China
Africa
Explore chapters and articles related to this topic
Hunter disease/mucopolysaccharidosis type II/iduronate sulfatase deficiency
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
Hearing loss is regularly observed. Retinal dysfunction may be documented by electroretinography [35]. Chronic papilledema has been reported in the absence of increased intracranial pressure [36, 37]. Hydrocephalus appears to be rare in the mild forms of Hunter disease [28]. Arachnoid cysts have been observed [38]. Spinal stenosis, especially cervical may cause cord compression [39].
Intravitreal toxicity of bevacizumab
Published in A Peyman MD Gholam, A Meffert MD Stephen, D Conway MD FACS Mandi, Chiasson Trisha, Vitreoretinal Surgical Techniques, 2019
Roberta PA Manzano, Gholam A Peyman
Because retinal toxicity is a primary concern when using intravitreal injections, we investigated the retinal toxicity of bevacizumab at various doses: 500μg/0.1 ml, 1.0 mg/0.1 ml, 2.5 mg/0.1 ml, and 5.0 mg/0.2 ml in the rabbit eye. Using clinical examination, electroretinography (Fig. 67.1) and histologic evaluation, we found no toxicity at any dose.
Mitochondrial Dysfunction and Hearing Loss
Published in Shamim I. Ahmad, Handbook of Mitochondrial Dysfunction, 2019
Although in the name of this mitochondrial disease HL does not appear, it is one of the cardinal clinical features. A case of mother and daughter who developed cataracts, deafness, cerebellar ataxia and diabetes, all of them in adult life but not at the same time, was described, and a 12258C-A heteroplasmic patogenic variant of the MT-TS2 gene was found. In other cases reported with the same patogenic variant, the characteristics were different, the patients have progressive sensorineural hearing loss and retinitis pigmentosa (Retinitis pigmentosa-deafness syndrome), these symptoms were very similar to those in the USHER syndrome type III. Patients begin with hearing problems first, shortly after in their 20s they note the ophthalmologic symptoms: impairment of night vision and loss of peripheral visual fields. It is important to note that the electroretinography responses are abnormal before the symptoms begin.36,37
Multifocal Electroretinogram in Keratoconus Patients without and with Scleral Lenses
Published in Current Eye Research, 2021
Ana Amorim-de-Sousa, Rute Macedo-de-Araújo, Paulo Fernandes, António Queirós, José M. González-Méijome
Electrophysiologic studies of vision in humans have been conducted since 1940.17 Electroretinography allows objective assessment of the retinal function by recording the retinal electrical activity when presented with a visual stimulus. The variety of electroretinogram (ERG) tests facilitates assessment of the functionality of different cellular layers or specific retinal pathways that contribute to distinct image processing.17–20 The multifocal ERG (mfERG) is a technique that can assess local ERG responses from different retinal regions between 40 and 60 degrees of field. The stimulus is a pattern comprised of hexagons scaled with eccentricity. Each hexagon corresponds to a retinal region and simultaneously follows a pseudo-random binary m-sequence cycle stimulation.21 Once recorded, the response of a set of hexagonal elements corresponding to a specific retinal area can be correlated and calculated.
Ultra-small-size Astragaloside-IV loaded lipid nanocapsules eye drops for the effective management of dry age-related macular degeneration
Published in Expert Opinion on Drug Delivery, 2020
Rong Sun, Anan Zhang, Ying Ge, Jingxin Gou, Tian Yin, Haibing He, Yanjiao Wang, Guimin Zhang, Jun Kong, Lixia Shang, Xiumei Tao, Yu Zhang, Xing Tang
Electroretinography is used to measure the electrical responses of various retina cells, including the photoreceptors, the inner retinal cells, and the ganglion cells. It has been widely used in both the clinic and in research for the diagnosis of various retinal diseases. The a-wave reflects the function of the cone cells and the rod cells, and the b-wave represents the function of the bipolar cells. Compared with the controls, the amplitudes of NaIO3-induced dAMD mice in the blank-LNCs-20 group was significantly decreased. In contrast, the amplitudes of mice administered with ASIV-LNCs-20 were markedly higher (Figure 8). In other words, a reduction of amplitudes could be successfully prevented by treatment with ASIV-LNCs-20. It can be seen from the line chart that the therapeutic index increased on 28 d generally, suggesting that daily ASIV-LNCs-20 treatment can protect the retina from NaIO3-induced damage in mice. Although some graphs showed a therapeutic index decrease at 28 d, the amplitude of ASIV- LNCs-20 group was still higher than blank-LNCs-20 group, indicating that the development of the disease has been effectively delayed.
Congenital Nystagmus and Its Congeners
Published in Journal of Binocular Vision and Ocular Motility, 2020
Neuroimaging is unnecessary in the diagnostic evaluation of congenital nystagmus, except in the specific instances discussed below. I almost never obtain hemispheric VEPs to rule out albinism, but instead rely on the finding of positive angle kappas in conjunction with other clinical signs such as iris transillumination, foveal hypoplasia, chorioretinal hypopigmentation, cutaneous hypopigmentation, and a positive family history. In the past, electroretinography was performed when clinical signs of congenital retinal dystrophy were present, followed by genetic testing when possible. More recently, this diagnostic sequence has inverted since electroretinographic waveforms do not fully mature until one year of age,49 and many genes that cause congenital retinal dystrophies are now quickly and easily identifiable.50,51 Optical coherence tomography (OCT) of the macula has been added as a core component of the diagnostic evaluation, and handheld OCT can often be used in young children.52–54 In addition to identifying subclinical foveal hypoplasia (even in some cases with the “idiopathic” FRMD7 mutation), OCT can show a progressive optical emptying of the fovea in patients with achromatopsia (previously thought to be a static disease).55