Thyroid dermopathy responds to teprotumumab therapy.

Summary: Thyroid dermopathy is an uncommon manifestation of thyroid disease that impairs the quality of life in certain cases. Currently, the available treatments offer limited results and a chance of recurrence. Teprotumumab, a novel medication that results in the regression of thyroid ophthalmopathy, may have similar effects on dermopathy.

Teprotumumab Efficacy, Safety, and Durability in Longer-Duration Thyroid Eye Disease and Re-treatment: OPTIC-X Study.

Purpose: To evaluate teprotumumab safety/efficacy in patients with thyroid eye disease (TED) who were nonresponsive or who experienced a disease flare.

Design: The Treatment of Graves' Orbitopathy to Reduce Proptosis with Teprotumumab Infusions in an Open-Label Clinical Extension Study (OPTIC-X) is a teprotumumab treatment and re-treatment trial following the placebo-controlled teprotumumab Phase 3 Treatment of Graves' Orbitopathy (Thyroid Eye Disease) to Reduce Proptosis with Teprotumumab Infusions in a Randomized, Placebo-Controlled, Clinical Study (OPTIC) trial.

Participants: Patients who previously received placebo (n = 37) or teprotumumab (n = 14) in OPTIC.

Teprotumumab in Clinical Practice: Recommendations and Considerations From the OPTIC Trial Investigators.

Background: Thyroid eye disease (TED) is a vision-threatening and debilitating condition that until very recently had no Food and Drug Administration (FDA)-approved medical therapies. Teprotumumab has recently been approved to treat TED. We aim to provide guidance for its use, based on the input of the US investigators who participated in Phase 2 and Phase 3 clinical trials.

Teprotumumab for the Treatment of Active Thyroid Eye Disease.

Background: Thyroid eye disease is a debilitating, disfiguring, and potentially blinding periocular condition for which no Food and Drug Administration-approved medical therapy is available. Strong evidence has implicated the insulin-like growth factor I receptor (IGF-IR) in the pathogenesis of this disease.

Methods: In a randomized, double-masked, placebo-controlled, phase 3 multicenter trial, we assigned patients with active thyroid eye disease in a 1:1 ratio to receive intravenous infusions of the IGF-IR inhibitor teprotumumab (10 mg per kilogram of body weight for the first infusion and 20 mg per kilogram for subsequent infusions) or placebo once every 3 weeks for 21 weeks; the last trial visit for this analysis was at week 24.

Dynamic contrast-enhanced MRI of orbital and anterior visual pathway lesions.

Purpose: The accurate diagnosis of orbital and anterior visual pathway lesions has clinical significance. We determined whether dynamic contrast-enhanced MRI could differentiate benign from malignant lesions and compared model-independent and model-dependent methods of data analysis.

Methods: We retrospectively reviewed dynamic contrast-enhanced MRI studies of 37 enhancing orbital and anterior visual pathway lesions.

Optical Coherence Tomography for the Radiologist.

Optical coherence tomography is an imaging technique using low coherence light sources to produce high-resolution cross-sectional images. This article reviews pertinent anatomy and various pathologies causing optic atrophy (eg, compressive, infiltrating, demyelinating) versus optic nerve swelling (from increased intracranial pressure known as papilledema or other optic nerve intrinsic pathologies). On optical coherence tomography, optic atrophy is often associated with reduced average retinal nerve fiber layer thickness, whereas optic nerve swelling is usually associated with increased average retinal nerve fiber layer thickness.

Diagnostic Ophthalmic Ultrasound for Radiologists.

Ophthalmic ultrasound is an invaluable tool that provides quick and noninvasive evaluation of the eye and the orbit. It not only allows the clinicians to view structures that may not be visible with routine ophthalmic equipment or neuroimaging techniques but also provides unique diagnostic information in various ophthalmic conditions. In this article, the basic principles of ophthalmic ultrasound and examination techniques are discussed.

Significance of Anti-retinal Autoantibodies in Cancer-associated Retinopathy with Gynecological Cancers.

Background: The presence of autoantibodies (AAbs) is the primary serological indicator of autoimmunity. Cancer-associated retinopathy (CAR) is associated with AAbs and different types of cancer. The goal of the study was to examine the profile of serum autoantibodies in women with gynecological cancers with and without paraneoplastic visual manifestation.

