Journal of Neuro-Ophthalmology最新文献

Background: Fulminant idiopathic intracranial hypertension (IIH) is a rare presentation of IIH characterized by rapid visual loss. Little is known regarding the cause of fulminant IIH and how to treat this condition. We conducted an analytical study aimed at characterizing disease, treatment, and visual outcomes while evaluating the time and frequency of recurrence of papilledema.

Methods: This retrospective cohort study used records at a single tertiary institution screened between June 1, 2012, and September 31, 2023, for having an international classification of diseases (ICD) code of IIH or papilledema. After meeting the revised Dandy diagnostic criteria and excluding secondary causes of IIH using clinical review, fulminant IIH was determined by rapid visual loss occurring within 1 month of symptom onset. Demographics, treatment, and visual outcomes data were collected with final visual outcomes reported between 3 months and 1 year after diagnosis. Surgical intervention and relapse of papilledema were noted. All analyses were performed in R Studio and Excel, with a P value <0.05 being significant.

Results: Of the 731 IIH patients who were evaluated during this interval, 3.2% had fulminant IIH (n = 24). Patients had a mean age of 27.6 years and presented with an average visual field mean deviation of -19.55 dB. Overall, 95.8% were overweight/obese, 87.5% female, and 50% were Black. In total, 62.5% of patients with fulminant IIH received optic nerve sheath fenestrations. At follow-up, 41.7% of patients were legally blind (with 20/400 vision or worse), including 13% having no light perception. Within our cohort, 42% had a significant visual deficit (visual field mean deviation worse than -7 dB or best-corrected visual acuity between 20/80 and 20/200). Although not statistically significant, patients who underwent cerebral venous sinus stenting had the smallest visual deficit. On average, patients recovered from their initial papilledema after 7.90 months. One quarter (25%) of patients had a recurrence at 4.5 months (SD ± 4.1) after the initial resolution of papilledema.

Conclusions: Fulminant IIH is a rare and blinding variant of IIH. Owing to the recurrence time after initial papilledema resolution, we recommend all such patients be monitored until 8 months after initial papilledema resolution. Future studies should evaluate optimal surgical interventions for preserving vision.

Background: In many parts of the world including India, the prevalence of autoimmune inflammatory diseases such as neuromyelitis optica spectrum disorders (NMOSD), myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), and multiple sclerosis (MS) is rising. A diagnosis is often delayed due to insufficient diagnostic tools. Machine learning (ML) models have accurately differentiated eyes of patients with MS from those of healthy controls (HCs) using optical coherence tomography (OCT)-based retinal images. Examining OCT characteristics may allow for early differentiation of these conditions. The objective of this study was to determine feasibility of ML analyses to distinguish between patients with different autoimmune inflammatory diseases, other ocular diseases, and HCs based on OCT measurements of the peripapillary retinal nerve fiber layer (pRNFL), ganglion cell-inner plexiform layer (GCIPL), and inner nuclear layers (INLs).

Methods: Eyes of people with MS (n = 99 patients), NMOSD (n = 40), MOGAD (n = 74), other ocular diseases (OTHER, n = 16), and HCs (n = 54) from the Mangalore Demyelinating Disease Registry were included. Support vector machine (SVM) classification models incorporating age, pRNFL, GCIPL, and INL were performed. Data were split into training (70%) and testing (30%) data and accounted for within-patient correlations. Cross-validation was used in training to choose the best parameters for the SVM model. Accuracy and area under receiver operating characteristic curves (AUROCs) were used to assess model performance.

Results: The SVM models distinguished between eyes of patients with each condition (i.e., MOGAD vs NMOSD, NMOSD vs HC, MS vs OTHER, etc) with strong discriminatory power demonstrated from the AUROCs for these comparisons ranging from 0.81 to 1.00. These models also performed with moderate to high accuracy, ranging from 0.66 to 0.81, with the exception of the MS vs NMOSD comparison, which had an accuracy of 0.53.

