Journal of Neuro-Ophthalmology最新文献

Background: Symmetric optic neuropathy (SON) is commonly seen in neuro-ophthalmic practice and is often discovered incidentally. Although multiple investigations might be performed to discover the underlying cause, they are not always indicated. The aim of this study was to report a clinically reasonable and cost-effective approach to investigating patients with SON.

Methods: SON was defined as bilateral optic neuropathy with normal and/or symmetrically decreased central visual acuity, absence of relative afferent pupillary defect, presence of symmetric optic disc pallor, symmetric thinning of peripapillary retinal nerve fiber layer on optical coherence tomography, and absence of other identifiable causes of optic neuropathy. Records of all patients diagnosed with SON seen at a tertiary university-affiliated neuro-ophthalmology practice from 2016 to 2022 were reviewed to identify the yield of various investigations. Clinical data from the initial and last follow-up visit were obtained. Subgroup analysis was performed to ascertain whether diagnostic yield is higher in patients with severe visual loss (central acuity worse than 20/40) compared with those with mild visual loss (acuity 20/40 or better).

Results: One hundred thirty-six patients met inclusion criteria. Testing for OPA1 and OPA2 mutations had the highest diagnostic yield (16.0%), followed by mitochondrial genome sequencing (13.6%), serum vitamin B12 (6.1%), and serum folate (1.6%). MRI brain was performed in 54.4% of patients and had a diagnostic yield of only 5%. Both patients who had abnormal MRI had symptoms of demyelination at presentation. Patients were followed for a mean of 15.0 (SD 21.3) months. The most frequently identified etiologies of SON were Leber hereditary optic neuropathy (8.1%), alcohol/tobacco amblyopia (7.4%), vitamin B12 deficiency (5.9%), and dominant optic atrophy (2.9%). Patients with severe visual impairment were more likely to have a final diagnosis compared with those with milder visual impairment (63.9% vs 12.0%, P < 0.001).

Conclusions: The diagnostic yield of investigating SON in patients with preserved visual function, normal diet, and absence of other neurological symptoms is very low. It is reasonable to observe patients with SON with mild visual impairment, reserving costly investigations for those with the visual acuity worse than 20/40 or progressive course.

Abstract: Selective motion blindness, also known as akinetopsia, is infrequently reported in the literature. Hence, little is known about the condition including its causes, time course, pathophysiology, and current diagnostic methods. In this investigation, we comprehensively surveyed the literature using a systematic review to identify each reported case of the condition. The purpose of this study was to provide an exhaustive catalog of every published occurrence to date to identify and discuss trends, commonalities, and differences among them. Our results revealed distinct characteristics for the various etiologies of this phenomenon in addition to a shared pathophysiologic pathway among them.

Background: Third nerve palsies (TNPs) may not resolve after intervention for intracranial aneurysms that have compressed the nerve. The disability related to this lingering condition has not been assessed with the support of patient self-report.

Methods: A single-institutional retrospective study of patients with TNP who had undergone interventions for intracranial aneurysms. We used residual primary-position diplopia, a narrow zone of single binocular vision, and vision-obscuring ptosis to divide TNP recovery into complete, incomplete nondisabling, and incomplete disabling outcomes based on medical record documentation and patient self-report derived from telephonic interviews.

Results: In a cohort of 33 patients, 13 (39%) had complete TNP recovery. There were 11 patients (33%) with lingering visual disability from diplopia or ptosis present before ophthalmic interventions. Of the 6 patients who underwent ophthalmic interventions, visual disability was relieved in only 2 patients, leaving 9 patients (27%) with lingering impairment in instrumental activities of daily living. Telephonic interviews of 23 patients (70% of the cohort) confirmed that the outcome criteria we applied were accurate in assessing visual disability in 17 patients (74%). Univariate analysis using the Fisher exact test showed that aneurysmal clipping as a treatment modality was the only clinical feature associated with a favorable TNP outcome.

Conclusions: In applying a novel method of assessing disability, this study showed that more than one-quarter of patients undergoing procedures for brain aneurysms had lingering disability from third nerve palsy-associated diplopia or ptosis, despite later ophthalmic interventions. Patient self-report gleaned from telephonic interviews was valuable in largely validating the assessment method derived from medical records and in revealing differences between physician and patient estimation of disability.

Background: Optic nerve sheath meningioma (ONSM) is a rare optic nerve cancer with considerable morbidity. This national analysis validates previously known ONSM concepts while providing insight into novel risk factors.

Methods: The Surveillance, Epidemiology, and End Results (SEER) Program was queried from 2000 to 2019 for all histologic subtypes of meningioma primary to the optic nerve. Relevant clinical and demographic variables were analyzed. Asymptotic one-sample test for binomial proportions and Cox proportional hazards modeling evaluated the significance of factor associations.

Results: A total of 51 ONSM cases were extracted. A greater proportion of cases were observed in females (N = 37, 73% , P < 0.001) and individuals with age 50 years or more (N = 29, 57% , P < 0.001); the mean number of months from diagnosis to treatment was 4.6 months (SD 4.1, range 13). Psychosocial epidemiologic parameter analysis demonstrated a greater proportion of patients with married status on diagnosis (N = 31, 61% , P < 0.001), listed total family income between $55,000 and $74,999 (N = 24, 47% , P < 0.001). Relative to cases diagnosed clinically only, cases diagnosed radiologically without microscopic confirmation experienced decreased all-cause mortality (HR = 0.041, P = 0.050).

Conclusions: Our SEER national analysis affirms previously characterized ONSM concepts. Upon ONSM diagnosis and if needed, treatment protocols are not significantly delayed. Novel psychosocial factors for ONSM were identified, including marital status, total family income, and non-Hispanic white race. Additional ONSM diagnostics may reduce longitudinal mortality burden.