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Brain Sciences
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Recent Advances in Neuro-Opthalmology
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Recent Advances in Neuro-Opthalmology

A special issue of Brain Sciences (ISSN 2076-3425). This special issue belongs to the section "Neuro-otology and Neuro-ophthalmology".

Deadline for manuscript submissions: 30 June 2024 | Viewed by 8352

Special Issue Editor

Dr. Raed Behbehani

E-Mail Website
Guest Editor
Al-Bahar Ophthalmology Center, Ibn Sina Hospital, Kuwait City, Kuwait
Interests: optical coherence tomography; optic neuropathy; idiopathic intracranial hypertension; optic neuritis; Leber mitochondrial optic neuropathy; nystagmus; multiple sclerosis; neuromyelitis optic; myelin oligodendrocyte (MOG); visual evoked potentitials (VEP); pituitary tumors and sellar lesions

Special Issue Information

Dear Colleagues,

In recent years, neuro-ophthalmology has witnessed significant advances in the diagnostic and therapeutic aspects. Neuro-ophthalmology has come a long way from a specialty that “admires disease” to one that employs sophisticated and highly-technological tools for early diagnosis and evidence-based guidelines in management. In addition, our understanding of many common neuro-ophthalmic conditions has changed and evolved over the years. Treatment of some genetic neuro-ophthalmic conditions such as Leber’s hereditary optic neuropathy using gene therapy is in ongoing trials.

This Special Issue of Brain Sciences will focus on recent advances in neuro-ophthalmology and will cover the specialty's state-of-the-art trends and current research advances. Authors are kindly invited to submit manuscripts that will address a broad range of topics including: optic neuritis (MS, MOG, NMO), idiopathic intracranial hypertension, the use of optical coherence tomography in neuro-ophthalmic (optic neuropathies and disc drusen) and neurologic diseases, diagnosis and treatment of Leber’s hereditary optic neuropathy, emerging therapeutic options for thyroid eye disease and other inflammatory orbital disorders, and innovative medical and surgical therapeutic options for various neuro-ophthalmic conditions.

Dr. Raed Behbehani
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Brain Sciences is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2200 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • optic neuritis
  • idiopathic intracranial hypertension
  • optic neuropathies
  • optical coherence tomography in neuro-ophthalmic diseases
  • disc drusen
  • Leber’s hereditary optic neuropathy
  • emerging therapeutic options for thyroid eye disease
  • other inflammatory orbital disorders
  • innovative medical and surgical therapeutic options for neurotrophic keratopathy
  • artificial intelligence in neuro-ophthalmology
  • retinal biomarkers for cerebrovascular disease

Published Papers (4 papers)

