Optic disc edema and visual loss from paracentral acute middle maculopathy mimicking optic neuritis

ABSTRACT Optic neuritis is an important cause of unilateral and acute visual loss in young adults, but other differential diagnoses should be considered, especially when the disease has an atypical presentation. This report presents the case of a young woman with reduced visual acuity in her right eye, associated with optic disc edema and a relative afferent pupillary defect, that was initially misdiagnosed as optic neuritis and subsequently found to have paracentral acute middle maculopathy, possibly secondary to subtle impending central retinal vein occlusion. This case emphasizes the need to remember that retinal vascular diseases can occasionally mimic optic neuritis. Detailed anamnesis and ophthalmic examination can avoid unnecessary interventions.

The optic nerve sheath in hypertensive disorders of pregnancy and perinatal outcomes: a cohort study.

BACKGROUND: Preeclampsia is implicated in 14% of maternal deaths worldwide, mostly due to complications such as intracranial hemorrhage and cerebral edema. Cerebral edema increases intracranial pressure, which can be predicted by ultrasonographic measurement of the optic nerve sheath diameter (ONSD). Greater diameters have been reported in women with preeclampsia and eclampsia; however, data are lacking on the possible association with maternal and neonatal adverse outcomes. This study aimed to determine whether there is an association between hypertensive disorders of pregnancy and the ONSD, and between this measurement and maternal and neonatal adverse outcomes. METHODS: This was a cohort study involving 183 women in the third trimester of pregnancy or within 24 h following childbirth, distributed as follows: control group (n = 30), gestational hypertension (n = 14), chronic hypertension (n = 12), preeclampsia without severe features (n = 12), preeclampsia with severe features (n = 62), superimposed preeclampsia (n = 23) and eclampsia (n = 30). The participants underwent ocular ultrasonography, and data on maternal and neonatal outcomes were collected from the medical records. To compare the groups, analysis of variance was used for the normally distributed numerical variables and the Kruskal-Wallis test was used for variables with non-normal distribution. Two-tailed p-values ≤ 0.05 were considered statistically significant. RESULTS: Overall comparison between the seven groups showed no statistically significant difference in the mean ONSD (p = 0.056). Nevertheless, diameters were significantly greater in the eclampsia group compared to the control group (p = 0.003). Greater diameters were associated with maternal admission to the intensive care unit (ICU) (p < 0.01) and maternal near miss (p = 0.01). There was no association between ONSD and admission to the neonatal ICU (p = 0.1), neonatal near miss (p = 0.34) or neonatal death (p = 0.26). CONCLUSIONS: No association was found between ONSD and the hypertensive disorders of pregnancy in the overall analysis; however, ONSD was greater in women with eclampsia compared to controls. Greater diameters were associated with maternal admission to the ICU and maternal near miss. These findings suggest a potential use for bedside ultrasound as an additional tool for stratifying risk in patients with hypertensive disorders of pregnancy.

Clinical, laboratory and neuroimaging profile of patient's cohort with septo-optic dysplasia treated at a pediatric university hospital.

OBJECTIVES: Septo-optic dysplasia (SOD) is a relatively rare clinical condition. However, there has been a significant increase in its incidence over the years. Diagnosis is clinical and made when there are at least 2 components of the classic triad: Optic nerve hypoplasia (ONH), midline malformation, and pituitary dysfunction. This study aims to describe the clinical and complementary exam characteristics of patients with SOD. METHODS: A retrospective study of review of medical records of 48 patients cohort (24 female) with SOD followed to 2023. RESULTS: The average age at diagnosis was 3.90 ±â€¯3.85 years. Maternal age was ≤ 25 years at the time of delivery in 50% (24/48) of cases. Visual and developmental impairment was observed in 21 (43,7%) and nystagmus in 15 patients. Fourteen of them developed short stature. Regarding the diagnostic criteria for SOD: 92.6% (38/41) had ONH (78.9% bilaterally), 95.3% (41/43) had structural midline abnormalities, 85.7% (24/28) had hypothalamic-pituitary region alterations, and 73% had at least one hormonal deficiency, of which 2/3 had multiple pituitary dysfunctions. The most frequent deficiencies were thyroid-stimulating hormone and growth hormone, and the average age at diagnosis of the first dysfunction was 4.25 ±â€¯3.71 years. CONCLUSION: Clinical manifestations that most led to early suspicion were developmental delay, nystagmus and visual impairment. More than 1/3 of the patients had the complete triad and 2/3 developed multiple pituitary deficiencies, with TSH deficiency being the most prevalent followed by GH deficiency. Patients with ONH or midline structural changes should undergo endocrine evaluation.

