An ultrastructural study of the retina in the Jansky-Bielschowsky type of neuronal ceroid-lipofuscinosis.

Abstract:

:A 7 1/2-year-old boy died of the Jansky-Bielschowsky type of neuronal ceroidlipofuscinosis. Although an electroretinogram showed reduced activity, pigmentary retinal atrophy was not demonstrable by ophthalmoscopy two years before death. At autopsy, the retina had severe loss of photoreceptor cells, pigment displacement, gliosis, and uniformly structured lipopigment bodies with an internal structure of curvilinear profiles in ganglionic and Müller cells, in pigment epithelia, in the remaining photoreceptors, and in the elements of the inner nuclear layer. These findings conformed to those in the juvenile type of neuronal ceroid-lipofuscinosis, differing only as to the severity.

journal_name

Am J Ophthalmol

authors

Goebel HH,Zeman W,Damaske E

doi

10.1016/0002-9394(77)90194-5

subject

Has Abstract

pub_date

1977-01-01 00:00:00

pages

70-9

issue

1

eissn

0002-9394

issn

1879-1891

pii

0002-9394(77)90194-5

journal_volume

83

pub_type

杂志文章
  • Hypotony caused by scleral buckle erosion in Marfan syndrome.

    abstract:PURPOSE:To describe hypotony caused by erosion of the conjunctiva and sclera by a silicone scleral buckle. METHODS:Interventional case report. A 33-year-old man with Marfan syndrome presented with hypotony maculopathy and a collapsed globe 17 months after repair of retinal detachment with a silicone sponge and silicon...

    journal_title:American journal of ophthalmology

    pub_type: 杂志文章

    doi:10.1016/s0002-9394(01)00993-x

    authors: Deramo VA,Haupert CL,Fekrat S,Postel EA

    更新日期:2001-09-01 00:00:00

  • Ocular abnormalities in the Freeman-Sheldon syndrome.

    abstract::Ocular abnormalities were present in five of 11 patients with Freeman-Sheldon syndrome. The predominant ocular findings were strabismus and blepharoptosis. Stiffness of the extraocular muscles, demonstrated in three patients by forced duction testing, made surgery difficult. Two patients underwent frontalis muscle sli...

    journal_title:American journal of ophthalmology

    pub_type: 杂志文章

    doi:10.1016/0002-9394(86)90009-7

    authors: O'Keefe M,Crawford JS,Young JD,Macrae WG

    更新日期:1986-09-15 00:00:00

  • Diffusion-weighted magnetic resonance imaging in Shaken Baby Syndrome.

    abstract:PURPOSE:To evaluate the role of diffusion-weighted magnetic resonance imaging (DWIMRI) in the diagnosis and management of children with suspected or confirmed Shaken Baby Syndrome (SBS). METHODS:This was a retrospective interventional case series of all infants and children younger than 2 years of age admitted to a ch...

    journal_title:American journal of ophthalmology

    pub_type: 杂志文章

    doi:10.1016/s0002-9394(01)01366-6

    authors: Biousse V,Suh DY,Newman NJ,Davis PC,Mapstone T,Lambert SR

    更新日期:2002-02-01 00:00:00

  • Current practices in the management of ocular toxoplasmosis.

    abstract::To determine current practices in the management of ocular toxoplasmosis, 72 of 85 uveitis specialists (85%) in the American Uveitis Society completed a detailed questionnaire. Questions involved the indications for beginning treatment, choice of antiparasitic/antimicrobial agents, and experience with treatment of ocu...

    journal_title:American journal of ophthalmology

    pub_type: 杂志文章

    doi:10.1016/s0002-9394(14)73706-7

    authors: Engstrom RE Jr,Holland GN,Nussenblatt RB,Jabs DA

    更新日期:1991-05-15 00:00:00

  • Gevokizumab in the Treatment of Autoimmune Non-necrotizing Anterior Scleritis: Results of a Phase I/II Clinical Trial.

    abstract:PURPOSE:To evaluate the safety and potential efficacy of gevokizumab, an anti-interleukin 1β (IL-1β) monoclonal antibody, in the treatment of active, noninfectious, non-necrotizing anterior scleritis. DESIGN:Phase 1/2, open label, nonrandomized, prospective, single-arm pilot trial. METHODS:Eight patients with active,...

