9,730 results match your criteria Optic Atrophy


Mitoquinone attenuates blood-brain barrier disruption through Nrf2/PHB2/OPA1 pathway after subarachnoid hemorrhage in rats.

Exp Neurol 2019 Feb 16. Epub 2019 Feb 16.

Department of Physiology and Pharmacology, Loma Linda University, 11041 Campus St, Risley Hall, Room 219, Loma Linda, CA 92354, USA. Electronic address:

Background And Purpose: Mitochondrial dysfunction is involved in the mechanism of early brain injury (EBI) following subarachnoid hemorrhage (SAH). Blood-brain barrier disruption is a devastating outcome in the early stage of SAH. In this study, we aimed to investigate the role of a mitochondria-related drug Mitoquinone (MitoQ) in blood-brain barrier disruption after SAH in rats. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.expneurol.2019.02.009DOI Listing
February 2019

Neurophysiological effect of transorbital electrical stimulation: Early results in advanced optic atrophy.

Brain Stimul 2019 Feb 7. Epub 2019 Feb 7.

Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy; Neurology Institute, Università Cattolica del Sacro Cuore, Rome, Italy.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.brs.2019.02.002DOI Listing
February 2019

The vicious circle between mitochondrial oxidative stress and dynamic abnormality mediates triethylene glycol dimethacrylate-induced preodontoblast apoptosis.

Free Radic Biol Med 2019 Feb 15. Epub 2019 Feb 15.

Institute of Stomatology, School and Hospital of Stomatology, Wenzhou Medical University, Wenzhou, PR China; Department of Endodontics, School and Hospital of Stomatology, Wenzhou Medical University, Wenzhou, PR China. Electronic address:

Oxidative stress (OS) plays crucial roles in triethylene glycol dimethacrylate (TEGDMA, a major component in dental resin)-induced apoptosis of dental pulp cells. Mitochondria are important target organelles for regulating the balance of OS, meanwhile, imbalance of the mitochondrial dynamic associated with mitochondrial dysfunction is one major molecular mechanism for oxidative damages. However, whether these mitochondrial dependent pathways were involved in the apoptosis of dental pulp cells induced by TDGDMA remains unclarified. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.freeradbiomed.2019.02.013DOI Listing
February 2019

Intrachiasmatic craniopharyngioma: Assessment of visual outcome with optical coherence tomography after complete surgical removal.

Surg Neurol Int 2019 21;10. Epub 2019 Jan 21.

Department of Neurosurgery, La Princesa University Hospital, Madrid, Spain.

Background: Optic chiasm invasion by a craniopharyngioma (CP) is exceptional. Surgical treatment of intrachiasmatic CPs associates a high risk of chiasm injury, which should be properly addressed before surgery.

Case Description: We present a 46-year-old woman admitted to the hospital with low visual acuity (0. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.4103/sni.sni_292_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6357539PMC
January 2019

Oxidative stress induced by fluoroquinolone enrofloxacin in zebrafish (Danio rerio) can be ameliorated after a prolonged exposure.

Environ Toxicol Pharmacol 2019 Feb 6;67:87-93. Epub 2019 Feb 6.

Department of Chemical, Biological, Pharmaceutical and Environmental Sciences, University of Messina, Viale Ferdinando Stagno d'Alcontres, 31, 98166, Agata-Messina, Italy. Electronic address:

The purpose of our experiment was to evaluate the effect of enrofloxacin on biotransformation, oxidative stress and mRNA expression of related genes in fish as a non-target organisms. Zebrafish (Danio rerio) juveniles were treated with enrofloxacin at concentrations of 5, 10 and 500 μg/L for 14 days. A three-day-long test caused changes of catalytic activity of glutathione peroxidase and glutathione-S-transferase. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.etap.2019.02.002DOI Listing
February 2019
1 Read

Occipital tip injury with homonymous central scotoma: OCT-NFL and RGC correlation.

Am J Ophthalmol Case Rep 2019 Jun 26;14:10-13. Epub 2019 Jan 26.

Department of Ophthalmology and Visual Sciences, Montefiore Medical Center, Albert Einstein School of Medicine, 3400 Bainbridge Avenue, Bronx, NY, United States.

Purpose: To report one case of homonymous paracentral scotoma with corresponding optical coherence tomography (OCT) findings in a young woman after injury to the left occipital lobe tip.

Observations: A young woman with past medical history of Hodgkin's lymphoma and large B-cell lymphoma metastatic to the left occipital lobe status post resection presented to the eye clinic with a chief complaint of a "blind spot" in the right eye since her surgery. Humphrey visual field (HVF) showed a right homonymous paracentral scotoma corresponding to the non-decussating and decussating optic radiation for central vision originating from the left lateral geniculate nucleus (LGN). Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ajoc.2019.01.008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6360247PMC

Rare Case of Sellar and Suprasellar Metastasis from Ewing's Sarcoma of Tibia.

J Neurosci Rural Pract 2019 Jan-Mar;10(1):158-161

Department of Neurosurgery, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India.