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Research

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11 pages, 1292 KiB  
Article
Non-Invasive Quantitative Approximation of Intracranial Pressure in Pediatric Idiopathic Intracranial Hypertension Based on Point-of-Care Ultrasound of the Optic Nerve Sheath Diameter
by Susanne Regina Kerscher, Julian Zipfel, Andrea Bevot, Nico Sollmann, Karin Haas-Lude, Jonas Tellermann and Martin Ulrich Schuhmann
Brain Sci. 2024, 14(1), 32; https://doi.org/10.3390/brainsci14010032 - 28 Dec 2023
Viewed by 900
Abstract
Background: To investigate whether ultrasound-based optic nerve sheath diameter (US-ONSD) is a reliable measure to follow up children with idiopathic intracranial hypertension (IIH). In addition, to analyze the inter- and intra-individual relationships between US-ONSD and intracranial pressure (ICP), and to investigate whether an [...] Read more.
Background: To investigate whether ultrasound-based optic nerve sheath diameter (US-ONSD) is a reliable measure to follow up children with idiopathic intracranial hypertension (IIH). In addition, to analyze the inter- and intra-individual relationships between US-ONSD and intracranial pressure (ICP), and to investigate whether an individualized mathematical regression equation obtained from two paired US-ONSD/ICP values can be used to approximate ICP from US-ONSD values. Methods: 159 US examinations and 53 invasive ICP measures via lumbar puncture (LP) were performed in 28 children with IIH. US-ONSD was measured using a 12 Mhz linear transducer and compared to ICP values. In 15 children, a minimum of 2 paired US-ONSD/ICP determinations were performed, and repeated-measures correlation (rmcorr) and intra-individual correlations were analyzed. Results: The cohort correlation between US-ONSD and ICP was moderate (r = 0.504, p < 0.01). Rmcorr (r = 0.91, p < 0.01) and intra-individual correlations (r = 0.956–1) of US-ONSD and ICP were excellent. A mathematical regression equation can be calculated from two paired US-ONSD/ICP values and applied to the individual patient to approximate ICP from US-ONSD. Conclusions: Related to excellent intra-individual correlations between US-ONSD and ICP, an individualized regression formula, created from two pairs of US-ONSD/ICP values, may be used to directly approximate ICP based on US-ONSD values. Hence, US-ONSD may become a non-invasive and reliable measure to control treatment efficacy in pediatric IIH. Full article
(This article belongs to the Special Issue Recent Advances in Neuro-Opthalmology)
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13 pages, 990 KiB  
Article
Swept-Source Optical Coherence Tomography Thresholds in Differentiating Clinical Outcomes in a Real-World Cohort of Treatment-Naïve Multiple Sclerosis Patients
by Łukasz Rzepiński, Jan Kucharczuk, Magda Tkaczyńska, Vincenzo Parisi and Andrzej Grzybowski
Brain Sci. 2023, 13(4), 591; https://doi.org/10.3390/brainsci13040591 - 31 Mar 2023
Viewed by 1516
Abstract
This study aimed to determine whether peripapillary retinal nerve fiber layer (pRNFL) and ganglion cell–inner plexiform layer (GCIPL) thickness thresholds for single-time-point swept-source optical coherence tomography (SS-OCT) measures can differentiate the clinical outcomes of treatment-naïve people with multiple sclerosis (pwMS). A total of [...] Read more.
This study aimed to determine whether peripapillary retinal nerve fiber layer (pRNFL) and ganglion cell–inner plexiform layer (GCIPL) thickness thresholds for single-time-point swept-source optical coherence tomography (SS-OCT) measures can differentiate the clinical outcomes of treatment-naïve people with multiple sclerosis (pwMS). A total of 275 patients with the clinically isolated syndrome (n = 23), benign MS (n = 8), relapsing–remitting MS (n = 185), secondary progressive MS (n = 28), primary progressive MS (n = 31), and with no history of optic neuritis were included. The mean Expanded Disability Status Scale (EDSS) score was 3.0 ± 1.6. The cut-off values of pRNFL (87 µm and 88 µm) and GCIPL (70 µm) thicknesses have been adopted from previous studies using spectral-domain OCT. PwMS with pRNFL ≤87 µm and ≤88 µm had a longer disease duration, more advanced disability, and more frequently progressive MS variants compared to those with greater pRNFL thicknesses. In distinguishing pwMS with disability greater than or equal to the mean EDSS score (EDSS ≥ 3) from those with less severe disability, GCIPL thickness <70 µm had the highest sensitivity, while pRNFL thickness ≤87 µm had the greatest specificity. The optimal cut-off values differentiating patients with EDSS ≥ 3 from those with less severe disability was 63 µm for GCIPL thickness and 93.5 µm for pRNFL thickness. In conclusion, pRNFL and GCIPL thickness thresholds for single-time-point SS-OCT measurements may be helpful in differentiating the disability status of treatment-naïve pwMS. Full article
(This article belongs to the Special Issue Recent Advances in Neuro-Opthalmology)
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Review

