BACKGROUND: To investigate the clinical features and serologic test results of optic neuritis through a multi-institutional study in Korea. METHODS: The is a retrospective cohort study from multicenters (21 centers) in K...BACKGROUND: To investigate the clinical features and serologic test results of optic neuritis through a multi-institutional study in Korea. METHODS: The is a retrospective cohort study from multicenters (21 centers) in Korea. A cohort consists of 181 Korean patients diagnosed with noninfectious optic neuritis from January 2022 to December 2023. The types of optic neuritis were classified and analyzed based on serologic and imaging tests. The positive rate of serologic tests was analyzed. In addition, demographics, visual function tests including visual acuity and visual field, and anatomical investigation using optical coherence tomography and fundus photography were compared and analyzed. RESULTS: Of the cohort, 55.2% were female, and 18.2% had bilateral involvement. At the onset, 67.4% of patients presented with ocular pain, and 98.3% of patients had visual impairment. In addition, 27.1% of patients experienced neurologic symptoms such as headaches and dizziness. Among 171 tested patients, 8.2% were positive for anti-aquaporin 4 antibody, 32.2% were positive for anti-myelin oligodendrocyte glycoprotein (MOG) antibody, and 56.7% were negative. The average visual acuity was 1.08 logMAR, with 65.7% of patients showing optic disc swelling. MRI revealed that 90.1% of patients had optic nerve contrast enhancement. The neuromyelitis optica group had a higher average age and more female patients, while the myelin oligodendrocyte glycoprotein-associated disease group had a higher rate of bilateral optic neuritis. CONCLUSIONS: In Korea, idiopathic optic neuritis was the most common, followed by myelin oligodendrocyte glycoprotein-related optic neuritis. Accordingly, clinical manifestations showed different tendencies depending on the causative disease. Therefore, understanding racial characteristics and appropriate planning during the diagnosis and treatment of optic neuritis are necessary.
BACKGROUND: Wolfram syndrome type 1 (WS1) is a rare autosomal recessive disorder classically associated with diabetes mellitus (DM) and optic atrophy (OA). We aimed to characterize OA in WS1 and evaluate optical coherenc...BACKGROUND: Wolfram syndrome type 1 (WS1) is a rare autosomal recessive disorder classically associated with diabetes mellitus (DM) and optic atrophy (OA). We aimed to characterize OA in WS1 and evaluate optical coherence tomography (OCT) and genetic biomarkers as tools for disease monitoring and prognostication. METHODS: We conducted a retrospective chart review of genetically confirmed patients with WS1 seen at Washington University or Indiana University neuro-ophthalmology clinics between July 2017 and 2024. Data included demographics, clinical history, best corrected visual acuity (BCVA), OCT retinal nerve fiber layer (RNFL) and ganglion cell complex (GCC) thickness, and WFS1 mutation severity scores. Linear regression analyses assessed correlations between BCVA and clinical, structural, and genetic variables. RESULTS: Thirty-six patients (22 women, 14 men; median age 20 years) were identified. Median mutation severity score was 3.5. Vision loss occurred in 31 patients; in 3 patients it was the only major symptom, in 5 patients it preceded DM, and in 6 patients it occurred without DM. Mean and median BCVA were 20/125 and 20/80, respectively. BCVA correlated inversely with RNFL thickness (P = 0.0017, R2 = 0.14), GCC thickness (P = 0.0018, R2 = 0.29), and mutation severity score (P = 0.031, R2 = 0.14). CONCLUSIONS: OA was the most common and sometimes earliest WS1 manifestation. Correlations between BCVA, OCT metrics, and mutation severity score support their potential value as biomarkers and prognostic tools. Findings also support considering genetic screening for WFS1 mutations in patients presenting with otherwise unexplained OA.
