Multicenter Validation of the Ocular Myasthenia Gravis Rating Scale Questionnaire.

Background And Objectives: Ocular myasthenia gravis (OMG) causes disabling ocular symptoms of ptosis and diplopia, but a validated disease-specific patient-reported outcome measure (PROM) has not been reported. We sought to validate a novel PROM for OMG, OMG Rating Scale Questionnaire (OMGRate-q), as a measure of visual functioning to support patient-centered care.

Methods: This was a prospective study of patients aged 18 years and older with OMG receiving care at 3 medical centers (January 2022-October 2023).

Central Retinal Artery Occlusion Associated with Takayasu Arteritis.

Takayasu arteritis is a chronic inflammatory vasculitis with granulomatous panarteritis particularly impacting large vessels including the aorta and its branches, especially the subclavian arteries, with clinical manifestation dependent on the involved artery. Sequelae of the active disease vary, including stenosis, occlusions, or aneurysmal dilatations of the large vessels. The prevalence of Takayasu arteritis is higher in the Asian population and in Japan, but quite low in the United States, varying from 0.

A Comparative Study of Large Language Models, Human Experts, and Expert-Edited Large Language Models to Neuro-Ophthalmology Questions.

Background: While large language models (LLMs) are increasingly used in medicine, their effectiveness compared with human experts remains unclear. This study evaluates the quality and empathy of Expert + AI, human experts, and LLM responses in neuro-ophthalmology.

Methods: This randomized, masked, multicenter cross-sectional study was conducted from June to July 2023.

Ocular Neuromyotonia: Clinical Features, Diagnosis, and Outcomes.

Purpose: The purpose of this study was to describe the clinical features, management, outcomes, and diagnostic pitfalls in a large series of patients with ocular neuromyotonia.

Design: Retrospective cohort.

Methods: Patients diagnosed with ocular neuromyotonia from January 1, 2004, through January 1, 2023, seen at one of the 3 Mayo Clinic sites in Rochester, MN, Scottsdale, AZ, and Jacksonville, FL, comprised the study population.

Visual Outcomes Following Plasma Exchange for Optic Neuritis: An International Multicenter Retrospective Analysis of 395 Optic Neuritis Attacks.

Purpose: To evaluate the effectiveness of plasma exchange (PLEX) for optic neuritis (ON).

Methods: We conducted an international multicenter retrospective study evaluating the outcomes of ON following PLEX. Outcomes were compared to raw data from the Optic Neuritis Treatment Trial (ONTT) using a matched subset.

Oculomotor nerve root split: incidental finding on MRI-case report and literature review.

Background: The oculomotor nerve (OMN) innervates the pupil, ciliary body, upper eyelid, and extraocular muscles through two divisions: a superior division that innervates the levator palpebrae superioris (LPS) and superior rectus (SR), and an inferior division that supplies the medial rectus (MR), inferior rectus (IR), inferior oblique (IO), and parasympathetic fibers to the pupil and ciliary body. We present a case of complete splitting of the cisternal segment of bilateral OMNs that was discovered incidentally on magnetic resonance imaging (MRI) in a patient who had no ocular complaints.

Case Report: A 69-year-old patient was found to have bilateral splitting of the cisternal segments of OMNs during an MRI for trigeminal neuralgia workup.

Frequency of Asymptomatic Optic Nerve Enhancement in a Large Retrospective Cohort of Patients With Aquaporin-4+ NMOSD.

Background And Objectives: Asymptomatic or persistent optic nerve enhancement in aquaporin-4 (AQP4)-immunoglobulin G (IgG)-positive neuromyelitis optica spectrum disorder (NMOSD) is thought to be rare. Improved understanding may have important implications for assessment of treatment efficacy in clinical trials and in clinical practice. Our objective was to characterize NMOSD interattack optic nerve enhancement.

Association of Maintenance Intravenous Immunoglobulin With Prevention of Relapse in Adult Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease.