Determination of toxicological relevant arsenic species in urine by hydride generation microwave-induced plasma optical emission spectrometry.

An analytical method for the determination of toxicological relevant species of arsenic in urine was developed and validated using hydride generation microwave-induced emission spectrometry (HG-MP-AES). This strategy can be used as an alternative to HG-HPLC-ICP-MS considered as a reference technique for arsenic speciation. This procedure is notably less expensive than other techniques and sample preparation and requires only a few steps.•Hydride generation with MP-AES detection has proven to be an effective technique for measuring arsenic metabolites in urine, which is relevant for occupational monitoring and health risk assessment purposes.•This method offers simplicity and cost-effectiveness, serving as an alternative to classical analytical procedures typically used for arsenic analysis in urine.•The methodology has been successfully applied for the purpose of workers' health surveillance.

The Neuroprotective Effect of Erythropoietin on the Optic Nerve and Spinal Cord in Rats with Experimental Autoimmune Encephalomyelitis through the Activation of the Extracellular Signal-Regulated Kinase 1/2 Signaling Pathway.

Experimental autoimmune encephalomyelitis is a demyelinating disease that causes paralysis in laboratory rats. This condition lacks treatment that reverses damage to the myelin sheaths of neuronal cells. Therefore, in this study, treatment with EPO as a neuroprotective effect was established to evaluate the ERK 1/2 signaling pathway and its participation in the EAE model. EPO was administered in 5000 U/Kg Sprague Dawley rats. U0126 was used as an inhibitor of the ERK 1/2 pathway to demonstrate the possible activation of this pathway in the model. Spinal cord and optic nerve tissues were evaluated using staining techniques such as H&E and the Luxol Fast Blue myelin-specific technique, as well as immunohistochemistry of the ERK 1/2 protein. The EPO-treated groups showed a decrease in cellular sampling in the spinal cord tissues but mainly in the optic nerve, as well as an increase in the expression of the ERK 1/2 protein in both tissues. The findings of this study suggest that EPO treatment reduces cellular death in EAE-induced rats by regulating the ERK pathway.

The real-world applicability of the 2023 international myelin oligodendrocyte glycoprotein antibody-associated disease criteria in a Latin American cohort.

BACKGROUND AND PURPOSE: The diagnostic criteria for myelin oligodendrocyte glycoprotein antibody (MOG-IgG)-associated disease (MOGAD) were published in 2023. We aimed to determine the performance of the new criteria in Latin American (LATAM) patients compared with the 2018 criteria and explore the significance of MOG-IgG titers in diagnosis. METHODS: We retrospectively reviewed the medical records of LATAM (Argentina, Chile, Brazil, Peru, Ecuador, and Colombia) adult patients with one clinical MOGAD event and MOG-IgG positivity confirmed by cell-based assay. Both 2018 and 2023 MOGAD criteria were applied, calculating diagnostic performance indicators. RESULTS: Among 171 patients (predominantly females, mean age at first attack = 34.1 years, mean disease duration = 4.5 years), 98.2% patients met the 2018 criteria, and of those who did not fulfill diagnostic criteria (n = 3), all tested positive for MOG-IgG (one low-positive and two without reported titer). Additionally, 144 (84.2%) patients met the 2023 criteria, of whom 57 (39.5%) had MOG-IgG+ titer information (19 clearly positive and 38 low-positive), whereas 87 (60.5%) patients had no MOG-IgG titer. All 144 patients met diagnostic supporting criteria. The remaining 27 patients did not meet the 2023 MOGAD criteria due to low MOG-IgG (n = 12) or lack of titer antibody access (n = 15), associated with the absence of supporting criteria. The 2023 MOGAD criteria showed a sensitivity of 86% (95% confidence interval = 0.80-0.91) and specificity of 100% compared to the 2018 criteria. CONCLUSIONS: These findings support the diagnostic utility of the 2023 MOGAD criteria in an LATAM cohort in real-world practice, despite limited access to MOG-IgG titration.

Long-term follow-up of a patient with partial optic nerve avulsion associated with submacular hemorrhage who underwent pneumatic displacement.