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27 pages, 2406 KiB  
Review
Mitochondrial Chronic Progressive External Ophthalmoplegia
by Ali Ali, Ali Esmaeil and Raed Behbehani
Brain Sci. 2024, 14(2), 135; https://doi.org/10.3390/brainsci14020135 - 27 Jan 2024
Viewed by 1665
Abstract
Background: Chronic progressive external ophthalmoplegia (CPEO) is a rare disorder that can be at the forefront of several mitochondrial diseases. This review overviews mitochondrial CPEO encephalomyopathies to enhance accurate recognition and diagnosis for proper management. Methods: This study is conducted based on publications [...] Read more.
Background: Chronic progressive external ophthalmoplegia (CPEO) is a rare disorder that can be at the forefront of several mitochondrial diseases. This review overviews mitochondrial CPEO encephalomyopathies to enhance accurate recognition and diagnosis for proper management. Methods: This study is conducted based on publications and guidelines obtained by selective review in PubMed. Randomized, double-blind, placebo-controlled trials, Cochrane reviews, and literature meta-analyses were particularly sought. Discussion: CPEO is a common presentation of mitochondrial encephalomyopathies, which can result from alterations in mitochondrial or nuclear DNA. Genetic sequencing is the gold standard for diagnosing mitochondrial encephalomyopathies, preceded by non-invasive tests such as fibroblast growth factor-21 and growth differentiation factor-15. More invasive options include a muscle biopsy, which can be carried out after uncertain diagnostic testing. No definitive treatment option is available for mitochondrial diseases, and management is mainly focused on lifestyle risk modification and supplementation to reduce mitochondrial load and symptomatic relief, such as ptosis repair in the case of CPEO. Nevertheless, various clinical trials and endeavors are still at large for achieving beneficial therapeutic outcomes for mitochondrial encephalomyopathies. Key Messages: Understanding the varying presentations and genetic aspects of mitochondrial CPEO is crucial for accurate diagnosis and management. Full article
(This article belongs to the Special Issue Recent Advances in Neuro-Opthalmology)
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21 pages, 4745 KiB  
Review
Artificial Intelligence Frameworks to Detect and Investigate the Pathophysiology of Spaceflight Associated Neuro-Ocular Syndrome (SANS)
by Joshua Ong, Ethan Waisberg, Mouayad Masalkhi, Sharif Amit Kamran, Kemper Lowry, Prithul Sarker, Nasif Zaman, Phani Paladugu, Alireza Tavakkoli and Andrew G. Lee
Brain Sci. 2023, 13(8), 1148; https://doi.org/10.3390/brainsci13081148 - 30 Jul 2023
Cited by 17 | Viewed by 3302
Abstract
Spaceflight associated neuro-ocular syndrome (SANS) is a unique phenomenon that has been observed in astronauts who have undergone long-duration spaceflight (LDSF). The syndrome is characterized by distinct imaging and clinical findings including optic disc edema, hyperopic refractive shift, posterior globe flattening, and choroidal [...] Read more.
Spaceflight associated neuro-ocular syndrome (SANS) is a unique phenomenon that has been observed in astronauts who have undergone long-duration spaceflight (LDSF). The syndrome is characterized by distinct imaging and clinical findings including optic disc edema, hyperopic refractive shift, posterior globe flattening, and choroidal folds. SANS serves a large barrier to planetary spaceflight such as a mission to Mars and has been noted by the National Aeronautics and Space Administration (NASA) as a high risk based on its likelihood to occur and its severity to human health and mission performance. While it is a large barrier to future spaceflight, the underlying etiology of SANS is not well understood. Current ophthalmic imaging onboard the International Space Station (ISS) has provided further insights into SANS. However, the spaceflight environment presents with unique challenges and limitations to further understand this microgravity-induced phenomenon. The advent of artificial intelligence (AI) has revolutionized the field of imaging in ophthalmology, particularly in detection and monitoring. In this manuscript, we describe the current hypothesized pathophysiology of SANS and the medical diagnostic limitations during spaceflight to further understand its pathogenesis. We then introduce and describe various AI frameworks that can be applied to ophthalmic imaging onboard the ISS to further understand SANS including supervised/unsupervised learning, generative adversarial networks, and transfer learning. We conclude by describing current research in this area to further understand SANS with the goal of enabling deeper insights into SANS and safer spaceflight for future missions. Full article
(This article belongs to the Special Issue Recent Advances in Neuro-Opthalmology)
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