    journal_title:American journal of ophthalmology

    pub_type: 临床试验,杂志文章

    doi:10.1016/j.ajo.2016.09.017

    authors: Knickelbein JE,Tucker WR,Bhatt N,Armbrust K,Valent D,Obiyor D,Nussenblatt RB,Sen HN

    更新日期:2016-12-01 00:00:00

  • Subacute sclerosing panencephalitis manifesting as viral retinitis: clinical and histopathologic findings.

    abstract:PURPOSE AND METHODS:To describe the clinical and histopathologic features of a patient with viral retinitis secondary to subacute sclerosing panencephalitis. RESULTS:The patient was a human immunodeficiency virus-negative intravenous drug abuser with an acute retinitis that later progressed to encephalitis despite agg...

    journal_title:American journal of ophthalmology

    pub_type: 杂志文章

    doi:10.1016/s0002-9394(14)70179-5

    authors: Park DW,Boldt HC,Massicotte SJ,Akang EE,Roos KL,Bodnar A,Pless J,Ghetti B,Pascuzzi RM

    更新日期:1997-04-01 00:00:00

  • Central retinal artery occlusion after carotoid angiography.

    abstract::A 4-year-old boy had a case of ischemic infarction of the retina that occurred immediately after percutaneous carotoid angiography via a femoral percutaneous approach. This appears to be the youngest reported patient with this complication. ...

    journal_title:American journal of ophthalmology

    pub_type: 杂志文章

    doi:10.1016/0002-9394(76)90203-8

    authors: Carlson MR,Pilger IS,Rosenbaum AL

    更新日期:1976-01-01 00:00:00

  • Hepatocyte growth factor levels in the vitreous of patients with proliferative vitreoretinopathy.

    abstract:PURPOSE:To determine whether the hepatocyte growth factor is increased in the vitreous of patients with proliferative vitreoretinopathy. METHODS:We assayed hepatocyte growth factor levels by enzyme-linked immunosorbent assay (ELISA) in vitreous samples from 65 consecutive eyes (65 patients) with proliferative vitreore...

    journal_title:American journal of ophthalmology

    pub_type: 杂志文章

    doi:10.1016/s0002-9394(00)00360-3

    authors: Mitamura Y,Takeuchi S,Matsuda A,Tagawa Y,Mizue Y,Nishihira J

    更新日期:2000-05-01 00:00:00

  • Bright-flash electroretinography for the evaluation of eyes with opaque vitreous.

    abstract::Standard retinal function tests are of limited value in assessing retinal function in eyes with opaque vitreous. We developed a method for obtaining an electroretinogram (ERG) from eyes with significant vitreous opacity by utilizing a much brighter than normal stimulating light. Of 115 eyes with vitreous opacities and...

    journal_title:American journal of ophthalmology

    pub_type: 杂志文章

    doi:10.1016/0002-9394(75)90135-x

    authors: Fuller DG,Knighton RW,Machemer R

    更新日期:1975-08-01 00:00:00

  • Primary oculomotor nerve synkinesis caused by an extracavernous intradural aneurysm.

    abstract::Primary oculomotor nerve synkinesis is almost always caused by an intracavernous meningioma or aneurysm. We treated a patient who had signs of primary oculomotor nerve synkinesis from an unruptured extracavernous aneurysm located at the junction of the internal carotid artery and the posterior communicating artery. Th...

    journal_title:American journal of ophthalmology

    pub_type: 杂志文章

    doi:10.1016/s0002-9394(14)72846-6

    authors: Varma R,Miller NR

    更新日期:1994-07-15 00:00:00

  • Effects of topical betaxolol, timolol, and placebo on pulmonary function in asthmatic bronchitis.

    abstract::To evaluate the effects of two beta-adrenergic blocking agents on cardiopulmonary function, we investigated two topical ophthalmologic preparations, betaxolol 1% and timolol 0.5%, in nine patients (eight men and one woman, ranging in age from 25 to 69 years) with reactive airway disease. The randomized, double-masked,...

    journal_title:American journal of ophthalmology

    pub_type: 杂志文章

    doi:10.1016/0002-9394(84)90450-1

    authors: Schoene RB,Abuan T,Ward RL,Beasley CH

    更新日期:1984-01-01 00:00:00

  • Tilt and decentration of three-piece foldable high-refractive silicone and hydrophobic acrylic intraocular lenses with 6-mm optics in an intraindividual comparison.

