Biomedical subjects
Publications and source records attributed to A Pinckers.
The cone dystrophies can be subdivided into 3 functional stages: central cone disease, peripheral cone disease and diffuse cone disease, respectively. In the patient material of our clinic the sex distribution of patients presenting with te diffuse cone disease stage was abnormal: 22 males and 3 females. The authors suggest that the diagnosis X-linked cone dystrophy often is overlooked.
The purpose of this study was to evaluate the effect of Nd.YAG-laser treatment on retinal function. All the patients treated showed a temporary decrease of visual acuity immediately after treatment. The ERG and EOG recordings were identical before and after treatment. The Umazume-Ohta test showed temporary central or paracentral relative field defects in all cases investigated.
Some practical tips are given. With the help of the caps of the FM 100 Hue test simpler tests can be made, such as a Panel D-15 test. If 16 additional grey caps are procured the light sensitivity can also be determined. The durability of a colour specimen is doubled if a ring is fitted into the cap.
We studied 12 patients from six unrelated families with a syndrome that has an autosomal recessive pattern of inheritance and can be diagnosed from clinical, histologic, and biochemical characteristics. The four major symptoms are congenital cataract, hypertrophic cardiomyopathy, mitochondrial myopathy of voluntary muscles, and exercise-related lactic acidosis. The patients had bilateral and total cataract in the first weeks of life, underwent cataract surgery, and developed nystagmus and strabismus. Corrected visual acuity was lower than 20/40 in aphakic eyes. Patients were mentally normal, and at school age they visited a school for blind and visually impaired children. The majority of the patients developed axial myopia with myopic fundus changes; aphakic refraction usually was lower than 10.0 diopters after the first decade. The cardiac myopathy was progressive and the cause of premature death. Three of the 12 patients died in the neonatal period and six patients died in early adulthood.
This is a study conducted on thirty adult strabismic and anisometropic amblyopes using colour vision tests, the lightness discrimination test, mesopic vision and visual evoked responses, in order to determine the probable site of the amblyopic defect. The results showed that, in its progressed stages, amblyopia is associated with eccentric fixation, abnormal hue discrimination, disturbed lightness discrimination and mesopic vision, and attenuation of the amplitude of the pattern visual evoked response. It is suggested that these abnormalities result from a defect involving the extrastriate higher visual association areas 18 & 19 of Brodmann.
The authors describe a family with atypical sector shaped pigmentary dystrophy in two generations with transmission from father to son. A review of the literature is given.
The authors describe a family presenting with a patterned dystrophy of the retinal pigment epithelium. The aspect of the macular area is granular to reticular with a tendency to a butterfly shape. Visual functions are normal or near-normal. A review of the literature is given.
The Standard Pseudoisochromatic Plates part 2 are able to detect acquired blue-yellow color vision defects as well as acquired and congenital red-green color vision defects. One test plate might be age dependent. The value of 3 test plates is not clear.
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It is easy to make a shortened Lightness Discrimination Test (LDT) using the grey caps of the New Color Test (NCT). The shortened test shows the reduction in contrast sensitivity as well as the original LDT in squint amblyopia multiple sclerosis, optic neuritis and cerebral tumours. So it is a useful complement to the NCT.
Bull's eye maculopathy is a non-specific reaction of the posterior pole of the eye. The concentric dispersion of pigment occurs when the disease interferes with the function of the retinal pigment epithelium. The site of the primary process may be the bipolar cell layer, the receptor cell layer or the retinal pigment epithelium. By means of electrodiagnostic techniques and color vision testing the site of the primary lesion as a rule can be retraced.
The fundus abnormalities of Leber's congenital amaurosis are extremely variable, from normal to salt-and-pepper changes to typical retinitis pigmentosa. A less commonly seen appearance is that of multiple, irregular shaped, yellowish white flecks deep in the midperipheral retina in a periarteriolar distribution. The nasal fundus as well as the posterior pole are spared. Such a case is presented along with a four-year follow-up together with the fluorescein angiographic findings. The flecks appear to be specific for this entity.
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This paper is a report on Neuronal Ceroid Lipofuscinosis (NCL) in The Netherlands (synonyms: Batten disease, Jansky-Bielschowsky disease, Batten-Mayou disease, Stock-Spielmeyer-Vogt disease). Discussed are the late infantile type with predominant accumulation of lipofuscin in the form of curvilinear bodies (Jansky-Bielschowsky) and the juvenile type with accumulation of lipofuscin in the form of fingerprint- and rectilinear profiles (Batten-Mayou disease and Stock-Spielmeyer-Vogt disease or F-type of NCL).
Acquired colour vision defects are directly related to the fixation mode: blue-yellow defects in foveolar fixation, blue-yellow or red-green defects in eccentric fixation. The primary localization of a disease can be retraced from the degree of cone damage. Optic nerve diseases essentially lack signs of cone damage. Processes at the level of the choriocapillaris/retinal pigment epithelium induce a non-selective receptor impairment. There are minor signs of cone damage. In cone dystrophies there is selective cone damage. Scotopization indicates a relatively well-preserved rod function.
In unilateral injuries, the tap to the eye by a penetrating foreign body is sufficient to depress the electro-oculogram (EOG). Total recovery is time-consuming. During the recovery period, complications such as siderosis and detachment may arise, acting with predilection on the light-sensitive EOG component. Blunt traumata and possibly surgical opening of the eyeball predominantly decrease the light-insensitive component of the EOG.
In untreated rheumatoid arthritis 20% of the patients has a EOG Lp/Dt ratio lower than the lower 5% limit calculated for normal patients. The EOG is more often disturbed in a long-standing chloroquin-induced retinopathy than in the acute phase of intoxication, the reason being progression from a maculopathy to a tapeto-retinal degeneration. The subnormal EOG in rheumatoid arthritis might be due to an auto-immune process against rhodopsin and uveal pigment. Withdrawal of synthetical antimalarial agents in cases suspected of an early drug-induced retinopathy based on EOG subnormality may enhance the underlying rheumatoid process thus causing a further decrease of the EOG. The EOG therefore is not a method of choice in detecting an early chloroquin-induced retinopathy.
The minimum requirements for a reliable study of (acquired) defects of colour vision have been formulated by Verriest. Taking these minimum requirements as a guide, about 200 patients were selected. For the differential diagnosis of disorders of the retina and the optic nerve the determination of the neutral zone and examination with the anomaloscope are important. This is true for both red-green and blue-yellow defects.