Importance: Recent studies suggest that maintenance intravenous immunoglobulin (IVIG) may be an effective treatment to prevent relapses in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD); however, most of these studies had pediatric cohorts, and few studies have evaluated IVIG in adult patients.

Objective: To determine the association of maintenance IVIG with the prevention of disease relapse in a large adult cohort of patients with MOGAD.

Design, Setting, And Participants: This was a retrospective cohort study conducted from January 1, 2010, to October 31, 2021.

Tough Questions in Total Hip and Total Knee Arthroplasty.

Some of today's tough questions surrounding hip and knee arthroplasty involve modifiable risks, bilateral staging, physiotherapy, postoperative activity, venous thromboembolism and infection prophylaxis, pain management, and outpatient settings. The available literature is reviewed to provide answers to difficult questions facing the orthopaedic surgeon. Preoperative questions focus on patient selection, medical optimization, and appropriateness for outpatient surgery.

OCT retinal nerve fiber layer thickness differentiates acute optic neuritis from MOG antibody-associated disease and Multiple Sclerosis: RNFL thickening in acute optic neuritis from MOGAD vs MS.

Background: Optic neuritis (ON) is the most common manifestation of myelin oligodendrocyte glycoprotein antibody associated disorder (MOGAD) and multiple sclerosis (MS). Acute ON in MOGAD is thought to be associated with more severe optic disk edema than in other demyelinating diseases, but this has not been quantitatively confirmed. The goal of this study was to determine whether optical coherence tomography (OCT) can distinguish acute ON in MOGAD from MS, and establish the sensitivity of OCT as a confirmatory biomarker of ON in these entities.

The Outpatient Approach to Dizziness.

The evaluation of the dizzy patient is complicated by many common pitfalls. The patient's description of symptoms and the standard neurologic examination are often nonspecific or unrevealing, and neuroimaging is most often normal. Over the past several years, research has demonstrated that a refocusing of history taking results in more reliable and diagnostically helpful information.

Lumbar Puncture for Diagnosis of Idiopathic Intracranial Hypertension in Typical Patients.

Background: Patients with typical features of pseudotumor cerebri syndrome (PTCS) must undergo lumbar puncture (LP) to demonstrate elevated opening pressure and cerebrospinal fluid (CSF) analysis to rule out alternative diagnoses. As LP may be associated with significant morbidity, this study aims to determine its necessity in diagnosing typical PTCS.

Methods: Retrospective chart review at 3 university-based neuro-ophthalmology practices included women aged 18-45 years with body mass index >25, papilledema, negative neuroimaging, and who met criteria for PTCS or probable PTCS.

OS reboot.

Cerebrospinal fluid (CSF) diversion or shunting procedures are the most commonly performed surgery for the treatment of hydrocephalus and are often employed in the management of elevated intracranial pressure due to a variety of diseases. Despite their popularity however, approximately 50% of shunts fail within the first two years, and several revisions are required within the first decade after placement. Ophthalmologists may encounter patients with a CSF shunt to evaluate for concerns of vision loss or diplopia and to determine if papilledema is present.

Neuro-Ophthalmological Findings in Early Fatal Familial Insomnia.

Fatal familial insomnia (FFI) is a rare inherited prion disease characterized by sleep, autonomic, and motor disturbances. Neuro-ophthalmological abnormalities have been reported at the onset of disease, although not further characterized. We analyzed video recordings of eye movements of 6 patients with FFI from 3 unrelated kindreds, seen within 6 months from the onset of illness.

Toward allele-specific targeting therapy and pharmacodynamic marker for spinocerebellar ataxia type 3.

Spinocerebellar ataxia type 3 (SCA3), caused by a CAG repeat expansion in the ataxin-3 gene (), is characterized by neuronal polyglutamine (polyQ) ATXN3 protein aggregates. Although there is no cure for SCA3, gene-silencing approaches to reduce toxic polyQ ATXN3 showed promise in preclinical models. However, a major limitation in translating putative treatments for this rare disease to the clinic is the lack of pharmacodynamic markers for use in clinical trials.