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A 71-year-old woman presented a non-arteritic anterior ischemic optic neuropathy in an optic nerve with previously registered superonasal peripapillary myelinated nerve fibers. Her past medical history was significant for controlled systemic hypertension, hyperlipidemia, and diabetes mellitus. The physiologic cup was absent in both optic discs. Non-arteritic anterior ischemic optic neuropathy mainly affected the temporal and inferior sectors of the peripapillary retinal nerve fiber layer, as could be demonstrated by retinal nerve fiber layer optical coherence tomography and optic disc optical coherence tomography angiography. Unlike other published reports, just a slight regression of the myelinated nerve fibers was observed after 1 year of follow-up. This occurred because ischemia mainly affected the temporal and inferior peripapillary sectors, whereas myelinated nerve fibers were superonasal to the optic disc.

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OBJECTIVE: The objective of this study was to investigate whether the measurement of mean optic nerve sheath diameter in patients with transient ischemic attack could be used to distinguish between control groups, the acute ischemic stroke group, and subgroups within the acute ischemic stroke category. METHODS: Retrospectively, the mean optic nerve sheath diameters of patients aged 18 years and older belonging to control, transient ischemic attack, acute ischemic stroke, and subgroups within the acute ischemic stroke category were measured with initial computed tomography conducted in the emergency department. RESULTS: Out of the 773 patients included in the study, 318 (41.1%) were in the control group, 77 (10%) had transient ischemic attack, and 378 (49%) were categorized as stroke patients. The average mean optic nerve sheath diameter was significantly higher in both the stroke and transient ischemic attack groups compared with the control group (p<0.001 for both comparisons). Furthermore, the mean optic nerve sheath diameter in the stroke subgroups was significantly higher than in both the transient ischemic attack and control groups (p<0.001 for all comparisons). In transient ischemic attack patients, the mean optic nerve sheath diameter showed a significant ability to predict transient ischemic attack (AUC=0.913, p<0.001), with a calculated optimal cutoff value of 4.72, sensitivity of 94.8%, and specificity of 73.9%. CONCLUSION: The mean optic nerve sheath diameter of patients in the transient ischemic attack group was lower compared with those in the stroke subgroups but higher compared with the control group.

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The vascular and the nervous systems share similarities in addition to their complex role in providing oxygen and nutrients to all cells. Both are highly branched networks that frequently grow close to one another during development. Vascular patterning and neural wiring share families of guidance cues and receptors. Most recently, this relationship has been investigated in terms of peripheral nervous system (PNS) regeneration, where nerves and blood vessels often run in parallel so endothelial cells guide the formation of the Büngner bands which support axonal regeneration. Here, we characterized the vascular response in regenerative models of the central and peripheral nervous system. After sciatic nerve crush, followed by axon regeneration, there was a significant increase in the blood vessel density 7 days after injury. In addition, the optic nerve crush model was used to evaluate intrinsic regenerative potential activated with a combined treatment that stimulated retinal ganglion cells (RGCs) regrowth. We observed that a 2-fold change in the total number of blood vessels occurred 7 days after optic nerve crush compared to the uncrushed nerve. The difference increased up to a 2.7-fold change 2 weeks after the crush. Interestingly, we did not observe differences in the total number of blood vessels 2 weeks after crush, compared to animals that had received combined treatment for regeneration and controls. Therefore, the vascular characterization showed that the increase in vascular density was not related to the efficiency of both peripheral and central axonal regeneration.

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A 45-year-old woman presented with sudden complete vision loss in her left eye and retroorbital pain worsened by eye movements. A previous milder episode of vision loss had occurred in the same eye 1 year before, with complete recovery after high-dose intravenous methylprednisolone. She had no light perception in the left eye with a swollen optic disc, but with a normal right optic disc. There were no systemic manifestations or infections. MR scan of the brain showed extensive enlargement and enhancement of the left optic nerve and optic chiasm. After excluding infections and autoimmune markers, a left optic nerve biopsy confirmed non-caseating granulomas, leading to a diagnosis of neurosarcoidosis.

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INTRODUCTION: Neuromonitoring represents a cornerstone in the comprehensive management of patients with traumatic brain injury (TBI), allowing for early detection of complications such as increased intracranial pressure (ICP) [1]. This has led to a search for noninvasive modalities that are reliable and deployable at bedside. Among these, ultrasonographic optic nerve sheath diameter (ONSD) measurement is a strong contender, estimating ICP by quantifying the distension of the optic nerve at higher ICP values. Thus, this scoping review seeks to describe the existing evidence for the use of ONSD in estimating ICP in adult TBI patients as compared to gold-standard invasive methods. MATERIALS AND METHODS: This review was conducted in accordance with the Joanna Briggs Institute methodology for scoping reviews, with a main search of PubMed and EMBASE. The search was limited to studies of adult patients with TBI published in any language between 2012 and 2022. Sixteen studies were included for analysis, with all studies conducted in high-income countries. RESULTS: All of the studies reviewed measured ONSD using the same probe frequency. In most studies, the marker position for ONSD measurement was initially 3 mm behind the globe, retina, or papilla. A few studies utilized additional parameters such as the ONSD/ETD (eyeball transverse diameter) ratio or ODE (optic disc elevation), which also exhibit high sensitivity and reliability. CONCLUSION: Overall, ONSD exhibits great test accuracy and has a strong, almost linear correlation with invasive methods. Thus, ONSD should be considered one of the most effective noninvasive techniques for ICP estimation in TBI patients.