We hereby report a case of metastatic Ewing's sarcoma presenting with rapid-onset total ophthalmoplegia, optic atrophy, and right temporal hemianopia. Comprehensive ophthalmic and neurological evaluation with targeted radioimaging revealed a tumor mass centered over the sella, compressing optic chiasma, extending to involve the left cavernous sinus and the left orbital apex. Whole-body imaging revealed the evidence of multifocal lung and mediastinal metastasis with focal lytic defect in the left femoral head. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.4103/jnrp.jnrp_139_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6338001PMC
February 2019

Diagnostic Utility of Optic Nerve Measurements with MRI in Patients with Optic Nerve Atrophy.

AJNR Am J Neuroradiol 2019 Feb 14. Epub 2019 Feb 14.

From the Departments of Radiology (B.Z., M.E., A.B., Y.-M.C, R.A.B.).

Background And Purpose: No MR imaging measurement criteria are available for the diagnosis of optic nerve atrophy. We determined a threshold optic nerve area on MR imaging that predicts a clinical diagnosis of optic nerve atrophy and assessed the relationship between optic nerve area and retinal nerve fiber layer thickness measured by optical coherence tomography, an ancillary test used to evaluate optic nerve disorders.

Materials And Methods: We evaluated 26 patients with suspected optic nerve atrophy (8 with unilateral, 13 with bilateral and 5 with suspected but not demonstrable optic nerve atrophy) who had both orbital MR imaging and optical coherence tomography examinations. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.3174/ajnr.A5975DOI Listing
February 2019

[Optic Disc Changes in Childhood].

Klin Monbl Augenheilkd 2019 Feb 14;236(2):203-218. Epub 2019 Feb 14.

The ophthalmological inspection of the optic disc is possible since the time of birth. Thereby, changes of normal optic disc configuration can already be detected in early infancy. Congenital optic disc anomalies can go along with congenital malformations of the brain or with syndromal systemic diseases. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1055/a-0818-1790DOI Listing
February 2019

[Impairment of the Visual System in Glaucoma].

Klin Monbl Augenheilkd 2019 Feb 14;236(2):134-141. Epub 2019 Feb 14.

Interdisziplinäres Zentrum für Präventive Augenheilkunde und Imaging, Augenklinik, Universitätsklinikum Erlangen.

MR Diffusion Tensor Imaging (DTI) enables visualisation of the visual system in glaucoma; it has been shown that the atrophy of the optic radiation is more pronounced in glaucoma than in age-matched controls. The atrophy of the optic radiation thereby correlates with OCT and visual field measures. Diffusion tensor imaging permits quantification of the axonal integrity of the optic radiation by calculation of fractional anisotropy (FA) and radial diffusivity (RD). Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1055/a-0762-0822DOI Listing
February 2019

[Ocular Phenotype and Complications in Patients with Tuberous Sclerosis Complex (TSC)].

Klin Monbl Augenheilkd 2019 Feb 14. Epub 2019 Feb 14.

Augenklinik, UniversitätsSpital Zürich, Schweiz.

Background: Evaluation and comparison of the ocular phenotype in patients diagnosed with tuberous sclerosis complex (TSC). Analysis of ocular complications during follow-up.

Patients And Methods: A retrospective chart review was performed of patients with TSC who had received an ocular examination at the eye clinic of the University Hospital Zurich. Read More

View Article

Download full-text PDF

Source
http://www.thieme-connect.de/DOI/DOI?10.1055/a-0804-1816
Publisher Site
http://dx.doi.org/10.1055/a-0804-1816DOI Listing
February 2019
2 Reads

Endoscopic Endonasal Removal of Primary/Recurrent Meningiomas in the Medial Optic Canal: Surgical Technique and Long-Term Visual Outcome.

Oper Neurosurg (Hagerstown) 2019 Feb 7. Epub 2019 Feb 7.

Department of Neurosurgery, Kurume University School of Medicine, Fukuoka, Japan.

Background: Tuberculum sellae meningiomas frequently extend into the optic canals, which leads to a progressive longitudinal visual loss. Therefore, in addition to tumor removal, unroofing and exploration inside the optic canal are important procedures.

Objective: To perform endoscopic endonasal tumor removal with optic canal decompression for small primary or recurrent meningiomas associated with a progressive visual loss at the inferior-medial optic canal, which corresponded to a blind corner in the ipsilateral pterional/subfrontal approach. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1093/ons/opz001DOI Listing
February 2019

Mitochondrial Morphology, Dynamics, and Function in Human Pressure Overload or Ischemic Heart Disease With Preserved or Reduced Ejection Fraction.

Circ Heart Fail 2019 Feb;12(2):e005131

Department of Cardiovascular Diseases, (A.H.C., M.M.R.), Mayo Clinic, Rochester, MN.

Background: The FOXO3a (forkhead box O3a)-BNIP3 (B-cell lymphoma 2/adenovirus E1B 19kDa interacting protein 3) pathway modulates mitochondrial dynamics and function and contributes to myocardial remodeling in rodent models of heart failure. We sought to investigate the expression of this pathway along with the expression of mitochondrial biogenesis (PGC-1α [peroxisome proliferator-activated receptor-γ coactivator-1α]), dynamics (DRP-1 [dynamin-related protein 1], OPA-1 [optic atrophy 1], and MFN 2 [mitofusin 2]), and oxidative phosphorylation (citrate synthase and electron transport chain complexes) markers and COX IV (cytochrome C oxidase) activity in myocardium from patients with valvular or ischemic heart disease and heart failure with preserved ejection fraction (HFpEF) or heart failure with reduced ejection fraction (HFrEF).