    abstract:PURPOSE:Intraindividual comparison of tilt and decentration of three-piece foldable intraocular lenses (IOLs) with 6-mm optics and different edge design and material. DESIGN:Prospective randomized study. METHODS:Twenty-five patients with senile cataract (group I) received a foldable silicone, sharp optic edge IOL in ...

    journal_title:American journal of ophthalmology

    pub_type: 杂志文章,多中心研究,随机对照试验

    doi:10.1016/j.ajo.2005.07.026

    authors: Baumeister M,Neidhardt B,Strobel J,Kohnen T

    更新日期:2005-12-01 00:00:00

  • The Taa1 restriction enzyme provides a simple means to identify the Q368STOP mutation of the myocilin gene in primary open angle glaucoma.

    abstract:PURPOSE:To identify a rapid and reliable method to detect the Glutamine 368 STOP (Q368STOP) disease-predisposing allele of the myocilin gene associated with adult onset, primary, open-angle glaucoma. METHODS:Individuals with the Q368STOP mutation of the myocilin gene were identified from a cohort of primary open-angle...

    journal_title:American journal of ophthalmology

    pub_type: 杂志文章

    doi:10.1016/s0002-9394(00)00812-6

    authors: Baird PN,Dickinson J,Craig JE,Mackey DA

    更新日期:2001-04-01 00:00:00

  • Risk Factors for Secondary Glaucoma in Herpetic Anterior Uveitis.

    abstract:PURPOSE:To determine the incidence of elevated intraocular pressure (IOP) and secondary glaucoma in herpetic anterior uveitis (AU), owing to either herpes simplex or varicella zoster virus, by using the Standardization of Uveitis Nomenclature (SUN) criteria, and to identify risk factors for the development of glaucoma....

    journal_title:American journal of ophthalmology

    pub_type: 杂志文章

    doi:10.1016/j.ajo.2017.06.013

    authors: Hoeksema L,Jansonius NM,Los LI

    更新日期:2017-09-01 00:00:00

  • Incidence and Outcomes of Intraoperative Descemet Membrane Perforations During Deep Anterior Lamellar Keratoplasty.

    abstract:PURPOSE:To report the incidence and outcomes of intraoperative Descemet membrane (DM) perforations during deep anterior lamellar keratoplasty (DALK). DESIGN:Retrospective, consecutive, interventional case series. METHODS:A retrospective audit of all DALK cases performed from 2004 to 2015 in a tertiary center, with an...

    journal_title:American journal of ophthalmology

    pub_type: 杂志文章

    doi:10.1016/j.ajo.2018.10.026

    authors: Huang OS,Htoon HM,Chan AM,Tan D,Mehta JS

    更新日期:2019-03-01 00:00:00

  • Topiramate use and the risk of glaucoma development: a population-based follow-up study.

    abstract:PURPOSE:To investigate the risk of glaucoma development after being prescribed topiramate. DESIGN:A retrospective, population-based cohort study using an administrative database. METHODS:The study group comprised 1956 patients who received their first prescription of topiramate between 2001 and 2007. The comparison c...

    journal_title:American journal of ophthalmology

    pub_type: 杂志文章

    doi:10.1016/j.ajo.2012.07.016

    authors: Ho JD,Keller JJ,Tsai CY,Liou SW,Chang CJ,Lin HC

    更新日期:2013-02-01 00:00:00

  • Topical linezolid 0.2% for the treatment of vancomycin-resistant or vancomycin-intolerant gram-positive bacterial keratitis.

    abstract:PURPOSE:To report the successful use of topical linezolid 0.2% in the treatment of gram-positive bacterial keratitis. STUDY DESIGN:Retrospective, interventional case series. METHODS:All cases of bacterial keratitis treated at the University of Illinois Eye and Ear Infirmary with topical linezolid were identified from...

    journal_title:American journal of ophthalmology

    pub_type: 杂志文章

    doi:10.1016/j.ajo.2013.01.010

    authors: Tu EY,Jain S

    更新日期:2013-06-01 00:00:00

  • Visual function after laser hyperthermia and chemotherapy for macular retinoblastoma.

