Proof-of-concept and Randomized, Placebo-controlled Trials of an FcRn Inhibitor, Batoclimab, for Thyroid Eye Disease.

Context: Inhibition of the neonatal fragment crystallizable receptor (FcRn) reduces pathogenic thyrotropin receptor antibodies (TSH-R-Ab) that drive pathology in thyroid eye disease (TED).

Objective: We report the first clinical studies of an FcRn inhibitor, batoclimab, in TED.

Design: Proof-of-concept (POC) and randomized, double-blind placebo-controlled trials.

Imaging of the medial rectus muscle predicts the development of optic neuropathy in thyroid eye disease.

Goal of the study was to evaluate bony orbit remodeling and extraocular muscle (EOM) volume in thyroid eye disease (TED) and their role as predicting factors for development of dysthyroid optic neuropathy (DON). Orbital computed tomography of 92 patients with TED with (76 orbits) or without DON (98 orbits) were retrospectively evaluated. Orbits (n = 40) of subjects without TED served as controls.

Sinogenic Orbital Complications.

Background: The term "orbital complication" does not designate an independent nosological entity, but is rather a collective designation for diseases or disease effects that involve the orbit and its internal structures by extension from outside. In general, their most prominent manifestation is swelling of the orbital soft tissues, usually unilaterally. The incidence of sinogenic orbital complications is approximately 1.

Retinal thinning in phenylketonuria and Gaucher disease type 3.

Purpose: Retinal alterations in inherited metabolic diseases associated with neurodegeneration are poorly studied. The objective was to study retinal thickness, specifically the components of the ganglion cell complex (GCC)-nerve fiber layer (NFL), ganglion cell layer (GCL), and inner plexiform layer (IPL)-using spectral-domain optical coherence tomography (SD-OCT) in two different diseases with potential dopaminergic depletion, phenylketonuria (PKU) and Gaucher disease type 3 (GD3).

Methods: Retinal layers in 19 patients with PKU, 15 patients with GD3, and 93 healthy individuals were measured using peripapillary ring scan and macular SD-OCT.

[IgG4-related disease].

Background: The IgG4-related systemic disease as well as the homonymous variant IgG4-related orbital disease were first described less than 15 years ago. The mostly subacute clinical symptoms can be multifarious and the classical case is characterized by an orbital inflammatory condition with a bilateral enlargement of the lacrimal glands; however, any other orbital tissue with the exception of the eyeball can be affected by the lymphocytic inflammatory infiltration.

Material And Methods: Based on the current literature the clinical picture, epidemiology, pathogenesis and treatment options are described.

Vertical saccadic palsy and foveal retinal thinning in Niemann-Pick disease type C.

Introduction: Niemann-Pick type C (NPC) is a lysosomal storage disease that is progressive and life-limiting, with an estimated incidence of 1:120,000 live births. In addition to systemic manifestation with (hepato-)splenomegaly, there are a number of neurological manifestations (ataxia, dysarthria, dementia, cataplexy, epileptic seizures, and psychiatric disorders). Characteristic is vertical supranuclear gaze palsy, which is often overlooked.

[Visual acuity in German preschool children-results of a cross-sectional study].

Background: Within the framework of the German pediatric screening examinations, the checks include visual functions. There is no ophthalmological screening examination in childhood in Germany. This study investigated whether participation in the pediatric screening examinations U8 (at the end of the fourth year of life) and U9 (at the beginning of the sixth year of life) is associated with the results of visual acuity, which are compiled at the school entry examinations (SEU).

Saccadic reaction time and ocular findings in phenylketonuria.

Background: Phenylketonuria (PKU) is an inherited metabolic disorder characterized by reduced activity of phenylalanine hydroxylase resulting in elevated blood phenylalanine (Phe) concentration. Despite some obvious ocular changes, the disorder has been poorly recognized by ophthalmologists. Neurophysiologic tests imply prolonged reaction time correlating with increased phenylalanine blood concentrations.

No Benefit of a Pediatric Screening in Discovering Reduced Visual Acuity in Children: Experiences from a Cross-Sectional Study in Germany.

Background: The newly introduced German pediatric screening examination at the end of the third year of life (U7a) incorporates visual function testing in particular; there is no ophthalmic screening during childhood in Germany. The purpose of this study is to investigate the relationship between participation in U7a and visual function at the preschool health examination (PHE) in the sixth year of life.

Methods: This study evaluated PHE data from school enrollment years 2009/2010 to 2014/2015 of Rhineland-Palatinate, Germany.

[Orbital Inflammation: Current Trends].

The field of idiopathic orbital inflammation has come into motion: Ever since the first description of IgG4-associated disease about 10 years ago, the group of idiopathic lesions has shrunk by about half. The differential diagnosis has become much more demanding because the clinical and imaging overlap is considerable. This article presents a review of idiopathic orbital inflammation, IgG4-associated disease, and orbital lymphoid hyperplasia.

