Journal of Neuro-Ophthalmology最新文献

Background: Multiple myeloma (MM) is a malignant disorder of plasma cells that results in tumor cells replacing the bone marrow. In extramedullary MM (EMM), however, tumor cells proliferate outside the bone marrow. EMM may produce ophthalmoplegia through direct invasion of the superior orbital fissure, cavernous sinus, and/or sphenoidal sinus. Several mechanisms have been proposed including cranial nerve palsies, direct infiltration of bone, extraocular muscle metastasis, myelomatous meningitis, and parenchymal or paraneoplastic effects.

Methods: We retrospectively reviewed the medical records of 7 patients at MD Anderson Cancer Center who suffered from ophthalmoplegia secondary to extramedullary MM between 2019 and 2021. We collected information regarding the symptoms, signs, radiographic and laboratory findings, management, complications, and prognosis of these patients throughout their disease course.

Results: Skull base MRI revealed 4 patients with ophthalmoplegias secondary to superior orbital fissure invasion, 2 patients with ophthalmoplegias secondary to cavernous sinus invasion, and 1 patient with ophthalmoplegia secondary to sphenoid sinus invasion.

Conclusions: This is a case series describing 7 patients with ophthalmoplegias secondary to EMM. Our article is unique because of the size of the included cohort, which is large when compared with most English language publications detailing such ophthalmoplegias.

Background: Idiopathic intracranial hypertension (IIH) has been associated with several MRI features. We assessed types of MRI findings and clinical-radiologic correlations in patients with IIH from one hospital system.

Methods: A retrospective chart review of IIH and control patients was conducted. Brain MRI/magnetic resonance venogram features were enumerated and assessed for correlation with body mass index (BMI) and lumbar puncture opening pressure (LPOP). Sensitivity, specificity, positive predictive value (PPV), and likelihood ratios (LRs) were calculated for each MRI sign. Significance was set at P < 0.05.

Results: One hundred one patients diagnosed with IIH, and 119 control patients had complete files and were included. Patients with IIH were predominantly female (92.8% vs 59.7%; P = <0.001), younger (30.6 years vs 46.4 years; P < 0.001), and more obese (mean BMI = 35.2 vs 29.3; P < 0.001) than controls. Mean (SD) number of MRI findings was 2.21 (1.8) in IIH and 0.6 (1.2) in controls; (P < 0.001). Vertical nerve tortuosity (44.1%; P < 0.001), TVSS (37.8%; P < 0.001), sheath expansion (36.0%; P < 0.001), globe flattening (22.5%; P < 0.001), slit ventricles (18.9%; P < 0.001), optic disc protrusion (9.9%; P = 0.007), and complete empty sella (12.6%; P < 0.042) were observed more in patients with IIH than control patients. In the IIH group, mean (SD) LPOP was 33.6 (11.11) cmH2O and weakly correlated with number of MRI findings (rho = 0.182, P = 0.057). TVSS (sensitivity 37.84%; confidence interval [CI] 29.3%-47.2%, specificity 98.32%; CI 93.5%-99.6%) had the highest PPV (95.45%) and positive LR (22.51) for IIH diagnosis.

Conclusions: These results are consistent with IIH predominance in young, obese females. In patients with IIH, the number of MRI findings exceeded controls and positively correlated with LPOP. TVSS was most predictive of IIH.

Background: Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, is a rare autoimmune disease characterized by inflammation of small- to medium-sized blood vessels (vasculitis). We described the 3 causes of GPA-associated optic neuropathy (compressive, inflammatory, or ischemic) and analyzed initial and final visual acuities (VAs) in each group, which could potentially help prognosticate visual outcomes depending on the etiology of optic neuropathy.

Methods: This was a retrospective chart review of patients who were diagnosed with GPA-associated optic neuropathy and were seen in the Department of Ophthalmology at Mayo Clinic in Rochester, Minnesota. Only patients who met the 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology GPA classification criteria were included.

Results: A total of 12 patients with GPA-associated optic neuropathy were identified. The etiology of the optic neuropathy was compressive in 6 patients, inflammatory in 4 patients, and ischemic in 3 patients. One patient had compressive optic neuropathy initially, then presented with GPA-related optic neuritis years later. Four patients in the compressive optic neuropathy group had orbital masses requiring orbitotomy for debulking, and the remaining 2 patients had compression from pachymeningitis. Average logarithm of the minimum angle of resolution (logMAR) VA at optic neuropathy onset was 1.50, 1.50, and 0.67 (Snellen equivalent 20/600, 20/600, and 20/100, respectively). At the last follow-up, average logMAR VA was 0.91, 1.73, and 1.10 (Snellen equivalent 20/160, 20/1,000, and 20/250, respectively) for each group.

Conclusion: Visual outcomes were variable, with compressive and inflammatory optic neuropathies showing improvement in 4 eyes and worsening in 3 eyes in total. Patients with ischemic optic neuropathy from GPA were either stable or worsened over the course of the disease.