Purpose: We report the case of a 16-year-old boy with partial optic nerve avulsion (ONA) and submacular hemorrhage (SMH) resulting from blunt ocular trauma who underwent pneumatic displacement and subsequent monitoring with optical coherence tomography (OCT) and fundus photography. Observations: Reduced visual acuity was observed in the right eye at presentation (20/2400). Vitreous hemorrhage, partial ONA, and SMH were observed during dilated fundus examination. SMH was managed via pneumatic displacement. Subsequent examination revealed improvement in the visual acuity of the right eye with a substantial reduction in the subfoveal hemorrhage. Further improvement in visual acuity was observed 6 months after the injury (20/150). A smaller optic nerve head excavation defect, foveal atrophy, and reabsorption of SMH were observed during fundus examination. OCT of the optic nerve revealed that glial growth had covered the avulsion excavation. However, atrophy of the outer retinal layer of the fovea was observed during macular OCT. Conclusions and importance: This case emphasizes the importance of performing multimodal imaging in cases of ONA as it enables the identification of alterations in the retinal layers and optic nerve. The subretinal hemorrhage was displaced from the subfoveal region without any adverse effects.

Could the thick of retinal nerve fiber layer be a potential measure of axonal loss in hearing loss?

OBJECTIVE: The objective of the study is to compare the optic coherence tomography (OCT) parameters of the healthy and affected sides of patients with idiopathic sudden sensorineural hearing loss (ISSNHL) and to investigate the relationships between these and the improvement in hearing levels. METHODS: A bilateral eye evaluation of patients diagnosed with ISSNHL was performed with OCT. The ganglion cell complex (GCC) and retina nerve fiber layer (RNFL) thickness values were recorded and the differences between the two eyes were examined. RESULTS: An evaluation was made of 39 patients with a mean age of 44.82 ± 14.90 years. The RNFL thickness of the eyes was determined to be mean 89.87 ± 3.65 µm on the affected side and 103.87 ± 3.98 µm on the healthy control side (p = 0.0001). The mean GCC was determined to be mean 90.46 ± 3.49 µm on the affected side and 103.77 ± 3.96 µm on the healthy control side (p = 0.0001). CONCLUSIONS: A statistically significant difference was observed between the healthy and affected eyes of patients with ISSNHL with respect to mean GCC and mean RNFL thickness. OCT could be a useful technique for measuring this neural degeneration.

OBJETIVO: Comparar e investigar los parámetros de la tomografía de coherencia óptica (OCT) de los lados sanos y afectados de pacientes con pérdida auditiva neurosensorial súbita idiopática (PANSI). MÉTODO: La evaluación ocular bilateral de los pacientes diagnosticados con PANSI se realizó con OCT. Se registraron los valores de espesor del complejo de células ganglionares (CCG) y de la capa de fibras nerviosas de la retina (CFNR), y se examinaron las diferencias entre los dos ojos. RESULTADOS: Se evaluaron 39 pacientes, con una edad media de 44.82 ± 14.90 años. Se determinó que el grosor de la CFNR de los ojos era una media de 89.87 ± 3.65 µm en el lado afectado y 103.87 ± 3.98 µm en el lado de control sano (p = 0.0001). Se determinó que el CCG medio era 90.46 ± 3.49 µm en el lado afectado y 103.77 ± 3.96 µm en el lado de control sano (p = 0.0001). CONCLUSIONES: Se encontró una diferencia estadísticamente significativa entre los ojos sanos y afectados de pacientes con PANSI con respecto al CCG medio y al espesor medio de la CFNR. La OCT podría ser una técnica útil para medir esta degeneración neuronal.

Permanent Visual Loss as a Complication of Decompressive Surgery for the Treatment of Posterior Fossa Tumours: A Report of Two Cases.

Posterior fossa tumours are one of the most common types of solid neoplasia in paediatric patients. Although impaired vision can occur at presentation, it usually stabilises or improves after decompressive surgery. However, cases of permanent and profound visual loss have been reported following successful tumour resection, despite receiving little attention from the medical community. In this paper, we present two cases of young patients who experienced severe and permanent visual loss following uncomplicated surgery for posterior fossa tumour removal. We discuss the possible mechanism involved in the visual loss and measures to prevent such a dreadful complication.

