involvement was highly variable, even with simple typical cases of classic disorders. As the chatbot's image recognition is currently evolving, its capacity to accurately interpret ophthalmologic images is still limited at this time.