In this issue, Drs. Mark L. Moster and Deborah I. Friedman review the following 5 articles, 3 of which were co-authored by our NANOS colleagues. Lee L, Vemuri JP, Belilos A, Sismanis A, Haines S, Felton W, Gharavi M, Tan...In this issue, Drs. Mark L. Moster and Deborah I. Friedman review the following 5 articles, 3 of which were co-authored by our NANOS colleagues. Lee L, Vemuri JP, Belilos A, Sismanis A, Haines S, Felton W, Gharavi M, Tang Y, Coelho DH. Number of radiologic abnormalities associated with idiopathic intracranial hypertension as a predictor of the presence of pulsatile tinnitus. Otol Neurotol Open. 2025;5:e072. doi: 10.1097/ONO.0000000000000072.Snowden A, Van Stavern GP, Leanne Stunkel L Persistence of pulsatile tinnitus in patients with idiopathic intracranial hypertension following resolution of papilledema. J Neurol Sci. 2025:476:123608. doi: 10.1016/j.jns.2025.123608.Nachira D, Congedo MT, Kuzmych K, Evoli A, Iorio R, Vita ML, Petracca-Ciavarella L, Nocera A, Sassorossi C, Evangelista J, Lyberis P, Comacchio GM, Brandolini J, Aprile V, Zifara CC, Mastromarino MG, Patirelis A, Asteggiano E, Anile M, Venuta F, Imperatori A, Ambrogi V, Solli P, Dell'Amore A, Lucchi M, Melfi F, Ibrahim M, Ruffini E, Rea F, Margaritora S, Meacci E. Thymectomy in ocular myasthenia gravis: results before and after generalization and prognostic predictors of outcomes. J Clin Med. 2025;14:7840. doi: 10.3390/jcm14217840.Clément G, Puisieux S, Pellerin D, Brais B, Bonnet C, Renaud M. Spinocerebellar ataxia 27B (SCA27B), a frequent late-onset cerebellar ataxia. Rev Neurol (Paris). 2024;80:410-416. doi: 10.1016/j.neurol.2024.03.007.Thakolwiboon S, Redenbaugh V, Chen B, Hewitt S, Shah S, Lotan I, Levy M, Forcadela M, Huda S, Pique J, Marignier R, Boutiere C, Audoin B, Poullin P, Champsas D, Choi D, Danesh-Meyer HV, Vasileiou E, Sotirchos ES, Davis JB, Henderson AD, Wilf-Yarkoni A, Stiebel-Kalish H, Maillart E, Bonelli L, Arnold AC, Boudot De La Motte M, Deschamps R, Jitprapaikulsan J, Moss HE, Villarreal Navarro SE, Mao-Draayer Y, Mishra M, Vorasoot N, Cacciaguerra L, Tisavipat N, Tajfirouz DA, Tillema JM, Lopez-Chiriboga SA, Palace J, Hacohen Y, Pittock SJ, Flanagan EP, Chen JJ. Outcomes after acute plasma exchange for myelin oligodendrocyte glycoprotein antibody-associated disease. Neurology. 2025;105:e213903. doi: 10.1212/WNL.0000000000213903.
A 61-year-old woman presented with episodic dizziness that met diagnostic criteria for vestibular migraine. Vestibular and ocular motor examination was normal, aside from a 1°/second downbeat nystagmus (DBN) that was onl...A 61-year-old woman presented with episodic dizziness that met diagnostic criteria for vestibular migraine. Vestibular and ocular motor examination was normal, aside from a 1°/second downbeat nystagmus (DBN) that was only seen with removal of fixation (in the dark) using video oculography. Two years later, lithium was initiated for treatment of depression and visual bouncing and jumping developed even when her head was still. On repeat examination, she had clear spontaneous (20°/second) DBN even with fixation in room light. Lithium therapy was discontinued, and for days and weeks, there was subjective improvement in oscillopsia and objective lessening of her DBN. However, DBN remained significant and symptomatic after several months. A broad search for reversible nutritional, metabolic, and immune-mediated causes of DBN and cerebellar dysfunction was completed and was unremarkable. Therapy with 4-aminopyridine was initiated and she had a robust improvement in oscillopsia and DBN. During the following years, she developed mild gait ataxia, and genetic testing revealed GAA repeat expansions in 1 copy of the fibroblast growth factor 14 (FGF14) gene, consistent with a diagnosis of spinocerebellar ataxia type 27B (SCA27B).
BACKGROUND: Noninvasive ophthalmic imaging and quantification of the optic nerve and retina using near-infrared optical coherence tomography (OCT) have become an integral part of neuro-ophthalmic practice. Advances in OC...BACKGROUND: Noninvasive ophthalmic imaging and quantification of the optic nerve and retina using near-infrared optical coherence tomography (OCT) have become an integral part of neuro-ophthalmic practice. Advances in OCT have allowed 3D mapping of the optic nerve and retinal microvascular using OCT angiography (OCTA) at micrometer precision. In this review, we discuss the utility of OCTA in neuro-ophthalmic and neurologic conditions with particular focus on current applications, needed progress, and integration into neuro-ophthalmic practice. EVIDENCE ACQUISITION: Evidence was acquired from PubMed searches. RESULTS: OCTA ocular imaging allows for qualitative and quantitative assessments of the microvasculature in the papillary, peripapillary, and macular regions in select retinal and subretinal layers. OCTA has been used in a number of neuro-ophthalmic disease contexts. Most OCTA studies highlight attenuation of vascular density in pathologic conditions with some overlapping patterns of microvascular changes. OCTA has also been applied in neurologic conditions presenting with a normal-appearing disc. There is a significant degree of overlap in the OCTA findings in these conditions, and the number of comparative studies are limited. CONCLUSIONS: OCTA has high potential to serve diagnostic and prognostic roles in neuro-ophthalmology. Future work that focuses on longitudinal evaluation of specific OCTA biomarkers includes comparative conditions with clinical overlap and uses functional correlative analyses that will promote the utility of OCTA in neuro-ophthalmic practice.