Methods And Results: Subepicardial left ventricular biopsies (10×1×1 mm) were obtained at aortic valve replacement (HFpEF, n=5; and HFrEF, n=4), coronary artery bypass grafting (HFpEF, n=5; and HFrEF, n=5), or left ventricular assist device implantation (HFrEF, n=4). Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1161/CIRCHEARTFAILURE.118.005131DOI Listing
February 2019
1 Read

A Novel Mutation of in a Child with 22q11.2 Deletion Syndrome Associated with MCLMR.

Mol Syndromol 2019 Jan 20;9(5):266-270. Epub 2018 Jul 20.

Department of Pediatric Genetics, Cerrahpaşa Medical Faculty, Istanbul University, Istanbul, Turkey.

Microcephaly with or without chorioretinopathy, lymphedema, or mental retardation (MCLMR; OMIM 152950) is a rare autosomal dominantly inherited syndrome. Mutations in the kinesin family member 11 () gene have been associated with this condition. Here, we report a de novo novel heterozygous missense mutation in exon 12 of the gene [c. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1159/000491568DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6362857PMC
January 2019
1 Read

Posttraumatic Emphysema of the Optic Nerve Sheath.

Ophthalmic Plast Reconstr Surg 2019 Jan 25. Epub 2019 Jan 25.

Department of Ophthalmology, New York University School of Medicine, New York, New York, U.S.A.

The authors describe the case of a 19-year-old female who suffered posttraumatic emphysema of the optic nerve sheath. She suffered massive head trauma requiring emergent neurosurgery and was incidentally found to have air in her optic nerve sheath on CT scan. At 6 weeks follow up, her visual acuity (20/25 uncorrected) and color perception in the affected eye were excellent. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1097/IOP.0000000000001312DOI Listing
January 2019
3 Reads

Lattice corneal dystrophy with familial amyloid polyneuropathy.

Int J Clin Pract 2019 Feb 6:e13320. Epub 2019 Feb 6.

Department of Ophthalmology, Osaka University Graduate School of Medicine, Osaka, Japan.

A 77-year-old Japanese female diagnosed with familial amyloid polyneuropathy type IV (FAP-IV) was referred for an ophthalmologic evaluation. Her general appearance showed a mask-like face because of facial nerve paralysis (Figure 1) and she exhibited tongue atrophy caused by hypoglossal nerve palsy (Figure 2). Corrected distance visual acuity was 6/9. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/ijcp.13320DOI Listing
February 2019

Long-term outcomes of cerebral venous sinus stenosis corrected by stenting

Curr Neurovasc Res 2019 02 6. Epub 2019 Feb 6.

Departments of Neurosurgery, Xuanwu Hospital, Capital Medical University, Beijing, China

Background And Purpose: Cerebral venous sinus stenosis (CVSS) usually results in severe intracranial hypertension (IH), which can be corrected by stenting immediately. However, there is a lack of evidence of the long-term good outcomes in patients with CVSS who underwent stenting.

Methods: A total of 62 patients with imaging confirmed non-thrombotic and non-external compression CVSS were enrolled into this single center real-world cohort study after undergoing stenting, and were continuously followed up for more than 12 years. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.2174/1567202616666190206185133DOI Listing
February 2019
1 Read

A Time Bomb Defused, In Time! A Traumatic Optic Neuropathy To Be Wary Of.

Neuroophthalmology 2019 Feb 13;43(1):32-35. Epub 2018 Jun 13.

Department of Radiology and Imaging Services, Senior Interventional Radiologist Apollo Hospitals, Chennai, India.

Authors describe a case of traumatic pseudoaneurysm of cavernous internal carotid artery in a young male who presented with complaints of progressive decrease in vision, inability to move the eye, and delayed epistaxis 8 months after the head injury sustained during a motor vehicle accident. The presence of optic atrophy and epistaxis in the setting of head trauma alerted the authors to look for this potentially life-threatening condition. Timely intervention in the form of endovascular coil occlusion of the internal carotid artery was performed and the epistaxis stopped without any ischaemic or thromboembolic sequaelae. Read More

View Article

Download full-text PDF

Source
https://www.tandfonline.com/doi/full/10.1080/01658107.2018.1
Publisher Site
http://dx.doi.org/10.1080/01658107.2018.1458886DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6351014PMC
February 2019
2 Reads

Ophthalmological manifestation in patients of tuberculous meningitis.

QJM 2019 Feb 5. Epub 2019 Feb 5.

Professor, Department of Ophthalmology, King George's Medical University, Lucknow, India.

Background: Vision impairment, blindness in particular is a devastating complication in patients with Tubercular meningitis. However information regarding ophthalmological manifestation and its impact on vision is sparse in the literature. This study evaluated the spectrum of ophthalmological manifestations in tuberculous meningitis, including retinal nerve fiber layer thickness assessment by optic coherence tomography and its correlation with visual and clinical outcome. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1093/qjmed/hcz037DOI Listing
February 2019
2 Reads

Response to: Comment on "Transorbital ultrasonography for measuring optic nerve atrophy in multiple sclerosis".

