ABSTRACT Background Paraneoplastic and autoimmune retinopathies can cause progressive visual loss before structural ophthalmic or neurologic findings are diagnostic. Case Presentation A 66‐year‐old woman developed bilateral visual decline and was diagnosed with small‐cell lung cancer‐associated paraneoplastic neuroretinal dysfunction, with persistent blindness despite radiotherapy and plasma exchange. An 81‐year‐old woman with negative malignancy evaluation and abnormal multifocal electroretinography reported visual improvement after intravitreal dexamethasone. Conclusions These cases emphasize that nyctalopia, ring scotomas, and photopsias in the context of abnormal electroretinography should prompt retinal evaluation even when structural findings are limited, and that MOG‐ and AQP4‐associated optic neuritis must be explicitly excluded. Early malignancy assessment, immunologic testing, and individualized immunomodulatory treatment are essential; visual recovery is limited once irreversible retinal injury is established.
ABSTRACT Objectives To characterize real‐world barriers to chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) management in the Philippines and identify pragmatic patient‐centered solutions aligned with international guidance and local health‐system realities. Methods We conducted a survey‐informed qualitative expert‐panel study involving six Philippine neurologists from public and private tertiary centers. Data sources included review of EAN/PNS‐2021 CIDP guidelines, contemporary literature, and pre‐meeting survey results on local clinical practice followed by structured discussions. Findings were organized using a patient‐journey framework and analyzed thematically across clinical, operational, policy, and economic domains. Results Real‐world challenges were identified across three domains. Clinical: clinical mimics, variability in electrodiagnostic interpretation, limited test availability, and delayed referral; Operational: partial or variable treatment response, corticosteroid side‐effects, inpatient infusion logistics, and difficulties maintaining therapy; and Policy/economic: high out‐of‐pocket costs, limited reimbursement, procurement delays, and regional inequities. Experts described several key components of patient‐centered CIDP care, including the use of standardized diagnostic pathways, improved access to neurophysiological testing, increased disease awareness, and structured monitoring using disability and impairment scales. Additional elements included the incorporation of patient‐reported outcomes, shared decision‐making, and individualized infusion and weaning strategies, and generation of local real‐world, cost‐effectiveness, budget‐impact, and patient‐reported outcome data. Conclusions This study identifies neurologist‐perceived clinical and system barriers affecting CIDP care across the patient journey in the Philippines. Findings support implementation of an evidence‐based patient‐centered framework aligned with international guidelines but adapted for the Philippine healthcare system. Such a framework may improve diagnosis, streamline treatment, and enhance equitable access.
ABSTRACT Background Molecular targeted therapies significantly reduce relapse rates in patients with aquaporin‐4 antibody‐positive neuromyelitis optica spectrum disorder (AQP4‐NMOSD). In the Phase 3 CHAMPION‐NMOSD trial of ravulizumab, a C5 inhibitor, no patients experienced a relapse. Case Presentation We report a patient who developed slowly progressive optic neuritis during ravulizumab therapy. A 61‐year‐old Japanese woman first developed left optic neuritis at age 59 and was diagnosed with AQP4‐NMOSD. After acute‐phase treatment, ravulizumab was initiated and prednisolone (PSL) was tapered. Twelve months after onset, she reported minimal blurring in a relatively spared visual field, though no significant changes were detected. Six months later, the blurring worsened, accompanied by declines in visual acuity and critical flicker frequency (CFF). Magnetic resonance imaging (MRI) revealed subtle gadolinium enhancement at the primary lesion, and a relapse was diagnosed. Owing to dependence on high‐dose PSL for relapse prevention, treatment was switched from ravulizumab to inebilizumab, a CD19 inhibitor. Follow‐up MRI subsequently showed diminished gadolinium enhancement, along with improved visual acuity and CFF. This chronic, progressive optic neuritis was diagnosed as an NMOSD relapse during ravulizumab therapy. A breakthrough relapse under C5 inhibition may present with atypical, insidious progression, underscoring the need for careful monitoring. In this patient, CD19‐directed therapy may have suppressed inflammation more effectively, suggesting that pathogenic immune mechanisms may vary among patients. Conclusions This is a rare case of AQP4‐NMOSD presenting with slowly progressive optic neuritis during ravulizumab therapy. Observing patients treated with novel molecular‐targeted drugs may reveal new clinical presentations and underlying pathologies.
ABSTRACT Objectives Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune inflammatory disease characterized by pathogenic anti‐aquaporin‐4 (AQP4) antibodies. Previous studies have shown increased circulating T follicular helper (cTfh) cells and altered Tfh subset frequencies in NMOSD, but the absolute counts of Tfh subsets remain unclear. We evaluated relative frequencies and absolute counts of cTfh subsets in acute‐phase AQP4‐IgG–positive NMOSD. Methods This retrospective single‐center study included 12 patients with AQP4‐IgG–positive NMOSD and seven disease controls. Flow cytometry assessed the frequency of cTfh cells among CD4 + T cells, the relative frequencies of Tfh1, Tfh2, and Tfh17 subsets, and their absolute counts. Acute‐phase NMOSD was compared with chronic‐phase NMOSD and disease controls. Results The frequency of cTfh cells among CD4 + T cells was significantly higher in acute‐phase NMOSD than in chronic‐phase NMOSD (15.7% vs. 12.7%, p = 0.026) and disease controls (15.7% vs. 12.2%, p = 0.012). Neither the relative frequencies of Tfh1, Tfh2, and Tfh17 subsets nor the (Tfh2 + Tfh17)/Tfh1 ratio differed significantly between groups. However, the absolute Tfh1 count was significantly higher in acute‐phase NMOSD than in chronic‐phase NMOSD (72.5 vs. 30.7 cells/μL, p = 0.034), whereas the absolute Tfh2 count was significantly higher than that in disease controls (50.5 vs. 32.4 cells/μL, p = 0.038). Conclusions Acute‐phase AQP4‐IgG–positive NMOSD is characterized by a higher absolute Tfh1 count than chronic‐phase NMOSD and a higher absolute Tfh2 count than disease controls, together with an increased cTfh frequency, despite unchanged relative Tfh‐subset frequencies and an unchanged (Tfh2 + Tfh17)/Tfh1 ratio. Analysis of absolute cTfh‐subset counts may complement conventional assessments based on relative Tfh‐subset frequencies and the (Tfh2 + Tfh17)/Tfh1 ratio, providing additional insight into the immunopathogenesis of NMOSD.