    abstract:PURPOSE:To determine whether treatment of macular retinoblastoma with chemotherapy and laser hyperthermia can lead to satisfactory visual acuity. METHODS:A child with unilateral macular retinoblastoma was treated with chemotherapy and laser hyperthermia. Visual function was assessed one year after treatment. RESULTS:...

    journal_title:American journal of ophthalmology

    pub_type: 杂志文章

    doi:10.1016/s0002-9394(14)75441-8

    authors: Lueder GT,Goyal R

    更新日期:1996-05-01 00:00:00

  • Ocular myasthenia gravis and Hashimoto's thyroiditis.

    abstract::Two patients had ocular myasthenia gravis coexistent with Hashimoto's thyroiditis. A 37-year-old woman presented with diplopia and displayed a head tremor and thyroid enlargement. Thyroid function tests showed an elevated thyroid stimulating hormone (TSH). A quantitative Tensilon test yeilded a "perverse" response and...

    journal_title:American journal of ophthalmology

    pub_type: 杂志文章

    doi:10.1016/0002-9394(75)90691-1

    authors: Osher RH,Smith JL

    更新日期:1975-06-01 00:00:00

  • A modified laser test for the identification of retinoschisis.

    abstract:PURPOSE:To describe a modified laser technique for identification of bullous retinoschisis. DESIGN:Retrospective interventional case series collected over 10 years. METHODS:Forty-one eyes with presumed bullous retinoschisis and breaks in the inner layers underwent a low-energy argon green laser application at the sam...

    journal_title:American journal of ophthalmology

    pub_type: 杂志文章

    doi:10.1016/s0002-9394(03)00485-9

    authors: Lincoff H,Kreissig I,Stopa M

    更新日期:2003-11-01 00:00:00

  • Anterior segment tumor biopsy using an aspiration cutter technique: clinical experience.

    abstract:PURPOSE:To report the results and complications of an aspiration cutter-assisted anterior segment tumor biopsy performed under viscoelastic. DESIGN:Retrospective, consecutive, interventional case series. METHODS:Fifty-five patients with anterior segment tumors underwent aspiration cutter-assisted biopsy at a single c...

    journal_title:American journal of ophthalmology

    pub_type: 杂志文章

    doi:10.1016/j.ajo.2011.04.032

    authors: Petousis V,Finger PT,Milman T

    更新日期:2011-11-01 00:00:00

  • A morphologic and autoradiographic study of cell death and regeneration in the retinal microvasculature of normal and diabetic rats.

    abstract::Cell loss and regeneration were investigated and compared in the retinal microvasculature of age- and sex-matched normal and streptozotocin diabetic rats. Selective pericyte loss in the diabetic rat was characterized by changes in the pericyte to endothelial cell ratio in retinal capillaries isolated for microscopy by...

    journal_title:American journal of ophthalmology

    pub_type: 杂志文章

    doi:10.1016/s0002-9394(14)74982-7

    authors: Sharma NK,Gardiner TA,Archer DB

    更新日期:1985-07-15 00:00:00

  • Sclerochoroidal calcification associated with Gitelman syndrome.

    abstract:PURPOSE:To investigate sclerochoroidal calcification in a patient with Gitelman syndrome. METHOD:Case report. Bilateral fundus abnormalities observed in a 58-year-old woman were documented with fluorescein angiography and tomodensitometry. RESULTS:Symmetric yellow-white subretinal lesions were observed in the superot...

    journal_title:American journal of ophthalmology

    pub_type: 杂志文章

    doi:10.1016/s0002-9394(99)00277-9

    authors: Bourcier T,Blain P,Massin P,Grünfeld JP,Gaudric A

    更新日期:1999-12-01 00:00:00

  • Evaluation of Tear Protein Markers in Dry Eye Disease with Different Lymphotoxin-Alpha Expression Levels.

    abstract:PURPOSE:To compare tear protein markers between normal subjects and patients with dry eye (DE) and high and low lymphotoxin-alpha (LT-α) levels. DESIGN:Prospective cross-sectional study. METHODS:Patients with DE were divided into low (≤700 pg/mL) and high (>700 pg/mL) LT-α groups. Twelve protein markers were measured...