Acta Neurol Scand 2019 Feb 5. Epub 2019 Feb 5.

Emergency and Urgency Care Service, Águilas, Murcia, Spain.

We thank the authors for the comments on our recent work (1) and we would offer some clarification. The blooming effect is a doppler imaging artifact (2). In our work the doppler mode has not been used, so it is not possible that the blooming effect could artifact the images. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/ane.13076DOI Listing
February 2019
1 Read

Papillitis and neuroretinitis of tuberculous etiology.

Arch Soc Esp Oftalmol 2019 Feb 1. Epub 2019 Feb 1.

Servicio de Medicina Interna, Hospital Universitario 12 de Octubre, Madrid, España.

A 57-year-old man with miliary tuberculosis reported visual loss in his right eye, a month after starting a four-drug antituberculous treatment regimen. On exploration, an inferior segmental optic disc edema was objectived and it was attributed to ischemic aetiology. Ethambutol was withdrawn and 60mg of oral prednisone daily were given with a tapering dosage. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.oftal.2018.12.010DOI Listing
February 2019
1 Read

Huangqi-Danshen Decoction Ameliorates Adenine-Induced Chronic Kidney Disease by Modulating Mitochondrial Dynamics.

Evid Based Complement Alternat Med 2019 1;2019:9574045. Epub 2019 Jan 1.

Department of Nephrology, Shenzhen Traditional Chinese Medicine Hospital, Guangzhou University of Chinese Medicine, Shenzhen, Guangdong, China.

Chronic kidney disease (CKD) is a leading public health problem with high morbidity and mortality. However, the therapies remain limited. Traditional Chinese medicine (TCM) has been used for treating kidney disease for thousands of years and is an effective alternative treatment for CKD patients in China and other Asian countries. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1155/2019/9574045DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6332985PMC
January 2019

Manual Acupuncture for Optic Atrophy: A Systematic Review and Meta-Analysis.

Evid Based Complement Alternat Med 2019 1;2019:1735967. Epub 2019 Jan 1.

Shanghai Research Institute of Acupuncture and Meridian, Shanghai 200030, China.

This systematic review aims to critically evaluate the efficacy of manual acupuncture for optic atrophy. Eight English and Chinese databases, including Cochrane Library, EMbase, PubMed, Chinese National Knowledge Infrastructure (CNKI), Wanfang Database, China Science and Technology Journal Database (VIP), and Chinese Biomedical Literature Database (CBM), as well as ongoing trials registered with the WHO International Clinical Trials Registry Platform, were searched to identify eligible randomized controlled trials (RCTs) studying manual acupuncture for optic atrophy compared to medication alone. The quality of evidence was assessed using Cochrane Collaboration's risk of bias tool. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1155/2019/1735967DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6332962PMC
January 2019
1 Read

Visual Outcome of Patients with Pituitary Adenomas Following Surgery and Its Contributory Factors at a Tertiary Hospital in Ghana.

Ethiop J Health Sci 2019 Jan;29(1):895-902

Department of Community Dentistry, School of Medicine and Dentistry, College of Health Sciences, University of Ghana, Accra, Ghana.

Background: Craniotomy and transphenoidal microsurgery are surgical options for treatment of pituitary adenoma at Korle Bu Teaching Hospital(KBTH). Despite major advances and reported success rates of transphenoidal resection globally, paucity of local data regarding visual outcome of either procedure exists. We evaluated the visual outcome of patient with pituitary adenoma following surgery in a tertiary hospital in Ghana. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.4314/ejhs.v29i1.11DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6341437PMC
January 2019

Nrf2 deficiency exacerbates frailty and sarcopenia by impairing skeletal muscle mitochondrial biogenesis and dynamics in an age-dependent manner.

Exp Gerontol 2019 Jan 25;119:61-73. Epub 2019 Jan 25.

Department of Gastrointestinal Surgery, Shanghai Tenth People's Hospital, School of Medicine, Tongji University, Shanghai, China; Shanghai Tenth People's Hospital Chongming Branch Affiliated to Tongji University, Shanghai, China. Electronic address:

Aim: Mitochondrial dysfunction during aging is a key factor that contributes to sarcopenia. Nuclear factor erythroid 2-related factor 2 (Nrf2) has been increasingly recognized to regulate mitochondrial function. The present study aimed to investigate the role of Nrf2 in the development of frailty and sarcopenia during aging, and to demonstrate whether Nrf2 contributes to the maintenance of muscle mass and function by regulation of mitochondrial biogenesis and dynamics during the aging process. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.exger.2019.01.022DOI Listing
January 2019

Clinical, radiological, and genetic characteristics of 16 patients with ACO2 gene defects: Delineation of an emerging neurometabolic syndrome.

J Inherit Metab Dis 2018 Dec 27. Epub 2018 Dec 27.

Rappaport Faculty of Medicine, Technion- Israel Institute of Technology, Haifa, Israel.

