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Optic neuritis (ON) is an inflammatory condition involving the optic nerve. Several important typical and atypical ON variants are now recognized. Typical ON has a more favorable prognosis; it can be idiopathic or represent an early manifestation of demyelinating diseases, mostly multiple sclerosis (MS). The atypical spectrum includes entities such as antibody-driven ON associated with neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein antibody disease (MOGAD), chronic/relapsing inflammatory optic neuropathy (CRION), and sarcoidosis-associated ON. Appropriate and timely diagnosis is essential to rapidly decide on the appropriate treatment, maximize visual recovery, and minimize recurrences. This review paper aims at presenting the currently available state-of-the-art treatment strategies for typical and atypical ON, both in the acute phase and in the long-term. Moreover, emerging therapeutic approaches and novel steps in the direction of achieving remyelination are discussed.

Purpose of ReviewImmune checkpoint inhibitor (ICI) use has been on the rise for treatment of many different malignancies. Subsequently, more has been learned about immune-related adverse events (irAEs) that occur up to 12 months after treatment. This review summarizes the latest findings and management of neuro-ophthalmic associated irAEs.Recent FindingsirAEs can affect the afferent and efferent neuro-ophthalmic pathways, thereby targeting central and peripheral nervous systems. As more cases are being reported, it is becoming apparent that neuro-ophthalmic irAEs often present with atypical features when compared to their spontaneous autoimmune counterparts. These neuro-ophthalmic presentations can also be signs of a more extensive inflammatory process that spans other organ systems, such as myopathies, endocrinopathies, and paraneoplastic syndromes.SummaryAwareness of neuro-ophthalmic irAEs and their atypical presentations can lead to early detection, termination of ICI treatment, and immunosuppressant therapy initiation.

We present a case of a man in his 40s with severe plaque psoriasis treated with guselkumab who developed bilateral lower extremity numbness and whose MRI demonstrated an acute contrast-enhancing demyelinating lesion in his cervical spine. The patient’s neurologic symptoms improved significantly following discontinuation of the biologic agent with concurrent treatment of intravenous methylprednisolone. Naranjo score of 6, indicating probable adverse drug reaction, further supports this finding. This case marks the first reported instance of transverse myelitis in a patient receiving guselkumab. Greater awareness of this potential adverse event is suggested prior to starting this treatment, particularly in patients with evidence of demyelinating lesions in their central nervous system. Additional studies are needed to evaluate the safety of guselkumab in these patients. We provide a review of psoriasis treatments and highlight those that have been shown to exacerbate neuroinflammatory conditions, like multiple sclerosis, which can lead to attacks of demyelination.

A 64-year-old woman was admitted with a 6-week history of cognitive impairment, headache, and memory loss. She had hyperreflexia and impaired attention and memory. MRI showed diffuse subarachnoid hyperintensities and leptomeningeal enhancement on FLAIR sequences. A diagnostic test was performed.

A 60-year-old woman presented with altered mental status and weakness on the left side during the Covid-19 pandemic. Diffusion-weighted MRI of the head revealed a punctate focus of restricted diffusion within the posterior limb of the right internal capsule, with no corresponding abnormality on fluid-attenuated inversion recovery images. Treatment decisions were made.

A Woman with Falls and Cognitive DeclineAn 82-year-old woman was admitted because of difficulty walking, falls, and cognitive decline. Light touch of the right hand, right knee, and both feet caused pain. A diagnosis was made.

A Woman with Abnormal Movements and ConfusionA 68-year-old woman was admitted because of confusion and abnormal movements of the face, arms, and legs. MRI of the head showed multiple strokes. A diagnosis was made.

Expanding the US Food and Drug Administration–approved indications for immune checkpoint inhibitors in patients with cancer has resulted in therapeutic success and immune-related adverse events (irAEs). Neurologic irAEs (irAE-Ns) have an incidence of 1%–12% and a high fatality rate relative to other irAEs. Lack of standardized disease definitions and accurate phenotyping leads to syndrome misclassification and impedes development of evidence-based treatments and translational research. The objective of this study was to develop consensus guidance for an approach to irAE-Ns including disease definitions and severity grading. A working group of four neurologists drafted irAE-N consensus guidance and definitions, which were reviewed by the multidisciplinary Neuro irAE Disease Definition Panel including oncologists and irAE experts. A modified Delphi consensus process was used, with two rounds of anonymous ratings by panelists and two meetings to discuss areas of controversy. Panelists rated content for usability, appropriateness and accuracy on 9-point scales in electronic surveys and provided free text comments. Aggregated survey responses were incorporated into revised definitions. Consensus was based on numeric ratings using the RAND/University of California Los Angeles (UCLA) Appropriateness Method with prespecified definitions. 27 panelists from 15 academic medical centers voted on a total of 53 rating scales (6 general guidance, 24 central and 18 peripheral nervous system disease definition components, 3 severity criteria and 2 clinical trial adjudication statements); of these, 77% (41/53) received first round consensus. After revisions, all items received second round consensus. Consensus definitions were achieved for seven core disorders: irMeningitis, irEncephalitis, irDemyelinating disease, irVasculitis, irNeuropathy, irNeuromuscular junction disorders and irMyopathy. For each disorder, six descriptors of diagnostic components are used: disease subtype, diagnostic certainty, severity, autoantibody association, exacerbation of pre-existing disease or de novo presentation, and presence or absence of concurrent irAE(s). These disease definitions standardize irAE-N classification. Diagnostic certainty is not always directly linked to certainty to treat as an irAE-N (ie, one might treat events in the probable or possible category). Given consensus on accuracy and usability from a representative panel group, we anticipate that the definitions will be used broadly across clinical and research settings.

Purpose of ReviewIn recent years, literature on neuroinflammatory disorders has dramatically expanded, as have options for treatment. However, few reviews have focused on skull-based manifestations of inflammatory disorders.Recent FindingsHere, we review the clinical manifestations, etiologies, diagnostic workup, and treatment of both systemic and localized inflammatory diseases of the skull base with a focus on recent updates to the literature.SummaryThis review aims to guide the workup and management of this complex set of diseases.