BACKGROUND: Central retinal artery occlusion (CRAO) is a vision-threatening neuro-ophthalmic emergency, analogous to acute ischemic stroke. Delayed presentation, limited therapeutic options, and lack of randomized prospe...BACKGROUND: Central retinal artery occlusion (CRAO) is a vision-threatening neuro-ophthalmic emergency, analogous to acute ischemic stroke. Delayed presentation, limited therapeutic options, and lack of randomized prospective trials continue to restrict effective treatment. EVIDENCE ACQUISITION: This review summarizes current evidence on the emergent management and evolving treatment strategies for CRAO, based on recent observational studies, meta-analyses, and early randomized trials. RESULTS: Conservative interventions, such as ocular massage, paracentesis, and vasodilator use, have shown no benefit and may be harmful. Limited data suggest that intravenous thrombolysis with recombinant t-PA (alteplase or tenecteplase [TNK]) within 4.5 hours of symptoms onset offers the strongest evidence for visual recovery, with a safety profile comparable to that of cerebral stroke treatment. TNK offers pharmacologic advantages but has not demonstrated clinical superiority over alteplase. Intra-arterial thrombolysis may extend the treatment window, although findings remain inconsistent. "Eye stroke" protocols incorporating teleophthalmology, nonmydriatic fundus photography, and artificial intelligence improved diagnostic efficiency. Hyperbaric oxygen therapy may serve as an adjunctive treatment when initiated early. CONCLUSIONS: CRAO should be managed as an acute ischemic stroke of the eye. Thrombolysis within 4.5 hours offers the highest potential for visual recovery, but further prospective, randomized studies are needed to determine efficacy, safety, and optimal management strategies. Improving public awareness, implementing streamlined multidisciplinary protocols, and expanding access to specialized care are essential to enabling timely intervention.
BACKGROUND: High rates of mental health conditions have been reported among patients with idiopathic intracranial hypertension (IIH). Cognitive-behavioral therapy (CBT) has been suggested as a complementary treatment to...BACKGROUND: High rates of mental health conditions have been reported among patients with idiopathic intracranial hypertension (IIH). Cognitive-behavioral therapy (CBT) has been suggested as a complementary treatment to help manage anxiety and headache pain in IIH. This study aims to assess the mental health of IIH patients through a psychiatric interview and to evaluate their suitability and interest in CBT. METHODS: Participants with IIH were recruited from Stanford Byers Eye Institute. Demographic and clinical characteristics were collected, and participants completed self-rated scales for depression (PHQ-9), anxiety (GAD-7), headaches (HIT-6), and disability (WHODAS-12). Diagnostic and Statistical Manual of Mental Disorders, 5th edition diagnoses, patient's suitability for CBT (SSCT scale), and preferred therapy focus were identified through a formal psychiatric interview. RESULTS: Fifty-three participants were enrolled and completed surveys, and 43 participants went on to have the psychiatric interview (mean age 38 years, 91% female). Among them, 76.7% had a GAD-7 score ≥5, indicating at least mild anxiety, and 76.7% of participants had a PHQ-9 score ≥5, indicating at least mild depression. In total, 81.4% of participants suffered from a mood, anxiety, or trauma- and stressor-related disorder. Participants who had received a venous stent or VP shunt had higher anxiety levels (average GAD-7 11.75 vs 6.52; P = 0.007); 81.4% were interested in CBT, and 83.7% were deemed good candidates for CBT (SSCT ≥ 30). Anxiety management most often emerged as their favored therapeutic focus. CONCLUSIONS: Our results confirm the high prevalence of mental health conditions and symptoms among patients with IIH. A majority of patients are interested in CBT, and many would likely benefit from this approach. CBT should therefore be considered in the therapeutic management of IIH. Further research is warranted to validate the efficacy of this intervention in this specific clinical population.