Mitochondrial aconitase is the second enzyme in the tricarboxylic acid (TCA) cycle catalyzing the interconversion of citrate into isocitrate and encoded by the nuclear gene ACO2. A homozygous pathogenic variant in the ACO2 gene was initially described in 2012 resulting in a novel disorder termed "infantile cerebellar retinal degeneration" (ICRD, OMIM#614559). Subsequently, additional studies reported patients with pathogenic ACO2 variants, further expanding the genetic and clinical spectrum of this disorder to include milder and later onset manifestations. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/jimd.12022DOI Listing
December 2018
3.365 Impact Factor

Visual Function in Mice Lacking GM3 Synthase.

Curr Eye Res 2019 Jan 27:1-7. Epub 2019 Jan 27.

a Department of Ophthalmology , School of Medicine, Sapporo Medical University , Sapporo , Japan.

Purpose: Most complex gangliosides in vertebrates are formed from ganglioside GM3. GM3 deficiency in humans can result in epilepsy and visual impairment. To investigate whether a deficiency of GM3 is involved in visual function, ST3GAL5 mice with mutations in the ST3GAL5 gene-coded GM3 synthase were employed. Read More

View Article

Download full-text PDF

Source
https://www.tandfonline.com/doi/full/10.1080/02713683.2019.1
Publisher Site
http://dx.doi.org/10.1080/02713683.2019.1576206DOI Listing
January 2019
2 Reads

Preliminary Evaluation of a Mobile Device for Dark Adaptation Measurement.

Transl Vis Sci Technol 2019 Jan 22;8(1):11. Epub 2019 Jan 22.

Schepens Eye Research Institute of Mass Eye & Ear, Harvard Medical School, Boston, MA, USA.

Purpose: We evaluated the feasibility of a smartphone application-based dark adaptation (DA) measurement method (MOBILE-DA).

Methods: On a Samsung Galaxy S8 smartphone, MOBILE-DA presented a 1.5° flashing stimulus (wavelength = 453 nm) between -1. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1167/tvst.8.1.11DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6343678PMC
January 2019

Withaferin A-mediated apoptosis in breast cancer cells is associated with alterations in mitochondrial dynamics.

Mitochondrion 2019 Jan 24. Epub 2019 Jan 24.

Department of Pharmacology & Chemical Biology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA; UPMC Hillman Cancer Center, Pittsburgh, PA, USA. Electronic address:

Withaferin A (WA), a steroidal lactone derived from a medicinal plant (Withania somnifera), inhibits cancer development in transgenic and chemically-induced rodent models of breast cancer but the underlying mechanism is not fully grasped. We have shown previously that WA treatment causes apoptotic cell death in human breast cancer cells that is preceded by inhibition of complex III of the mitochondrial electron transport chain. This study extends these observations to now demonstrate alterations in mitochondrial dynamics in WA-induced apoptosis. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.mito.2019.01.003DOI Listing
January 2019
1 Read
3.249 Impact Factor

A yeast-based screening assay identifies repurposed drugs that suppress mitochondrial fusion and mtDNA maintenance defects.

Dis Model Mech 2019 Feb 7;12(2). Epub 2019 Feb 7.

Research Center on Animal Cognition (CRCA) and Center of Developmental Biology (CBD), Center for Integrative Biology (CBI), Toulouse University, CNRS, UPS, 118 route de Narbonne, 31062 Toulouse, France.

Mitochondria continually move, fuse and divide, and these dynamics are essential for the proper function of the organelles. Indeed, the dynamic balance of fusion and fission of mitochondria determines their morphology and allows their immediate adaptation to energetic needs as well as preserving their integrity. As a consequence, mitochondrial fusion and fission dynamics and the proteins that control these processes, which are conserved from yeast to human, are essential, and their disturbances are associated with severe human disorders, including neurodegenerative diseases. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1242/dmm.036558DOI Listing
February 2019
1 Read

The Oculome Panel Test: Next-Generation Sequencing to Diagnose a Diverse Range of Genetic Developmental Eye Disorders.

Ophthalmology 2019 Jan 14. Epub 2019 Jan 14.

UCL Great Ormond Street Institute of Child Health, and NIHR Biomedical Research Centre at Great Ormond Street Hospital for Children NHS Foundation Trust and University College London, London, United Kingdom. Electronic address:

Purpose: To develop a comprehensive next-generation sequencing panel assay that screens genes known to cause developmental eye disorders and inherited eye disease and to evaluate its diagnostic yield in a pediatric cohort with malformations of the globe, anterior segment anomalies, childhood glaucoma, or a combination thereof.

Design: Evaluation of diagnostic test.

Participants: Two hundred seventy-seven children, 0 to 16 years of age, diagnosed with nonsyndromic or syndromic developmental eye defects without a genetic diagnosis. Read More

View Article

Download full-text PDF

Source
https://linkinghub.elsevier.com/retrieve/pii/S01616420183185
Publisher Site
http://dx.doi.org/10.1016/j.ophtha.2018.12.050DOI Listing
January 2019
7 Reads

Optical Coherence Tomography of the Retinal Ganglion Cell Complex in Leber's Hereditary Optic Neuropathy and Dominant Optic Atrophy.

