Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0038379 (strabismus)
 9,317 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 38-year-old patient showed monocular nonprogressing pigmentary changes for 8 years, with nearly normal blood vessels and scarcely restricted dark-adaptation. ERG and VER were different from those of the other eye. The affected eye had full vision and an annular scotoma corresponding to the pigmentary changes. The other eye was amblyopic due to strabismus. Phenotypes, similar to retinitis pigmentosa, and chorioretinitis diffusa are discussed. This is probably a congenital anomaly.
 ...
PMID:[A case of monocular retinitis pigmentosa (author's transl)]. 120 57

Rod ERGs were obtained over a broad range of stimulus intensities from young patients with retinitis pigmentosa and progressive cone-rod degeneration. Intensity-response functions suggest that children with retinitis pigmentosa have reductions in the number of functioning rods and that surviving rods generating the ERG have reduced sensitivity. Children with cone-rod degeneration also have reduced numbers of rods, but the remaining rods have normal sensitivity. These findings suggest that the underlying mechanisms of rod degeneration differ in these two forms of disease and demonstrate that insights into the pathogenesis of hereditary retinal degeneration can be obtained by testing children. The ability to distinguish between retinitis pigmentosa and cone-rod degeneration in children is also important because of differences in the rates of progression between the two classes of progressive retinal degeneration.
J Pediatr Ophthalmol Strabismus
PMID:Rod ERGs in children with hereditary retinal degeneration. 377 90

The authors have recorded pattern ERGs in 62 amblyopic children. In 12 who were occluded up until the morning of the test, the occluded eye response was reduced, and the ratio ([response amplitude of amblyopic eye]/[response amplitude of fellow eye]) was greater than unity. In the remainder, the ratio was considerably less than unity. Ratios of less than 1.0 were found for anisometropic, esotropic, exotropic, and microtropic amblyopes. The ratio was not related to visual acuity or to squint but was higher in those children whose vision was improved by orthoptic treatment. In a group of older children recalled to the clinic, the ratio was 1.0 in those who had maintained equal visual acuity and was less than unity in those children whose acuity had regressed after treatment, or had not improved during treatment. The reduction of the pattern ERG in amblyopes occurs without a corresponding reduction in the focal ERG. In adult amblyopes, the relationship between loss of acuity, loss of grating contrast sensitivity, and the reduction in the PERG is complex and may differ according to the type of amblyopia.
 ...
PMID:Pattern ERG in amblyopia. 396 59

Electroretinographic recordings were made from 20 different skin points by skin-electrodes when the eye was fixed in a straight forward, lateral and nasal gaze. Three dimensional distributions of the percent amplitude of the b-wave were made using a microcomputer. The best recordings were obtained upon lateral gaze from skin points on the lower eye lid or near the lateral canthus. The skin-electrode ERG recording from these skin points upon lateral gaze may provide a useful diagnostic tool, especially in young children.
J Pediatr Ophthalmol Strabismus
PMID:Topographical distribution of electroretinographic potentials monitored by skin-electrodes. 688 55

Close ophthalmological follow-up of a case of arthrogryposis multiplex congenita disclosed a progressing paresis of the lateral recti, a gradual decrease in the corneal blinking reflex by the age of eight weeks and a clear deterioration of the visual evoked responses by the first 16 weeks of life along with preservation of normal ERG responses. These observations are believed to represent the gradual affection of the motor and sensorial cranial nerves occurring post-natally in this case of AMC.
J Pediatr Ophthalmol Strabismus
PMID:Ocular findings in arthrogryposis multiplex congenita. 709 64

Pattern-reversal electroretinograms were recorded in 10 normals and 10 adult patients with squint amblyopia (visual acuity 0.3 or less). The effects of artificial anisometropia and simulated eccentric fixation were tested in normals. The pattern-reversal ERG amplitude was reduced linearly with increased defocusing (0 to + 3 D). A significant amplitude reduction was observed when defocusing amounted to + 1 D. The amplitudes were not reduced below the range of normal variability at 4 degrees of simulated eccentric fixation. In the amblyopia patients, refractive errors were corrected. None had eccentric fixation of more than 4 degrees. Yet, the pattern-reversal ERGs were lower in the amblyopic eyes compared with the opposite normal eyes. The difference in amplitude means was statistically significant. The finding supports the view that retinal function is impaired in human squint amblyopia.
 ...
PMID:Pattern-reversal electroretinograms in squint amblyopia, artificial anisometropia and simulated eccentric fixation. 713 23

A case of Chediak-Higashi syndrome diagnosed by its characteristic ocular findings is described. The appearance of the optic disc and the contrast between total (or nearly total) lack of pigment in the pigment epithelium and the relatively unaffected choroidal pigmentation are believed to be observations of paramount importance in the differential diagnosis by ophthalmoscopic examination. Electrophysiological tests of our case showed abnormal responses and a further deterioration of the ERG and VEP on repeated examinations. Electron microscopic study of a conjunctival biopsy revealed the presence of pathognomonic giant intracytoplasmic lysosomal granules in stromal fibroblasts and, thus, can be used as an adjunct test in suspected cases of Chediak-Higashi syndrome.
J Pediatr Ophthalmol Strabismus
PMID:Chediak-Higashi syndrome: ocular findings. 739

Twenty cases of the morning glory disc anomaly are reviewed and compared with a new case. The relationships between optic nerve hypoplasia, optic disc colobomas, and the morning glory disc are reviewed. Some pure disc colobomas and the morning glory syndrome may both be due to the optic nerve dysplasia. Associated neurological abnormalities are uncommon. The present case demonstrated a white pupillary light reflex, grossly diminished ERG, and absence of the VER.
J Pediatr Ophthalmol Strabismus
PMID:The morning glory disk anomaly: case report and literature review. 739 2

We described a family in which two siblings were suffering from Alport's syndrome. Both had identical ocular lesions: whitish-grey dots in the superficial layers of the retina mainly in the posterior pole. The visual acuity, the visual field, and the fundus fluorescein angiogram were normal. These lesions were associated with an impaired ERG. The ocular finding seems to be characteristic of the Alport's syndrome.
J Pediatr Ophthalmol Strabismus
PMID:Retinal lesions in Alport's syndrome. 741 40

In amblyopic subjects the electroretinogram in response to pattern stimuli (P-ERG) has been variously described as normal or abnormal. While this was found with a reversing stimulus pattern, we presently recorded the P-ERG by exposing the eye to a checkerboard pattern (element size 10, 30, or 60 min of arc) alternating with a uniform field of equal mean luminance (5 or 500 cd/m2). Eight subjects with anisometropic or squint amblyopia and ten normal subjects took part in the investigation. No differences in P-ERG amplitudes were found in amblyopic eyes in response to pattern offset. However, the P-ERG to pattern onset was diminished in the amblyopic eyes compared with the healthy fellow eyes and with the eyes of normal subjects. Both the early positive (p-q) and the late negative (q-r) components of the onset response were affected. Furthermore, the normal amplitude profile of the onset response (largest response at 30 min, smaller responses at 10 and 60 min, i.e. pattern tuning) was lost in the amblyopic eyes. According to Arden [2] and Korth [9], the P-ERG in response to pattern onset represents the pattern component and that in response to pattern offset, the luminance component. From this we conclude that in subjects with anisometropic and squint amblyopia the retinal information processing of pattern stimuli is disturbed, whereas that for luminance discrimination remains normal.
 ...
PMID:[Amblyopic eyes produce an abnormal electroretinogram in pattern presentation with the on-off technique]. 801 32


1 2 Next >>