Diversity in autoimmunity against retinal, neuronal, and axonal antigens in acquired neuro-retinopathy.

Purpose: Autoimmune retinopathies and optic neuropathies are complex disorders of the retina and the optic nerve, in which patients develop autoantibodies (AAbs) against retinal and optic nerve proteins. Autoimmunity might significantly influence the outcome of retinal and optic nerve degenerative process but the pathogenic process is not fully elucidated. To better understand the role of AAbs in pathogenicity of these suspected autoimmune visual disorders, we focused on unique AAbs specificities associated with the syndrome to identify their antigenic targets in the optic nerve and retina.

Neuro-ophthalmologic complications and manifestations of upper and lower motor neuron facial paresis.

The facial nerve (cranial nerve VII) courses a long pathway beginning in the precentral gyrus and ending at the facial muscles, lacrimal and salivary glands, and structures of the inner ear. Lesions along this pathway, clinically divided into upper and lower motor neuron lesions, present with unique characteristics that assist the physician in identifying the lesion site. The sequelae particularly of peripheral CN VII palsies, may result in significant and chronic damage to the cornea that may be challenging for the physician and patient.

Cancer-associated retinopathy in neuroendocrine carcinoma of the fallopian tube.

A 70-year-old woman developed progressive visual loss with compromised visual acuity and visual fields, cells in the anterior chamber and vitreous, attenuated retinal arterioles, and macular edema. She had undergone right oophorectomy and partial salpingectomy nearly 50 years earlier. Full-field and multifocal electroretinography showed waveforms of markedly attenuated amplitudes, findings consistent with cancer-associated retinopathy (CAR).

Superior ophthalmic vein thrombosis in a patient with chronic myeloid leukemia receiving antifibrinolytic and thrombopoietin receptor agonist therapy.

Isolated superior ophthalmic vein (SOV) thrombosis is a rare condition usually related to inflammation of the orbit or paranasal sinuses. Patients present with acute orbital signs, including proptosis, ophthalmoplegia, globe dystopia, and periorbital edema, and may have diminished vision secondary to optic neuropathy. SOV thrombosis is typically seen in the setting of septic cavernous sinus thrombosis, and antimicrobial therapy is the treatment of choice.

Comparison of optical coherence tomography and scanning laser polarimetry measurements in patients with multiple sclerosis.

Purpose: To compare optical coherence tomography (OCT) and scanning laser polarimetry (GDx) measurements of the retinal nerve fiber layer (RNFL) in multiple sclerosis (MS) patients with and without optic neuritis (ON).

Methods: OCT and GDx were performed on 68 MS patients. Qualifying eyes were divided into two groups: 51 eyes with an ON history > or =6 months before (ON eyes) and 65 eyes with no history of ON (non-ON eyes).

Comparison of multifocal visual evoked potential, standard automated perimetry and optical coherence tomography in assessing visual pathway in multiple sclerosis patients.

Background: Multifocal visual evoked potentials (mfVEP) measure local response amplitude and latency in the field of vision.

Objective: To compare the sensitivity of mfVEP, Humphrey visual field (HVF) and optical coherence tomography (OCT) in detecting visual abnormality in multiple sclerosis (MS) patients.

Methods: mfVEP, HVF, and OCT (retinal nerve fiber layer [RNFL]) were performed in 47 MS-ON eyes (last optic neuritis [ON] attack >or=6 months prior) and 65 MS-no-ON eyes without ON history.

Assessing visual pathway function in multiple sclerosis patients with multifocal visual evoked potentials.

Multifocal visual evoked potentials provide a topographic measure of visual response amplitude and latency. The objective of this study was to evaluate the sensitivity and specificity of the multifocal visual evoked potential technique in detecting visual abnormalities in patients with multiple sclerosis. Multifocal visual evoked potentials were recorded from 74 patients with multiple sclerosis with history of optic neuritis (MS-ON, n = 74 eyes) or without (MS-no-ON, n = 71 eyes), and 50 normal subjects (controls, n = 100 eyes) using a 60-sector pattern reversal dartboard stimulus (VERIS).

Spectrum of pediatric neuromyelitis optica.

Objective: Our goal was to describe the spectrum of clinical phenotypes, laboratory and imaging features, and treatment in pediatric patients with neuromyelitis optica.