Curr Eye Res 2019 Jan 16:1-7. Epub 2019 Jan 16.

a Department of Ophthalmology , Doheny Eye Center , Los Angeles , CA , USA.

Background: Mitochondrial optic neuropathies such as Leber's Hereditary Optic Neuropathy (LHON) and Dominant Optic Atrophy (DOA) have been shown to produce an optic neuropathy secondary to retinal ganglion cell loss with thinning of the retinal ganglion cell complex (RGCC).

Methods: We performed a retrospective analysis assessing the thicknesses of the peripapillary retinal nerve fiber layer (pRNFL) along with the macular retinal ganglion cell-inner plexiform layer (RGC-IPL) using optical coherence tomography (OCT). We compared these changes among acute and chronic LHON, DOA, and normal healthy control patients. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1080/02713683.2019.1567792DOI Listing
January 2019
4 Reads

Exogenous Hydrogen Sulfide Supplement Attenuates Isoproterenol-Induced Myocardial Hypertrophy in a Sirtuin 3-Dependent Manner.

Oxid Med Cell Longev 2018 17;2018:9396089. Epub 2018 Dec 17.

Department of Pharmacology, School of Pharmacy, Nantong University, Key Laboratory of Inflammation and Molecular Drug Target of Jiangsu Province, Nantong, 226001 Jiangsu, China.

Hydrogen sulfide (HS) is a gasotransmitter with a variety of cardiovascular protective effects. Sirtuin 3 (SIRT3) is closely related to mitochondrial function and oxidative stress. We found that NaHS increased SIRT3 expression in the preventive effect on isoproterenol- (ISO-) induced myocardial hypertrophy. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1155/2018/9396089DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6311776PMC
February 2019
1 Read

Medical interventions for traumatic hyphema.

Cochrane Database Syst Rev 2019 01 14;1:CD005431. Epub 2019 Jan 14.

Department of Special Surgery-Ophthalmology, Faculty of Medicine, The University of Jordan, P.O. Box 13046, Amman, Jordan.

Background: Traumatic hyphema is the entry of blood into the anterior chamber (the space between the cornea and iris) subsequent to a blow or a projectile striking the eye. Hyphema uncommonly causes permanent loss of vision. Associated trauma (e. Read More

View Article

Download full-text PDF

Source
http://doi.wiley.com/10.1002/14651858.CD005431.pub4
Publisher Site
http://dx.doi.org/10.1002/14651858.CD005431.pub4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6353164PMC
January 2019
5 Reads

Imaging in myopia: potential biomarkers, current challenges and future developments.

Br J Ophthalmol 2019 Jan 12. Epub 2019 Jan 12.

Singapore National Eye Centre, Singapore, Singapore.

Myopia is rapidly increasing in Asia and around the world, while it is recognised that complications from high myopia may cause significant visual impairment. Thus, imaging the myopic eye is important for the diagnosis of sight-threatening complications, monitoring of disease progression and evaluation of treatments. For example, recent advances in high-resolution imaging using optical coherence tomography may delineate early myopic macula pathology, optical coherence tomography angiography may aid early choroidal neovascularisation detection, while multimodal imaging is important for monitoring treatment response. Read More

View Article

Download full-text PDF

Source
http://bjo.bmj.com/lookup/doi/10.1136/bjophthalmol-2018-3128
Publisher Site
http://dx.doi.org/10.1136/bjophthalmol-2018-312866DOI Listing
January 2019
8 Reads
2.976 Impact Factor

Phenotype prediction of Mohr-Tranebjaerg syndrome (MTS) by genetic analysis and initial auditory neuropathy.

BMC Med Genet 2019 Jan 11;20(1):11. Epub 2019 Jan 11.

Institute of Otolaryngology, Chinese PLA General Hospital, Medical School of Chinese PLA, Beijing, 100853, China.

Background: Mohr-Tranebjaerg syndrome (MTS) is a rare X-linked recessive neurodegenerative disorder resulting in early-onset hearing impairment, gradual dystonia and optic atrophy. MTS is caused by variations in the nuclear TIMM8A gene, which is involved in mitochondrial transport of metabolites. This study aimed to identify the pathogenic gene variations in three Chinese families associated with predicted MTS with or without X-linked agammaglobulinaemia. Read More

View Article

Download full-text PDF

Source
https://bmcmedgenet.biomedcentral.com/articles/10.1186/s1288
Publisher Site
http://dx.doi.org/10.1186/s12881-018-0741-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6330410PMC
January 2019
6 Reads
2.083 Impact Factor

The Relationship Between Interocular Asymmetry of Visual Field Defects and Optic Nerve Head Blood Flow in Patients with Glaucoma.

J Glaucoma 2018 Jan 7. Epub 2018 Jan 7.

Department of Ophthalmology, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan.

Purpose: To investigate the association between asymmetry of visual field defects and optic nerve head blood flow in patients with glaucoma using laser speckle flowgraphy.