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Abstract Background: Positive end-expiratory pressure (PEEP) can overcome respiratory changes that occur during pneumoperitoneum application in laparoscopic procedures, but it can also increase intracranial pressure. We investigated PEEP vs. no PEEP application on ultrasound measurement of optic nerve sheath diameter (indirect measure of increased intracranial pressure) in laparoscopic cholecystectomy. Methods: Eighty ASA I-II patients aged between 18 and 60 years scheduled for elective laparoscopic cholecystectomy were included. The study was registered in the Australian New Zealand Clinical Trials (ACTRN12618000771257). Patients were randomly divided into either Group C (control, PEEP not applied), or Group P (PEEP applied at 10 cmH20). Optic nerve sheath diameter, hemodynamic, and respiratory parameters were recorded at six different time points. Ocular ultrasonography was used to measure optic nerve sheath diameter. Results: Peak pressure (PPeak) values were significantly higher in Group P after application of PEEP (p = 0.012). Mean respiratory rate was higher in Group C at all time points after application of pneumoperitoneum (p < 0.05). The mean values of optic nerve sheath diameters measured at all time points were similar between the groups (p > 0.05). The pulmonary dynamic compliance value was significantly higher in group P as long as PEEP was applied (p = 0.001). Conclusions: During laparoscopic cholecystectomy, application of 10 cmH2O PEEP did not induce a significant change in optic nerve sheath diameter (indirect indicator of intracranial pressure) compared to no PEEP application. It would appear that PEEP can be used safely to correct

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BACKGROUND: Intracerebral hemorrhage (ICH) is a deadly disease and increased intracranial pressure (ICP) is associated with worse outcomes in this context. OBJECTIVE: We evaluated whether dilated optic nerve sheath diameter (ONSD) depicted by optic nerve ultrasound (ONUS) at hospital admission has prognostic value as a predictor of mortality at 90 days. METHODS: Prospective multicenter study of acute supratentorial primary ICH patients consecutively recruited from two tertiary stroke centers. Optic nerve ultrasound and cranial computed tomography (CT) scans were performed at hospital admission and blindly reviewed. The primary outcome was mortality at 90-days. Multivariate logistic regression, ROC curve, and C-statistics were used to identify independent predictors of mortality. RESULTS: Between July 2014 and July 2016, 57 patients were evaluated. Among those, 13 were excluded and 44 were recruited into the trial. Their mean age was 62.3 ± 13.1 years and 12 (27.3%) were female. On univariate analysis, ICH volume on cranial CT scan, ICH ipsilateral ONSD, Glasgow coma scale, National Institute of Health Stroke Scale (NIHSS) and glucose on admission, and also diabetes mellitus and current nonsmoking were predictors of mortality. After multivariate analysis, ipsilateral ONSD (odds ratio [OR]: 6.24; 95% confidence interval [CI]: 1.18-33.01; p = 0.03) was an independent predictor of mortality, even after adjustment for other relevant prognostic factors. The best ipsilateral ONSD cutoff was 5.6mm (sensitivity 72% and specificity 83%) with an AUC of 0.71 (p = 0.02) for predicting mortality at 90 days. CONCLUSION: Optic nerve ultrasound is a noninvasive, bedside, low-cost technique that can be used to identify increased ICP in acute supratentorial primary ICH patients. Among these patients, dilated ONSD is an independent predictor of mortality at 90 days.

ANTECEDENTES: A hemorragia intraparenquimatosa (HIP) aguda apresenta elevada morbimortalidade e a presença de hipertensão intracraniana (HIC) confere um pior prognóstico. OBJETIVO: Avaliamos se a dilatação do diâmetro da bainha do nervo óptico (DBNO) através do ultrassom do nervo óptico (USNO) na admissão hospitalar seria preditora de mortalidade. MéTODOS: Estudo multicêntrico e prospectivo de pacientes consecutivos com HIP supratentorial primária aguda admitidos em dois centros terciários. Ultrassom do nervo óptico e tomografia computadorizada (TC) de crânio foram realizados na admissão e revisados de forma cega. O desfecho primário do estudo foi a mortalidade em 3 meses. Análises de regressão logística, curva de característica de operação do receptor (ROC, na sigla em inglês) e estatística-C foram utilizadas para identificação dos preditores independentes de mortalidade. RESULTADOS: Entre julho de 2014 e julho de 2016, 44 pacientes foram incluídos. A idade média foi 62,3 (±13,1) anos e 12 (27,3%) eram mulheres. Na análise univariada, o volume da HIP na TC de crânio, DBNO ipsilateral à HIP, glicemia, escala de coma de Glasgow (ECG) e NIHSS na admissão hospitalar, e também diabetes mellitus e não-tabagista foram preditores de mortalidade. Após análise multivariada, o DBNO ipsilateral à HIP permaneceu como preditor independente de mortalidade (odds ratio [OR]: 6,24; intervalo de confiança [IC] de 95%: 1,18­33,01; p = 0,03). O melhor ponto de corte do DBNO ipsilateral como preditor de mortalidade em 3 meses foi 5,6mm (sensibilidade 72% e especificidade 83%) e área sob a curva (AUC, na sigla em inglês) 0,71 (p = 0,02). CONCLUSãO: O USNO é um método não-invasivo, beira-leito, de baixo custo, que pode ser empregado para estimar a presença de HIC em pacientes com HIP supratentorial primária aguda. A presença de DBNO dilatada é um preditor independente de mortalidade em 3 meses nesses pacientes.