    journal_title:American journal of ophthalmology

    pub_type: 杂志文章

    doi:10.1016/j.ajo.2020.03.013

    authors: Chen H,Chen H,Liang L,Zhong Y,Liang Y,Yu Y,Huang S,Lu X

    更新日期:2020-09-01 00:00:00

  • Management options for early stages of acutely symptomatic macular holes.

    abstract:PURPOSE:To evaluate management options for acutely symptomatic stage I macular holes, and vitrectomy with intraocular gas tamponade for advanced stage I macular holes. METHODS:Retrospective noncomparative study. SETTING:Clinical practice. STUDY POPULATION:Acutely symptomatic stage I macular holes. MANAGEMENT OPTIONS...

    journal_title:American journal of ophthalmology

    pub_type: 杂志文章

    doi:10.1016/s0002-9394(01)01266-1

    authors: Kokame GT

    更新日期:2002-02-01 00:00:00

  • A dose-response study of piloplex for duration of action.

    abstract::The duration of reduction in intraocular pressure after single-dose administration of three concentrations of piloplex and the vehicle of the drug was evaluated in 12 patients with open-angle glaucoma in a randomized, double-masked, crossover study. Piloplex lowered intraocular pressure in a dose-related fashion, with...

    journal_title:American journal of ophthalmology

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:10.1016/s0002-9394(14)75861-1

    authors: Klein HZ,Lugo M,Shields MB,Leon J,Duzman E

    更新日期:1985-01-15 00:00:00

  • Angiogenic and Immunologic Proteins Identified by Deep Proteomic Profiling of Human Retinal and Choroidal Vascular Endothelial Cells: Potential Targets for New Biologic Drugs.

    abstract:PURPOSE:Diseases that involve retinal or choroidal vascular endothelial cells are leading causes of vision loss: age-related macular degeneration, retinal ischemic vasculopathies, and noninfectious posterior uveitis. Proteins differentially expressed by these endothelial cell populations are potential drug targets. We ...

    journal_title:American journal of ophthalmology

    pub_type: 杂志文章

    doi:10.1016/j.ajo.2018.03.020

    authors: Smith JR,David LL,Appukuttan B,Wilmarth PA

    更新日期:2018-09-01 00:00:00

  • Racial differences in age-related macular degeneration rates in the United States: a longitudinal analysis of a managed care network.

    abstract:PURPOSE:To compare the incidence, prevalence, and hazard of nonexudative and exudative age-related macular degeneration (AMD) among different races throughout the United States. DESIGN:Retrospective longitudinal cohort study. METHODS:Billing records of all encounters for 2 259 061 beneficiaries aged ≥40 enrolled in a...

    journal_title:American journal of ophthalmology

    pub_type: 杂志文章

    doi:10.1016/j.ajo.2011.02.004

    authors: Vanderbeek BL,Zacks DN,Talwar N,Nan B,Musch DC,Stein JD

    更新日期:2011-08-01 00:00:00

  • Postoperative recurrence of Reis-Bücklers' corneal dystrophy.

    abstract::A 68-year-old white man with a documented family history of Reis-Bücklers' corneal dystrophy had a penetrating keratoplasty of the right eye in 1962. In 1977, vision decreased and foreign body sensation returned. Examination indicated a Reis-Bücklers' dystrophy that had appeared in the graft 15 years after the transpl...

    journal_title:American journal of ophthalmology

    pub_type: 杂志文章

    doi:10.1016/s0002-9394(14)75256-0

    authors: Caldwell DR

    更新日期:1978-04-01 00:00:00

  • Incidence of cystoid macular edema after cataract surgery in patients with and without uveitis using optical coherence tomography.

    abstract:PURPOSE:To determine the incidence of cystoid macular edema (CME) after cataract surgery among eyes with and without uveitis using optical coherence tomography (OCT) and to determine risk factors for postoperative CME among eyes with uveitis. DESIGN:Prospective, comparative cohort study. METHODS:Single-center, academ...

    journal_title:American journal of ophthalmology

    pub_type: 杂志文章

    doi:10.1016/j.ajo.2009.02.029

    authors: Bélair ML,Kim SJ,Thorne JE,Dunn JP,Kedhar SR,Brown DM,Jabs DA

    更新日期:2009-07-01 00:00:00