Methods: 170 eyes of 85 patients with primary open angle glaucoma (POAG) were included. Intraocular pressure, visual fields (VF, Humphrey 24-2, SITA program), mean blur rate in the tissue area of the optic nerve head (MBR-T) measured by laser speckle flowgraphy, axial length, circumpapillary retinal nerve fiber layer thickness (cpRNFLT), disc area, cup/disc area ratio, and parapapillary atrophy area (PPA) was measured in each eye. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1097/IJG.0000000000001181DOI Listing
January 2018

Novel neuroblastoma amplified sequence () mutations in a Japanese boy with fever-triggered recurrent acute liver failure.

Hum Genome Var 2019 7;6. Epub 2019 Jan 7.

1Department of Pediatrics, Kawasaki Medical School, Okayama, 701-0192 Japan.

Biallelic mutations in the neuroblastoma amplified sequence () gene have been reported to cause two different clinical spectra: short stature with optic nerve atrophy and Pelger-Huët anomaly (SOPH) syndrome and infantile liver failure syndrome 2 (ILFS2). Here, we describe a case of a 3-year-old Japanese boy who presented with fever-triggered recurrent acute liver failure (ALF). The clinical characteristics were considerable elevation of liver enzymes, severe coagulopathy, and acute renal failure. Read More

View Article

Download full-text PDF

Source
http://www.nature.com/articles/s41439-018-0035-5
Publisher Site
http://dx.doi.org/10.1038/s41439-018-0035-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6323122PMC
January 2019
7 Reads

Optic nerve thinning and neurosensory retinal degeneration in the rTg4510 mouse model of frontotemporal dementia.

Acta Neuropathol Commun 2019 Jan 7;7(1). Epub 2019 Jan 7.

UCL Institute of Ophthalmology, University College London, 11-43 Bath Street, London, EC1V 9EL, UK.

Visual impairments, such as difficulties in reading and finding objects, perceiving depth and structure from motion, and impaired stereopsis, have been reported in tauopathy disorders, such as frontotemporal dementia (FTD). These impairments however have been previously attributed to cortical pathologies rather than changes in the neurosensory retina or the optic nerve. Here, we examined tau pathology in the neurosensory retina of the rTg(tauP301L)4510 mouse model of FTD. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1186/s40478-018-0654-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6322294PMC
January 2019

Orbital apex venous cavernous malformation with optic neuropathy: treatment with multisession gamma knife radiosurgery.

Br J Ophthalmol 2019 Jan 5. Epub 2019 Jan 5.

Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea

Purpose: To evaluate the efficacy and safety of multisession gamma knife radiosurgery (GKRS) for orbital apex venous cavernous malformation causing optic neuropathy.

Methods: Retrospective cohort study in a single tertiary institution from January 2007 to December 2016 on patients who underwent multisession GKRS for orbital apex venous cavernous malformations causing optic neuropathy.

Results: There were 12 patients included in our study. Read More

View Article

Download full-text PDF

Source
http://bjo.bmj.com/lookup/doi/10.1136/bjophthalmol-2018-3128
Publisher Site
http://dx.doi.org/10.1136/bjophthalmol-2018-312893DOI Listing
January 2019
6 Reads

Postoperative infection following strabismus surgery: case series and increased incidence in a single referral center.

J AAPOS 2019 Jan 3. Epub 2019 Jan 3.

Department of Ophthalmology, Duke University Medical Center, Durham, North Carolina. Electronic address:

Purpose: To identify and analyze cases of postoperative infection following strabismus surgery at a large referral center and to report the incidence, risk factors, and outcomes.

Methods: An electronic database search identified strabismus procedures at Duke Eye Center from July 1996 to October 2017. Diagnosis codes for periocular infections were used to further identify patients with possible infections following strabismus surgery. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jaapos.2018.09.007DOI Listing
January 2019
2 Reads

Mitochondrial ribosomal protein PTCD3 mutations cause oxidative phosphorylation defects with Leigh syndrome.

Neurogenetics 2019 Jan 3. Epub 2019 Jan 3.

Diagnostics and Therapeutics of Intractable Diseases, Intractable Disease Research Center, Graduate School of Medicine, Juntendo University, Hongo 2-1-1, Bunkyo-ku, Tokyo, 113-8421, Japan.

Pentatricopeptide repeat domain proteins are a large family of RNA-binding proteins involved in mitochondrial RNA editing, stability, and translation. Mitochondrial translation machinery defects are an expanding group of genetic diseases in humans. We describe a patient who presented with low birth weight, mental retardation, and optic atrophy. Read More

View Article

Download full-text PDF

Source
http://link.springer.com/10.1007/s10048-018-0561-9
Publisher Site
http://dx.doi.org/10.1007/s10048-018-0561-9DOI Listing
January 2019
8 Reads

Development of Retinal Infarct Due to Intracameral Cefuroxime Injection Following Complicated Cataract Surgery

Turk J Ophthalmol 2018 Dec;48(6):317-319

Muğla Sıtkı Koçman University Faculty of Medicine, Department of Ophthalmology, Muğla, Turkey

We present the case of a 60-year-old patient who underwent a complicated cataract surgery with cefuroxime injection (1 mg/0.1 mL) into the anterior chamber at the end of surgery. The patient presented to our hospital due to decrease in visual acuity (VA) after surgery. Read More

View Article

Download full-text PDF

Source
http://cms.galenos.com.tr/Uploads/Article_21070/TJO-48-317-E
Publisher Site
http://dx.doi.org/10.4274/tjo.61580DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6330669PMC
December 2018
8 Reads

The relationship between optic nerve head deformation and visual field defects in myopic eyes with primary open-angle glaucoma.