Conclusions: ML models are useful for distinguishing between autoimmune inflammatory diseases and for distinguishing these from HCs and other ocular diseases based on OCT measures. This study lays the groundwork for future deep learning studies that use analyses of raw OCT images for identifying eyes of patients with such disorders and other etiologies of optic neuropathy.

Background: Patients with craniosynostosis are at high risk of developing elevated intracranial pressure (ICP) causing papilledema and secondary optic atrophy. Diagnosing and monitoring optic neuropathy is challenging because of multiple causes of vision loss including exposure keratopathy, amblyopia, and cognitive delays that limit examination. Peripapillary hyperreflective ovoid mass-like structures (PHOMS) are an optical coherence tomography (OCT) finding reported in association with papilledema and optic neuropathy. We investigated the prevalence of PHOMS in patients with syndromic craniosynostosis and the relationship with known risk factors of optic neuropathy.

Methods: This was a cross-sectional retrospective study was performed at Boston Children's Hospital and included 118 eyes (60 patients) with syndromic craniosynostosis that had at least one good quality optic nerve OCT volumetry scan between January 2010 and December 2023. Testing was reviewed by 2 neuro-ophthalmologists to determine the presence of PHOMS. Information collected included demographics, and clinical course including possible Chiari malformation, obstructive sleep apnea (OSA), history of elevated ICP, best-corrected visual acuity (VA), spherical equivalent (SE), retinal nerve fiber layer thickness, macular ganglion cell layer volume, and funduscopic examination. Our primary outcome measure was presence of PHOMS, and secondary outcome measure was the relationship between clinical characteristics and the presence of PHOMS.

Results: Mean age at imaged OCT was 9.8 ± 5.2 years and 41/60 (68.3%) patients were female. The prevalence of PHOMS was 17/60 patients (28.3%) or 26/118 eyes (22.0%), higher than previously reported in children without craniosynostosis (P < 0.001). PHOMS were significantly associated with a history of elevated ICP (odds ratio [OR] 14.4, 95% CI: 1.9-111.0, P < 0.001) and with concurrent papilledema (OR 40.4, 95% CI: 6.6-248.0, P < 0.001). OSA, Chiari malformation, best-corrected VA, craniosynostosis variant, and SE were not independently associated with PHOMS.

Conclusions: Eyes in children with craniosynostosis had a higher prevalence of PHOMS than reported in children without craniosynostosis. PHOMS were significantly more common with a history of elevated ICP and with concurrent papilledema. PHOMS may serve as a clinically useful indicator of optic neuropathy, and of recurrence of papilledema in patients with craniosynostosis and in other populations characterized by multiple sources of vision loss and cognitive changes that limit evaluation.

Background: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare and poorly understood inflammatory disorder of the central nervous system centered on the pons. It has a characteristic imaging appearance with enhancing and T2-hyperintense punctate and curvilinear lesions in the pons. The lesions lack restricted diffusion and have relatively little perilesional edema. Although patients typically present with gait ataxia and other sequelae of brainstem inflammation including diplopia, there is scant literature focusing on patients who present with primarily neuro-ophthalmic manifestations.

Methods: Case series of 3 patients presenting with diplopia who had a final diagnosis of CLIPPERS.

Results: Case descriptions of a 71-year-old man, 61-year-old woman, and 38-year-old man are reported. Diplopia was the chief presenting complaint, owing to internuclear ophthalmoplegia, sixth nerve palsy, or skew deviation. All patients had nystagmus and gait ataxia. Brain MRI displayed punctate or curvilinear enhancement of pontine/middle cerebellar peduncle lesions without restricted diffusion. All patients achieved rapid improvement after corticosteroid treatment.

Conclusions: In 3 patients with CLIPPERS, the main presenting complaint was diplopia. The distinctive imaging signs led to a strong presumption of CLIPPERS, permitting a truncated evaluation and early corticosteroid treatment, which provided rapid reversal of clinical and imaging manifestations.