Cranial Nerve Palsies in the Setting of Arachnoid Cysts: A Case Series and Literature Review.

BACKGROUND: Arachnoid cysts (ACs) are benign lesions typically believed to not cause neurologic defects in the adult population and are most often found incidentally on imaging. We describe 2 patients with ACs potentially leading to isolated cranial nerve (CN) dysfunction. METHODS: We describe 2 patients, 1 with a fourth nerve palsy and the other with a sixth nerve palsy found to have ACs on MRI brain imaging in locations that potentially caused a compressive CN palsy. We review previous literature of ACs presenting with CN III, IV, or VI palsy. RESULTS: Patient 1 was a 62-year-old man who presented with a 22-year history of diplopia with strabismus examination consistent with a congenital CN IV palsy. Despite multiple surgeries, his CN IV palsy insidiously worsened. An AC in the posterior fossa with mass effect on the quadrigeminal plate and asymmetric atrophy of the right superior oblique was identified on imaging. Patient 2 was an 80-year-old man who presented with an 18-year history of diplopia and was found to have a left esotropia and abduction deficit consistent with complete CN VI palsy. An AC in the left cavernous sinus was identified on imaging. He underwent strabismus surgery with satisfactory resolution of diplopia. We identified a total of 18 previously published cases: 8 reports of CN III palsy, 4 reports of CN IV palsy, and 6 reports of CN VI palsy. Patient ages ranged from 1 to 67 with a median of 34.5. In 16/18 (89%) cases, the diagnosis of ACs was made within 1 year of symptom onset. Surgical removal of the AC was successful in resolving diplopia in 7/12 (58%) cases. In no case was strabismus surgery performed as primary treatment. CONCLUSIONS: Although ACs are typically congenital asymptomatic lesions, we present a case series of 2 patients with ACs in anatomic locations that potentially caused chronic, progressive, isolated CN palsies leading to strabismus. Our literature review revealed that most published cases detailing this clinical scenario resulted in neurosurgical fenestration of ACs with mixed results. Our cases represent 2 patients with AC-associated CN palsy treated with strabismus surgery.

Comparison of Uveitis Incidence by Medication in Juvenile Idiopathic Arthritis and Implications for Screening.

PURPOSE: To determine uveitis incidence in juvenile idiopathic arthritis (JIA) patients treated with disease-modifying antirheumatic drugs (DMARD) medications, and to evaluate uveitis risk-stratification protocols. DESIGN: Retrospective clinical cohort study. METHODS: Medical records of patients with JIA seen by ophthalmology at a single institution from April 2014 to April 2022 and ≥18 months' follow-up were reviewed. Exclusion criteria included uveitis history prior to study period, Still disease, or <18 months' follow-up. Patient characteristics, medications, and uveitis status were recorded. Factors associated with uveitis development were analyzed and statistically significant metrics used to determine empiric risk-stratification criteria. These criteria and American College of Rheumatology (ACR) risk-stratification guidelines were applied retroactively to determine predictive power. RESULTS: One hundred eighty-four patients met inclusion criteria and were included. Twenty-one new cases of uveitis developed during the study period. There were no statistically significant differences between no DMARD treatment, methotrexate (MTX), and etanercept (ETA) groups in uveitis incidence, whereas the adalimumab (ADA) and other biologics groups had no uveitis cases. Under the empirically determined criteria, the ratio of uveitis incidence between high- and low-risk groups was 8.21 (2.68-33.55; P < .0001), whereas it was 1.90 (0.72-4.93; P = .15) under the ACR criteria. CONCLUSION: Patients on MTX, ETA, and no DMARD treatment were comparable in JIA-associated uveitis incidence, whereas there were no new cases with ADA or other biologics. Further, we found increased predictive power in the empiric criteria in comparison to current ACR risk stratification.

Identification of a Novel TCF4 Isoform in the Human Corneal Endothelium.

PURPOSE: Alternative splice isoforms of TCF4, a gene implicated in Fuchs corneal dystrophy, have been identified in multiple human tissues outside of the eye. The aim of this study was to identify the transcriptional profile of TCF4 in the corneal endothelium. METHODS: We extracted RNA from the donor corneal endothelium and performed rapid amplification of cDNA ends. We tested the expression pattern of 1 newly identified isoform (7b) in a panel of cDNA derived from multiple human tissues and included cDNA from corneal endothelial (CE) and retinal pigment epithelial cell lines. To further delineate differential expression of TCF4 splice variants that span CTG18.1, we analyzed expression of 6 alternative splice isoforms that are transcribed from either exon 2 or 3 in RNA extracted from the corneal endothelium of 3 normal donors and a CE cell line. RESULTS: We identified 11 different isoforms in control CE tissue, including 1 isoform (7b) not reported previously. This isoform is enriched specifically in the corneal endothelium and placenta compared with other tissues in a panel of human cDNA. CONCLUSIONS: We demonstrate the complex expression profile of TCF4 in the human corneal endothelium and reveal expression of alternative splice variants of TCF4.