Patients And Methods: The study consisted of a retrospective chart review of patients followed in a pediatric multiple sclerosis center with a diagnosis of neuromyelitis optica spectrum disorder.

Results: Nine patients with neuromyelitis optica spectrum disorders were included, all of whom were female.

Concurrent decrease in IL-10 with development of immune-related adverse events in a patient treated with anti-CTLA-4 therapy.

The cytotoxic T lymphocyte antigen-4 (CTLA-4) molecule on T cells acts to maintain homeostasis by regulating the proliferation of recently activated T cells. Blockade of CTLA-4 by anti-CTLA-4 antibody enhances T cell responses and has elicited significant tumor regression in some cancer patients. Clinical trials are ongoing to investigate the efficacy of anti-CTLA-4 antibody as a cancer therapeutic.

Retinal toxicity secondary to Plaquenil therapy.

Background: Hydroxychloroquine sulfate (Plaquenil; Sanofi-Aventis, Bridgewater, New Jersey) is an antimalarial agent, which is sometimes used for the treatment of certain autoimmune disorders. Its use has been associated with ocular side effects; the most concerning is toxic maculopathy.

Case Report: A 71-year-old arthritic white woman requested a second opinion regarding retinal Plaquenil toxicity.

The relationship between visual field and retinal nerve fiber layer measurements in patients with multiple sclerosis.

Purpose: To investigate the relationship between visual function, measured by standard automated perimetry (SAP), and retinal nerve fiber layer (RNFL) thickness, measured by optical coherence tomography (OCT), in patients with multiple sclerosis (MS).

Methods: SAP and RNFL thickness were measured in patients with MS in 28 eyes with the last optic neuritis (ON) >or=6 months prior (ON group) and 33 eyes without ON history (non-ON group). Abnormal overall or quadrant RNFL thickness was defined by measured values below 5% of the norm.

Evaluation and treatment of papilledema in pregnancy.

Papilledema is defined as optic disk edema that is secondary to increased intracranial pressure. During pregnancy, papilledema poses additional diagnostic and therapeutic challenges. As in the nonpregnant patient, the primary goal is to urgently determine the cause of the papilledema followed by implementing appropriate management for life-threatening conditions in a timely fashion while safeguarding the fetus.

Compressive optic neuropathy after use of oxidized regenerated cellulose in orbital surgery: review of complications, prophylaxis, and treatment.

Purpose: We report 2 cases of compressive optic neuropathy after use of oxidized regenerated cellulose (ORC) in orbital surgery. To our knowledge, no complications have been reported previously after use of this material in orbital surgery. We also review the complications related to its retention at operative sites outside the orbit and recommend precautions to avoid them.

Telemedicine for eye care.

Tele-ophthalmology has been employed mainly for patients in under-served rural areas in need of specialty care, but other applications such as telementoring have also been used. In certain populations, cost containment is a significant issue and telemedicine is a solution. Tele-ophthalmology can be performed in realtime, by store-and-forward mode, or by hybrid techniques.

Her vision was tied down.

A 50-year-old female presented with progressive painless vision loss in the left eye and was subsequently diagnosed to have a pituitary macroadenoma, consistent with a prolactinoma, which was compressing the chiasm primarily on the left and involving the left cavernous sinus. She was treated with oral bromocriptine, resulting in marked shrinkage of the tumor and significant visual field recovery. Subsequently, she again began noting progressive vision loss in both eyes and was initially thought to have pituitary tumor regrowth.

Photopic ERGs in patients with optic neuropathies: comparison with primate ERGs after pharmacologic blockade of inner retina.

Purpose: To determine whether anterior ischemic optic neuropathy and compressive optic neuropathy in humans alter the photopic flash ERG and to investigate the cellular origins of the waves that are affected by pharmacologic agents in primates.

Methods: Photopic flash ERGs were recorded differentially, with DTL electrodes, between the two eyes of 22 patients with diagnosed optic neuropathy (n = 17, anterior ischemic optic neuropathy [AION]; n = 5, compressive optic neuropathy) and 25 age-matched control subjects and in 17 eyes of 13 monkeys (Macaca mulatta). The stimulus consisted of brief (<5 ms) red (lambda(max) = 660 nm) Ganzfeld flashes (energy range, 0.