PLoS One 2018 31;13(12):e0209755. Epub 2018 Dec 31.

Department of Ophthalmology, Cathay General Hospital, Taipei, Taiwan.

Purpose: To investigate the relationship between the morphologic features of myopic optic nerve head (ONH) and visual field (VF) defects in myopic subjects with primary open-angle glaucoma (POAG) by intraindividual comparison.

Methods: Myopic POAG subjects with unilateral glaucomatous VF defect were recruited. The morphologic features of myopic ONH, including optic disc tilt, optic disc rotation, and β-zone parapapillary atrophy (PPA) were measured from color fundus photographs. Read More

View Article

Download full-text PDF

Source
http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0209755PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6312218PMC
December 2018

SOPH Syndrome with Growth Hormone Deficiency, Normal Bone Age, and Novel Compound Heterozygous Mutations in NBAS.

Fetal Pediatr Pathol 2018 Dec 28:1-7. Epub 2018 Dec 28.

a Department of Endocrinology, Shanghai Children's Medical Center , Shanghai Jiaotong University School of Medicine , Shanghai , China.

Background: Short stature with optic atrophy and Pelger-Huet anomaly (SOPH; MIM 614800) syndrome is a genetic disease caused by mutation in the neuroblastoma amplified sequence (NBAS) gene.

Case Report: We present a 11-year-old Chinese boy with SOPH syndrome, growth hormone deficiency with a normal bone age. Gene sequencing in the patient revealed a novel compound heterozygous mutation of c. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1080/15513815.2018.1509406DOI Listing
December 2018
2 Reads

Neurological Complications of Congenital Zika Virus Infection.

Pediatr Neurol 2019 Feb 22;91:3-10. Epub 2018 Nov 22.

Department of Pediatrics, School of Medicine, Universidade Federal de Goiás - UFG, Goiânia, GO, Brazil. Electronic address:

Background: In utero Zika virus infection resulted in many newborns with congenital defects; this public health issue was followed by unprecedented scientific productivity in this field. Many questions remain about congenital Zika virus infection and its maternal transmission, pathogenesis, clinical events, and the resulting neurological damage. There are few review articles that synthesize the current knowledge of congenital neurological complications as well as the gaps in the pediatric literature. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.pediatrneurol.2018.11.003DOI Listing
February 2019
1 Read

Subclinical Leber's hereditary optic neuropathy with pediatric acute spinal cord onset: more than meets the eye.

BMC Neurol 2018 Dec 27;18(1):220. Epub 2018 Dec 27.

Department of Pathophysiology and Transplantation, Dino Ferrari Centre, University of Milan, Milan, Italy.

Background: Leber's hereditary optic neuropathy (LHON) is a mitochondrial disease characterized by visual loss consequent to optic nerve atrophy. In some cases, LHON is associated with heterogeneous neurological extraocular manifestations and is referred to as "Leber plus disease"; rarely it is associated with a multiple sclerosis (MS)-like syndrome known as Harding disease, but no pediatric extraocular acute spinal onset is reported.

Case Presentation: We describe the case of a 5-year-old girl carrying the G3460A mtDNA mutation who was referred to clinical examination for bilateral upper and lower limb weakness with no sign of optic neuropathy. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1186/s12883-018-1227-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6307307PMC
December 2018
10 Reads

Clinical and Biochemical Features in a Patient With Gene Alteration.

Front Genet 2018 7;9:625. Epub 2018 Dec 7.

Unit of Child Neurology and Psychiatry, Department of Human Neuroscience, Sapienza University of Rome, Rome, Italy.

Mitochondrial Fission Factor (MFF) is part of a protein complex that promotes mitochondria and peroxisome fission. Hitherto, only 5 patients have been reported harboring mutations in , all of them with the clinical features of a very early onset Leigh-like encephalopathy. We report on an 11-year-old boy with epileptic encephalopathy. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.3389/fgene.2018.00625DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6292958PMC
December 2018
1 Read

Diffuse Unilateral Subacute Neuroretinitis (DUSN).

Adv Exp Med Biol 2018 ;1085:239-241

Department of Ophthalmology, Columbia University, Edward S. Harkness Eye Institute, NewYork-Presbyterian Hospital, New York, NY, USA.

Diffuse unilateral subacute neuroretinitis (DUSN) is caused by a subretinal live and mobile nematode. Acute phase: Patients usually present with severe pain, decreased vision, vitritis/papillitis, and tracks of grayish-white lesions-and a live nematode. Late phase: Arterial narrowing, optic atrophy, diffuse disruption of the retinal pigment epithelium (RPE), with severe visual loss. Read More

View Article

Download full-text PDF

Source
http://link.springer.com/10.1007/978-3-319-95046-4_50
Publisher Site
http://dx.doi.org/10.1007/978-3-319-95046-4_50DOI Listing
January 2018
9 Reads