BACKGROUND: Ocular motor control (OMC) and cognitive dysfunction are common persistent sequelae in persons with mild traumatic brain injury (mTBI). Combat and training operations frequently expose military Service Member...BACKGROUND: Ocular motor control (OMC) and cognitive dysfunction are common persistent sequelae in persons with mild traumatic brain injury (mTBI). Combat and training operations frequently expose military Service Members to biomechanical and blast events that render them susceptible to mTBI, and problems such as OMC disturbances and cognitive dysfunction are frequent long after injury. However, these problems can be difficult to detect, often only becoming clinically evident with physical or psychological stress. Knowledge of the relationship between OMC and cognitive dysfunction in chronic mTBI, and of clinical tools to assess this issue, is limited. METHODS: Setting: Academic laboratory; Marcus Institute for Brain Health, University of Colorado; Design: 2-arm, examiner-blinded cross-sectional observational study. Participants: Military Veterans with chronic mTBI (experimental; n = 38) whose most recent mTBI was more than 3 months before enrolment, and Veterans without a history of TBI (control; n = 40); Measures: The computerized King-Devick (K-D) test assessed rapid number naming tasks; the Right Eye computerized eye tracker system measured antisaccade tasks; the Conners' Continuous Performance Test (CPT) tested aspects of selective and sustained attention and impulsivity; the FAS test measured the ability to name as many common nouns that start with "F," "A," and "S" as a method to assess phonemic verbal fluency, attention, and initiation; and the Posttraumatic Stress Disorder (PTSD) Checklist for DSM-5 (PCL-5) was used as a self-report of posttraumatic stress-related symptoms. RESULTS: Veterans in the experimental group had a median of 2 mTBIs, and these occurred approximately 11 years before the study. On the K-D Test, the experimental group had significantly more errors and took significantly more time (51.32 seconds) compared with the control group (43.00 seconds). Significantly greater antisaccade latencies were found in the experimental group for target only, on target distractor, and ipsilateral proximal distractor paradigms, and antisaccade error rates were significantly greater in the experimental group for the contralateral proximal distractor paradigm. Significantly greater PCL-5, and worse FAS test scores and CPT commissions and omissions scores were found in the experimental group. For the experimental group, time since most recent TBI correlated with antisaccade on target distractor error rates. Regression modeling showed that FAS test scores were a significant determinant of K-D test performance. Separate regression modeling for each of the antisaccade task paradigms indicated that group status was significantly associated with antisaccade latency scores for the ipsilateral proximal distractor paradigm. PCL-5 was a significant factor for the on target distractor paradigm, and age and cognitive function denoted by FAS test and CPT scores were significant factors contributing to error rates in multiple specified antisaccade paradigm task performances. CONCLUSIONS: Results support the conclusion that OMC and cognitive performance are persistent co-occurring problems in Veterans with chronic mTBI. Notably, these deficits can be detected even after as few as 2 mTBIs that occurred 11 years earlier, indicating that an OMC-cognition axis of sequelae may exist in the chronic stage of mTBI. The results also identify cognitive correlates of the OMC task paradigms, aiding in the clinical application and interpretation of these tests in chronic mTBI.
BACKGROUND: Preliminary work suggests that glymphatic transit is abnormal and dynamic in idiopathic intracranial hypertension (IIH), although its incomplete characterization across the disease course has led to debate as...BACKGROUND: Preliminary work suggests that glymphatic transit is abnormal and dynamic in idiopathic intracranial hypertension (IIH), although its incomplete characterization across the disease course has led to debate as to its pathophysiologic relevance. We seek to clarify whether glymphatic transit varies across IIH's disease course, correlates with intracranial pressure, and might be measured radiographically to aid diagnosis. METHODS: Diffusion tensor imaging along the perivascular space (ALPS) was used to measure perivascular diffusivity and generate indices of glymphatic transit (ALPS-indices). We studied healthy controls and participants with IIH cross-sectionally, the latter stratified into untreated, treated, or cured IIH. Participants with untreated IIH were substratified as "acute" if imaged <6 months from onset or "chronic" if imaged ≥6 months from onset. RESULTS: Forty participants were studied. We identified a positive, nonlinear correlation between ALPS-indices and disease duration in patients with untreated IIH. ALPS-indices differed between participants with chronic, treated, and acute IIH, in descending order. Healthy controls exhibited lower ALPS-indices than participants with chronic IIH and higher ALPS-indices than participants with acute IIH. ALPS-indices correlated positively with lumbar puncture opening pressures in participants with chronic IIH. Receiver-operating-characteristic curves demonstrated high areas-under-the curve in distinguishing between participants with untreated IIH and controls. CONCLUSIONS: These data support the hypothesis that changes in glymphatic transit are likely a result rather than a cause of IIH, that radiographic indices of glymphatic transit may be leveraged diagnostically, and that ALPS-indices measuring perivascular diffusivity are likely a physiologically valid reflection of glymphatic transit in humans.