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Although the placement of an intraventricular catheter remains the gold standard method for the diagnosis of intracranial hypertension (ICH), the technique has several limitations including but not limited to its invasiveness. Current noninvasive methods, however, still lack robust evidence to support their clinical use. We aimed to estimate, as an exploratory hypothesis generating analysis, the discriminative power of four noninvasive methods to diagnose ICH. We prospectively collected data from adult intensive care unit (ICU) patients with subarachnoid hemorrhage (SAH), intraparenchymal hemorrhage (IPH), and ischemic stroke (IS) in whom invasive intracranial pressure (ICP) monitoring had been placed. Measures were simultaneously collected from the following noninvasive methods: optic nerve sheath diameter (ONSD), pulsatility index (PI) using transcranial Doppler (TCD), a 5-point visual scale designed for brain Computed Tomography (CT), and two parameters (time-to-peak [TTP] and P2/P1 ratio) of a noninvasive ICP wave morphology monitor (Brain4Care[B4c]). ICH was defined as a sustained ICP > 20 mmHg for at least 5 min. We studied 18 patients (SAH = 14; ICH = 3; IS = 1) on 60 occasions with a mean age of 52 ± 14.3 years. All methods were recorded simultaneously, except for the CT, which was performed within 24 h of the other methods. The median ICP was 13 [9.8-16.2] mmHg, and intracranial hypertension was present on 18 occasions (30%). Median values from the noninvasive techniques were ONSD 4.9 [4.40-5.41] mm, PI 1.22 [1.04-1.43], CT scale 3 points [IQR: 3.0], P2/P1 ratio 1.16 [1.09-1.23], and TTP 0.215 [0.193-0.237]. There was a significant statistical correlation between all the noninvasive techniques and invasive ICP (ONSD, r = 0.29; PI, r = 0.62; CT, r = 0.21; P2/P1 ratio, r = 0.35; TTP, r = 0.35, p < 0.001 for all comparisons). The area under the curve (AUC) to estimate intracranial hypertension was 0.69 [CIs = 0.62-0.78] for the ONSD, 0.75 [95% CIs 0.69-0.83] for the PI, 0.64 [95%Cis 0.59-069] for CT, 0.79 [95% CIs 0.72-0.93] for P2/P1 ratio, and 0.69 [95% CIs 0.60-0.74] for TTP. When the various techniques were combined, an AUC of 0.86 [0.76-0.93]) was obtained. The best pair of methods was the TCD and B4cth an AUC of 0.80 (0.72-0.88). Noninvasive technique measurements correlate with ICP and have an acceptable discrimination ability in diagnosing ICH. The multimodal combination of PI (TCD) and wave morphology monitor may improve the ability of the noninvasive methods to diagnose ICH. The observed variability in non-invasive ICP estimations underscores the need for comprehensive investigations to elucidate the optimal method-application alignment across distinct clinical scenarios.

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BACKGROUND: Wieacker-Wolff syndrome is an ultra-rare disease with X-linked inheritance characterized by arthrogryposis, intellectual disability, microcephaly, and distal limb muscle atrophy. Ophthalmic abnormalities such as ptosis, strabismus, and oculomotor apraxia have been reported in half of the patients. Wieacker-Wolff syndrome female-restricted (WRWFFR) is an even rarer disease recently used for females with a more severe phenotype. MATERIALS AND METHODS: Clinical geneticist and ophthalmic examination, neuroimaging, and exome sequencing. RESULTS: A 4 years-old girl with developmental and language delay, microcephaly, camptodactyly, digital pads, and arthrogryposis was identified by the clinical geneticist. Ophthalmic examination revealed deep-set eyes, high hyperopic astigmatism in both eyes, and reduced retinal nerve fiber layer thickness measured by optical coherence tomography. Exome sequencing identified a novel, probably pathogenic variant in the ZC4H2 gene NM_018684.3:c.145A>T p. (Lys49*) in heterozygosis. DISCUSSION: WRWFFR is an ultra-rare disease with X-linked inheritance by variants in the ZC4H2 gene. This case reports a girl with a novel nonsense variant in the ZC4H2 gene and a severe phenotype; previous reports have identified WRWFFR in females with large deletions and nonsense mutations which could explain the manifestations in the current case report. A complete ophthalmic examination should be considered in patients with WRWFFR to detect the possibly associated optic nerve involvement and other previously described manifestations such as ptosis and strabismus.

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