Knockout mice with pituitary malformations help identify human cases of hypopituitarism.

BACKGROUND: Congenital hypopituitarism (CH) and its associated syndromes, septo-optic dysplasia (SOD) and holoprosencephaly (HPE), are midline defects that cause significant morbidity for affected people. Variants in 67 genes are associated with CH, but a vast majority of CH cases lack a genetic diagnosis. Whole exome and whole genome sequencing of CH patients identifies sequence variants in genes known to cause CH, and in new candidate genes, but many of these are variants of uncertain significance (VUS). METHODS: The International Mouse Phenotyping Consortium (IMPC) is an effort to establish gene function by knocking-out all genes in the mouse genome and generating corresponding phenotype data. We used mouse embryonic imaging data generated by the Deciphering Mechanisms of Developmental Disorders (DMDD) project to screen 209 embryonic lethal and sub-viable knockout mouse lines for pituitary malformations. RESULTS: Of the 209 knockout mouse lines, we identified 51 that have embryonic pituitary malformations. These genes not only represent new candidates for CH, but also reveal new molecular pathways not previously associated with pituitary organogenesis. We used this list of candidate genes to mine whole exome sequencing data of a cohort of patients with CH, and we identified variants in two unrelated cases for two genes, MORC2 and SETD5, with CH and other syndromic features. CONCLUSIONS: The screening and analysis of IMPC phenotyping data provide proof-of-principle that recessive lethal mouse mutants generated by the knockout mouse project are an excellent source of candidate genes for congenital hypopituitarism in children.

Enfermedad desmielinizante asociada a anticuerpos MOG y encefalitis de tallo crónica: cuándo sospecharla / Demyelinating disease associated with MOG-IgG antibodies and chronic brainstem encephalitis: When to suspect it

Resumen Introducción: La enfermedad asociada a la glicoproteína oligodendrocitaria de mielina (MOGAD) comprende un espectro amplio de manifestaciones clínicas y hallazgos imagenológicos, donde la evidencia reciente indica que la encefalitis de tallo es una posible manifestación de MOGAD. Presentación de caso: Se presenta el caso de una paciente femenina de 32 años con curso de enfermedad fluctuante y buena respuesta a esteroides, con afectación predominante del tallo cerebral de nueve años de evolución y posterior compromiso del nervio óptico. La paciente no cumplía criterios para esclerosis múltiple o neuromielitis óptica y otros diagnósticos diferenciales fueron excluidos. Finalmente, el diagnóstico de MOGAD fue confirmado mediante la detección de anticuerpos IgG contra la proteína MOG. Discusión: Las lesiones de tallo cerebral en MOGAD se han descrito en el 30 % de los casos de una cohorte española de pacientes positivos para MOG-IgG. Las lesiones tienden a ser bilaterales, mal definidas y de gran tamaño. A pesar de que los bajos títulos del examen pueden generar dudas sobre el diagnóstico, Banwell et al. recientemente propusieron unos nuevos criterios diagnósticos que incluyen bajos títulos de MOG-IgG y, ante ello, los criterios clínicos de soporte apoyan el diagnóstico. Conclusiones: Los pacientes con signos de encefalitis de tallo sin clara etiología deben ser evaluados con anticuerpos asociados a la proteína MOG, para confirmar o descartar el diagnóstico.

Abstract Introduction: MOG antibody-associated disease (MOGAD) comprises a broad spectrum of clinical manifestations and imaging findings. Recent evidence indicates that brainstem encephalitis is a possible manifestation of MOGAD. Case presentation: We present the case of a 32-year-old female patient with a 9-year history of a fluctuating disease course and good response to steroids, with almost exclusive brainstem involvement and subsequent optic nerve involvement. The patient did not meet the criteria for multiple sclerosis or neuromyelitis optica, and other differentials were excluded. The diagnosis of MOGAD was confirmed by IgG antibodies against the MOG protein. Discussion: Brainstem lesions in MOGAD have been described in 30% of a Spanish cohort of MOG-IgG positive patients. The lesions tend to be bilateral, poorly defined, and large. Although low titers on the assay may raise doubts about the diagnosis, Banwell et al recently proposed new diagnostic criteria that include low MOG-IgG titers. In the face of low titers, the supporting clinical criteria support the diagnosis. Conclusions: Patients with signs of brainstem encephalitis without a clear etiology should be evaluated for MOG-associated antibodies to confirm or rule out the diagnosis.