[Periocular Necrobiotic Xanthogranuloma with Mono- and Biclonal Gammopathy].

Necrobiotic xanthogranuloma is a very rare disease with granulomatous lesions of the skin with possible association with paraproteinemia. We report two cases of orbital necrobiotic xanthogranuloma in patients with mono/biclonal gammopathy of undetermined significance. Both patients underwent successful conservative treatment.

Intact Visuospatial Cognition in Amblyopia: Results From the Gutenberg Health Study.

Purpose: To determine whether amblyopia interferes with cognitive functions requiring visuospatial processing, measured by the Tower of London (ToL) test.

Methods: The current study was based on a sub-cohort from the population-based Gutenberg Health Study and included 1,569 participants aged 35 to 44 years. Amblyopia was defined as a visual acuity of 0.

A comprehensive monocentric ophthalmic study with Gaucher disease type 3 patients: vitreoretinal lesions, retinal atrophy and characterization of abnormal saccades.

Background: The differentiation between Gaucher disease type 3 (GD3) and type 1 is challenging because pathognomonic neurologic symptoms may be subtle and develop at late stages. The ophthalmologist plays a crucial role in identifying the typical impairment of horizontal saccadic eye movements, followed by vertical ones. Little is known about further ocular involvement.

The Diagnosis and Treatment of Idiopathic Facial Paresis (Bell's Palsy).

Background: Peripheral facial nerve palsy is the most com- mon functional disturbance of a cranial nerve. 60-75% of cases are idiopathic.

Methods: This review is based on a selective literature search proceeding from the current, updated German-language guideline on the diagnosis and treatment of idiopathic facial nerve palsy.

Ophthalmological Findings in Mucopolysaccharidoses.

The mucopolysaccharidoses (MPS) are a heterogenous group of lysosomal storage disorders caused by the accumulation of glycosaminoglycans (GAGs). The accrual of these compounds results in phenotypically varied syndromes that produce multi-organ impairment with widespread systemic effects. The low incidence of MPS (approximately 1/25,000 live births) in conjunction with the high childhood mortality rate had limited the availability of research into certain clinical features, especially ocular manifestations.

Ocular ductions after rectus muscle recession and resection in thyroid eye disease.

: Recession and resection of rectus muscles for correction of strabismus in Thyroid Eye Disease (TED) is relatively unpopular as it is assumed to enhance the restriction of ocular ductions. Therefore, the purpose of this study was to compare ductions of recession only and recess/resect procedures. : We retrospectively reviewed the charts of 119 patients who underwent strabismus surgery for TED from 1991 to 2015, of which 102 were included in the present study.

Corneal topometric, aberrometric and biomechanical parameters in mucopolysaccharidosis patients.

Aims: To report corneal topometric and aberrometric values in mucopolysaccharidosis (MPS) and to investigate their correlation with biomechanical corneal parameters.

Methods: One randomly chosen eye of 20 MPS patients with no to moderate corneal clouding and one eye of 23 healthy controls with comparable age were prospectively included into this study. Corneal surface regularity was assessed by index of surface variance (ISV), -vertical asymmetry (IVA), -height asymmetry (IHA), -height decentration (IHD); keratoconus index (KI), central keratoconus index (CKI) and Zernike indices of anterior and posterior corneal surface using Scheimpflug imaging (Pentacam).

[Orbital Manifestation of IgG4-Related Disease: Three Case Reports].

IgG4-related orbital disease is rare. It belongs to an entity of a multisystemic disorder - IgG4-related disease - which has emerged recently. Differential diagnosis comprises idiopathic orbital inflammation, endocrine orbitopathy and orbital neoplasms.

[Primary Orbital Liposarcoma].

A 30-year-old male patient presented with a painful globe subluxation of the left eye since two weeks. Painless left proptosis had been noted at least 2 years earlier. An MRI performed one year after onset of symptoms showed a retrobulbar mass, which on biopsy was diagnosed as spindle cell lipoma.

Neuro-Ophthalmology at the Bedside: A Clinical Guide.

Neuro-ophthalmological signs and symptoms are common in the emergency department but are a frequent source of diagnostic uncertainties. However, neuro-ophthalmological signs often allow a precise neuro-topographical localization of the clinical problem. A practical concept is presented how to perform a neuro-ophthalmological examination at the bedside and to interpret key findings under the aspect of emergency medicine with limited resources.

Age dependent normative data of vertical and horizontal reflexive saccades.

Purpose: There is some controversy whether or not saccades change with age. This cross-sectional study aims to clarify the characteristics of reflexive saccades at various ages to establish a normative cohort in a standardized set-up. Second objective is to investigate the feasibility of saccadometry in daily ophthalmological practice.