MRI Indices of Glymphatic Function Correlate With Disease Duration in Idiopathic Intracranial Hypertension.

Background: The glymphatic system represents an extravascular network of astrocytic channels responsible for interstitial fluid and solute transit through the brain parenchyma. Its dysfunction has been considered as a possible cause of idiopathic intracranial hypertension (IIH).

Methods: We enrolled participants with active IIH, treated or cured IIH, and controls.

Risk of Nonarteritic Anterior Ischemic Optic Neuropathy in Patients Prescribed Semaglutide.

Importance: Anecdotal experience raised the possibility that semaglutide, a glucagon-like peptide 1 receptor agonist (GLP-1 RA) with rapidly increasing use, is associated with nonarteritic anterior ischemic optic neuropathy (NAION).

Objective: To investigate whether there is an association between semaglutide and risk of NAION.

Design, Setting, And Participants: In a retrospective matched cohort study using data from a centralized data registry of patients evaluated by neuro-ophthalmologists at 1 academic institution from December 1, 2017, through November 30, 2023, a search for International Statistical Classification of Diseases and Related Health Problems, Tenth Revision code H47.

Novel uses of complement inhibitors in myasthenia gravis-Two case reports.

Introduction/aims: Myasthenia gravis (MG) is a rare, life-threatening immune-related adverse effect (irAE) of immune checkpoint inhibitor (ICI) treatment. C5-complement inhibitors are effective treatments for acetylcholine receptor antibody (AChR ab) positive generalized MG. We describe the use of eculizumab/ravulizumab in two patients with MG receiving concomitant pembrolizumab.

SARS-CoV-2 Parainfectious Optic Neuropathy: 3 Case Reports and a Review of the Literature.

Background: Parainfectious optic neuritis is an inflammatory reaction that occurs shortly after an infection without direct invasion by a pathogen. The clinical profile depends on the infectious organism. Cases of SARS-CoV-2 parainfectious optic neuritis have been reported in the literature, but there are no reviews that have applied strict inclusion criteria to more definitively establish the clinical profile associated with SARS-CoV-2.

Evidence-based management of optic neuritis.

Purpose Of Review: Optic neuritis can result from several distinct causes, including multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), and myelin oligodendrocyte glycoprotein antibody disease (MOGAD), when not idiopathic. This review discusses evidence-based treatment approaches contingent upon each specific cause of optic neuritis.

Recent Findings: Current evidence highlights the need for prompt plasmapheresis as adjunct to intravenous methylprednisolone (IVMP) in patients with NMOSD-associated optic neuritis.

Clinical Reasoning: A Woman With Progressive Painless Sequential Monocular Vision Loss.

A 68-year-old woman with a history of diabetes mellitus type 2, depression, and migraines presented with painless, acute, consecutive vision loss affecting the right eye for 1 week and the left eye for 2 weeks. Neuro-ophthalmic examination was notable for visual acuities of finger-counting peripherally, a central scotoma, anterior uveitis, vitritis, and placoid macular pigmentary changes in each eye (OU). Proprioception was diminished in the bilateral lower extremities.

Fluorescein Angiography Findings in Susac Syndrome: A Multicenter Retrospective Case Series.

Background: Susac syndrome is a vasculopathy, resulting in the classic triad of branch retinal artery occlusion (BRAO), inner ear ischemia, and brain ischemia. In this retrospective chart review, we characterize fluorescein angiography (FA) findings and other ancillary studies in Susac syndrome, including the appearance of persistent disease activity and the occurrence of new subclinical disease on FA.

Methods: This multicenter, retrospective case series was institutional review board-approved and included patients with the complete triad of Susac syndrome evaluated with FA, contrasted MRI of the brain, and audiometry from 2010 to 2020.

Giant Cell Arteritis Presenting With Multiple Cranial Neuropathies - Case Report.

Vision loss accounts for most ophthalmic presentations of giant cell arteritis (GCA), but an important minority of patients present with diplopia and other cranial neuropathies. Here we present the case of an 84-year-old woman with a prior history of multiple cancers who was admitted to our hospital after developing double vision. She was found to have mydriasis, ptosis, and ophthalmoplegia in the right eye (OD) consistent with a combined R CNIII/CNVI neuropathy, as well as highly elevated inflammatory markers.

MRI findings differentiating tonsillar herniation caused by idiopathic intracranial hypertension from Chiari I malformation.