Cerebral hemodynamics and optic nerve sheath diameter acquired via neurosonology in critical patients with severe coronavirus disease: experience of a national referral hospital in Peru.

Aim: We aimed to describe the neurosonological findings related to cerebral hemodynamics acquired using transcranial Doppler and to determine the frequency of elevated ICP by optic nerve sheath diameter (ONSD) measurement in patients with severe coronavirus disease (COVID-19) hospitalized in the intensive care unit of a national referral hospital in Peru. Methods: We included a retrospective cohort of adult patients hospitalized with severe COVID-19 and acute respiratory failure within the first 7 days of mechanical ventilation under deep sedoanalgesia, with or without neuromuscular blockade who underwent ocular ultrasound and transcranial Doppler. We determine the frequency of elevated ICP by measuring the diameter of the optic nerve sheath, choosing as best cut-off value a diameter equal to or >5.8 mm. We also determine the frequency of sonographic patterns obtained by transcranial Doppler. Through insonation of the middle cerebral artery. Likewise, we evaluated the associations of clinical, mechanical ventilator, and arterial blood gas variables with ONSD ≥5.8 mm and pulsatility index (PI) ≥1.1. We also evaluated the associations of hemodynamic findings and ONSD with mortality the effect size was estimated using Poisson regression models with robust variance. Results: This study included 142 patients. The mean age was 51.39 ± 13.3 years, and 78.9% of patients were male. Vasopressors were used in 45.1% of patients, and mean arterial pressure was 81.87 ± 10.64 mmHg. The mean partial pressure of carbon dioxide (PaCO2) was elevated (54.08 ± 16.01 mmHg). Elevated intracranial pressure was seen in 83.1% of patients, as estimated based on ONSD ≥5.8 mm. A mortality rate of 16.2% was reported. In the multivariate analysis, age was associated with elevated ONSD (risk ratio [RR] = 1.07). PaCO2 was a protective factor (RR = 0.64) in the cases of PI ≥ 1.1. In the mortality analysis, the mean velocity was a risk factor for mortality (RR = 1.15). Conclusions: A high rate of intracranial hypertension was reported, with ONSD measurement being the most reliable method for estimation. The increase in ICP measured by ONSD in patients with severe COVID-19 on mechanical ventilation is not associated to hypercapnia or elevated intrathoracic pressures derived from protective mechanical ventilation.

Avalanche-assisted transient optical phenomenon in aggregated toluidine blue dye.

Pulse-modulated CW laser heat deposition modulates the darkness or the transparency of an aggregated medium in the high signal optical regimen. A recently reported work found that transient optical responses of molecular aggregates can be different depending on whether the sample is excited with a laser wavelength tuned within the absorption band of the monomer or within the absorption band of the aggregates. The different transient responses were attributed to different dynamic processes during the laser-induced disassembling of the molecular aggregates and may have implications in the field of organic electronics and optical devices, such as optical logical gates, optical power limiters and all-optical switching. In this paper laser beams with wavelengths of 663 nm and 532 nm were used to produce sudden changes in the thermodynamic equilibrium of the aggregation states of the ortho-toluidine blue dye, which allowed to observe the occurrence of the avalanche - mediated transient phenomenon in the laser-induced disassembling of ortho-toluidine blue (TBO) aggregates. A double exponential model was adjusted to the registered transient data. The obtained values for the fast components of the transient time responses of ortho-toluidine blue dye, for the studied concentrations, ranged from âˆ¼ 6.5 to 9.5 ms at 532 nm, and from âˆ¼ 43 to 48 ms at 663 nm. A single beam experiment was employed to evaluate the performance of the ortho-toluidine blue dye in a beam power-damping device, driven by the simultaneous and cooperative actions of the laser induced disassembling of aggregated dye units and the thermal lensing effect. It was found that the phenomenon of laser-induced dye disassembling of TBO, acting cooperatively with the thermal lensing effect, damps the laser beam power faster than the thermal lensing phenomenon alone. In addition, the results showed that the speed of the laser beam power-damping is dye dependent.

Optic neuritis and mydriasis after vaccination: a case report.

BACKGROUND: Optic neuritis (ON) is an inflammatory demyelinating condition of the optic nerve, with various causes. Its incidence is higher in children and young adults than in older adults of both genders, but is more common in women than in men. ON is rarely associated with mydriasis, and it is seldom triggered by vaccines against tetanus and diphtheria. CASE REPORT: A 36-year-old Caucasian woman presented with bilateral ON that had started 18 days after administration of a booster dose of the double adult vaccine (dT) against diphtheria and tetanus. Bilateral mydriasis persisted after treatment and clinical resolution of the ON. She experienced severe headache, blurred vision, decreased visual acuity in the right eye and bilateral mydriasis, a diagnosis confirmed by imaging tests. Treatment with oral corticosteroids resulted in rapid resolution of the neuritis; however, mydriasis persisted for several months. CONCLUSION: This study describes a very unusual case of bilateral ON associated with prolonged mydriasis after vaccination against tetanus and diphtheria that regressed after treatment with oral corticosteroids. Prolonged mydriasis was the manifestation that differed from the other cases previously described.

Early detection of optic nerve head changes using optical coherence tomography after using mesenchymal stromal cells as intravitreal therapy in rabbit models of ocular hypertension.

Background and Aim: Stem cell therapy is considered a promising treatment for several neurodegenerative diseases. However, there are very few studies on the use of this therapy in glaucoma models. By detecting the changes produced by glaucoma early, cell therapy could help prevent the events that lead to blindness. In this study, early changes in the optic nerve head (ONH) as detected by optical coherence tomography (OCT) after the application of human Wharton's jelly-derived mesenchymal stromal cells (hWJ-MSCs) in an experimental model of ocular hypertension (OH) were evaluated. Materials and Methods: Fifteen New Zealand rabbits were randomly divided into the following three groups: G1: OH, G2: hWJ-MSCs, and G3: OH + hWJ-MSCs. An OH model was constructed, and the intraocular pressure (IOP) was measured regularly. At week 7, 105/100 µL hWJ-MSCs were intravitreally injected. Retinography and OCT were used to evaluate structural changes in ONH. Results: IOP increased significantly in G1 and G3 from week 3 onward. Retinography revealed more significant optic nerve changes, that is, papillary asymmetry suggestive of optic nerve excavation, vascular alterations, and irregular hypopigmentation peripheral to the optic disk margin, in G1 compared with G3. OH locates the hWJ-MSCs solution in the vitreous in front of the optic nerve. OCT revealed retinal nerve fiber layer (RNFL) reduction in all groups, reduced optic cup volume in G2 and G3 between weeks 1 and 9, and significant ganglion cell layer thickness reduction in G1 and a slight increase in G3. Conclusion: Intravitreal hWJ-MSCs injection produced changes in optic cup volume, which were detected early on by OCT; however, RNFL could not be restored in this OH model.

Ophthalmologic Findings in a Premature Infant leading to a Zika Diagnosis during the COVID-19 Pandemic: A Case Report.

We report on the first case of congenital Zika syndrome to be identified during the COVID-19 pandemic in Puerto Rico. The Zika virus (ZIKV) infection was first seen in Puerto Rico in December 2015. It is a flavivirus with vertical transmission, spreading from infected mothers to their fetuses and having a broad spectrum of clinical manifestations, of which microcephaly is the most worrisome. In Puerto Rico, routine ZIKV screening during pregnancy was implemented in October 2016. However, this practice has become less frequent over time. Nevertheless, the transmission of ZIKV continues, so it is important to ensure routine ZIKV screening in endemic regions, such as Puerto Rico.

Radiation therapy for optic nerve sheath meningiomas: Local control and treatment related visual changes.

Objective: Our primary objective is to evaluate the local control of optic nerve sheath meningiomas (ONSMs) treated with ionizing radiation and related visual changes after treatment. Our secondary objective is to describe the clinical characteristics and perform an analysis of the treatment impact on the functional status of this group of patients. Methods: We present our series of 19 patients treated with ionizing radiation therapy at our radio-neurosurgery unit between 2016 and 2022. The setting, ophthalmological follow-up, morbidity, and survival are analyzed and discussed. Results: Patients were followed up, and the impact of treatment on local disease control, visual alterations of the affected eye, and functional status of the patient were analyzed. The progression-free survival (PFS) median was 60 months (95% CI 50.3-69.6 months). The estimated PFS rates at 48 and 66 months were 100% and 66%, respectively. At diagnosis, nine (47.3%) eyes were in amaurosis and ten (52.6%) with vision. Of the ten patients without amaurosis at the time of diagnosis, three (30%) maintained unchanged visual acuity, and seven (70%) had decreased visual acuity; three of them developed amaurosis during the first year after treatment (p = 0.018). Conclusions: Using ionizing radiation therapy is a successful treatment for the local control of ONSMs. This therapeutic modality can compromise the visual acuity of the affected eye and improve dyschromatopsia and campimetry defects. The life prognosis is good for these patients, with a zero mortality rate, but their vision prognosis is poor.