Purpose: Some patients with idiopathic intracranial hypertension (IIH) have cerebellar tonsillar herniation ≥ 5 mm mimicking Chiari malformation I (CMI), which can result in misdiagnosis and unjustified treatment. Our purpose was to identify IIH patients with tonsillar herniation ≥ 5 mm (IIH) and compare with CMI patients to assess imaging findings that could distinguish the two conditions.

Methods: Ninety-eight patients with IIH, 81 patients with CMI, and 99 controls were retrospectively assessed.

Clinical Reasoning: A 48-Year-Old Woman With 6 Months of Vivid Visual Hallucinations.

Visual hallucinations are frequently encountered in clinical practice. It is critical for neurologists, particularly those in training, to develop a systematic approach for evaluating patients presenting with such unique and often perplexing symptoms. In this study, we present the case of a 48-year-old woman who developed vivid visual hallucinations after receiving nivolumab for melanoma.

Does Tocilizumab Influence Ophthalmic Outcomes in Giant Cell Arteritis?

Background: Despite appropriate use of corticosteroids, an important minority of patients with giant cell arteritis (GCA) develop progressive vision loss during the initial stages of the disease or during corticosteroid tapering. Tocilizumab is the only clearly effective adjunctive treatment to corticosteroids in the management of GCA, but questions regarding its efficacy specifically in the neuro-ophthalmic population and its role in mitigating vision loss have not been broached until recently.

Evidence Acquisition: The authors queried Pubmed using the search terms "GCA" and "tocilizumab" in order to identify English-language publications either explicitly designed to evaluate the influence of tocilizumab on the ophthalmic manifestations of GCA or those which reported, but were not primarily focused on, ophthalmic outcomes.

Clinical Correlation Between Vertical Gaze Palsy and Midbrain Volume in Progressive Supranuclear Palsy.

Background: Supranuclear vertical gaze palsies and slowed vertical saccades are characteristic clinic features of progressive supranuclear palsy (PSP). The "hummingbird sign," reflective of midbrain atrophy, is a classic radiographic sign of PSP. Correlation between eye movement abnormalities and radiographic findings in PSP has been reported previously.

Variation in Evolving Optic Neuritis.

Background: The typical natural history of optic neuritis is subjected to important exceptions. Recognition of these exceptions has led to valuable insights regarding specific etiologies of optic neuritis. Exceptions to the natural history of recovering optic neuritis are well-defined (e.

Fulminant Idiopathic Intracranial Hypertension.

Purpose Of Review: The goal of this review is to describe the presenting features of fulminant idiopathic intracranial hypertension (IIH) and outline the multimodal approach to its treatment.

Recent Findings: Venous sinus stenting may be an appropriate alternative to optic nerve sheath fenestration or cerebrospinal fluid shunting in select patients with fulminant IIH. Prompt surgical intervention maximizes the chance of visual recovery in patients with fulminant IIH.

The Pupil.

Purpose Of Review: The goal of this article is to review the anatomy and physiology of pupillary function and then employ that information to develop a comprehensive framework for understanding and diagnosing pupillary disorders.

Recent Findings: The contribution of rods and cones to the pupillary light reflex has long been known. A third photosensitive cell type, the intrinsically photosensitive retinal ganglion cell, has recently been discovered.

The Comprehensive Management of Cerebellar Ataxia in Adults.

Purpose Of Review: In this review, we present the multidisciplinary approach to the management of the many neurological, medical, social, and emotional issues facing patients with cerebellar ataxia.

Recent Findings: Our holistic approach to treatment, developed over the past 25 years in the Massachusetts General Hospital Ataxia Unit, is centered on the compassionate care of the patient and their family, empowering them through engagement, and including the families as partners in the healing process. We present the management of ataxia in adults, beginning with establishing an accurate diagnosis, followed by treatment of the multiple symptoms seen in cerebellar disorders, with a view to maximizing quality of life and effectively living with the consequences of ataxia.

Neuroimaging diagnostic and monitoring approaches in ophthalmology.

Purpose Of Review: We review new applications of optical coherence tomography (OCT) technology in neuro-ophthalmology. We also describe new technologies for visualizing the extracranial vessels in the diagnosis of giant cell arteritis (GCA).

Recent Findings: Newer OCT modalities are expanding the evaluation of the optic disc, and are being applied to a number of neurologic conditions such as demyelinating and neurodegenerative disease.