Optic nerve haemangioblastoma in association with von Hippel-Lindau syndrome: case report and literature review.

Optic nerve haemangioblastoma (ONH) is an uncommon, benign, non-meningothelial, mesenchymal tumour of unclear origin. Most are associated with von Hippel-Lindau (VHL) syndrome (71%), and only 40 cases have been reported in the medical literature. Most of the patients develop non-specific visual symptoms, including decreased visual acuity and/or loss of visual fields, exophthalmos, trigeminal neuralgia, and retroorbital pain. Optic nerve sheath meningioma and optic nerve glioma are among the differential diagnoses that may be considered in this location. Contrast-enhanced MRI is considered an optimal diagnostic tool, which helps to determine some characteristics that guide towards an adequate diagnosis and treatment. We present a 42-year-old patient with a history of VHL syndrome in whom a cerebellar lesion and optic nerve lesions were evidenced, and we did a review of the literature and case analysis.

Ultrasonografía del diámetro de la vaina del nervio óptico en voluntarios sanos

Fundamento: La ultrasonografía del diámetro de la vaina del nervio óptico ha sido utilizada como medidor no invasivo de la presión intracraneal. Una de las deficiencias enunciadas resulta, la no existencia de un valor estándar, que sugiera el límite entre la normalidad y la hipertensión intracraneal. Objetivo: Determinar el diámetro de la vaina del nervio óptico mediante ecografía en voluntarios sanos. Métodos: Se realizó un estudio ecológico de poblaciones múltiples en 160 voluntarios sanos que cumplieron con los criterios de inclusión. Se realizaron 3 mediciones ultrasonográficas en modo B en cada ojo, se tomó como valor final el promedio. Las variables estudiadas fueron: edad, sexo, color de la piel y diámetro de la vaina del nervio óptico. Se calculó el porcentaje, media, mediana, límite y estándar. Con un valor de referencia al 95 % del intervalo de confianza. La relación entre las variables se determinó mediante análisis de regresión logística simple con el paquete estadístico de SPSS 22.0. Resultados: El diámetro de la vaina del nervio óptico, en los sujetos, se cuantificó entre 4,62 - 4,80 mm y 4,66 - 4,83 mm para los ojos derecho e izquierdo respectivamente. Al análisis de regresión lineal simple, solo el sexo se asoció al valor del diámetro de la vaina del nervio óptico (p= 0,000). Conclusiones: En una población de voluntarios sanos el valor del diámetro de la vaina del nervio óptico fue similar a otras descripciones, con diferencias entre el sexo, lo cual sugiere tomar en consideración esta variable al hacer la medición.

Foundation: Ultrasonography of the diameter of the optic nerve sheath has been used as a non-invasive measure of intracranial pressure. One of the deficiencies stated is the non-existence of a standard value that suggests the limit between normality and intracranial hypertension. Objective: Determine the diameter of the optic nerve sheath using ultrasound in healthy volunteers. Methods: A multi-population ecological study was conducted on 160 healthy volunteers who met the inclusion criteria. Three ultrasonographic measurements were performed in B mode in each eye, the average was taken as the final value. The variables studied were: age, sex, skin color and diameter of the optic nerve sheath. The percentage, mean, median, limit and standard were calculated. With a reference value at the 95% confidence interval. The relationship between the variables was determined by simple logistic regression analysis with the SPSS 22.0 statistical package. Results: That diameter of the optic nerve sheath, in the subjects, was quantified between 4.62 - 4.80 mm and 4.66 - 4.83 mm for the right and left eyes respectively. In the simple linear regression analysis, only sex was associated with the value of the diameter of the optic nerve sheath (p= 0.000). Conclusions: In a population of healthy volunteers, the value of the diameter of the optic nerve sheath was similar to other descriptions, with differences between sex, which suggests taking this variable into consideration when making the measurement.