Biomedical subjects
Publications and source records attributed to A Pinckers.
Pathologic scotopization, an important symptom of retinal disease, can be studied by means of the Nagel II anomaloscope. This method is called the micro-screw method. The micro-screw method was performed in 14 congenital and 13 acquired colour vision defective individuals. The method proves to be useful in detecting symptoms of rod intrusion in colour vision under photopic conditions.
UNLABELLED: Eight patients (four men, four women), with low visual acuity caused by autosomal dominant cystoid macular dystrophy, were treated daily with oral 250 mg dose acetazolamide. Treatment ranged from two to 17 months. None of these eight patients had improvement of visual acuity of more than 0.1. CONCLUSION: Treatment with 250 mg acetazolamide appears not to be an effective therapy for cystoid macular oedema in dominant cystoid macular dystrophy. The electroretinography b-wave/a-wave ratio was normal. The primary lesion in dominant cystoid macular dystrophy remains obscure.
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Examination of a great number of patients resulted in the depth localisation theory. The combination of this theory with the fixation-eccentrisation theory is clinically useful: acquired colour vision defects can be subdivided by the fixation mode and by signs of receptor damage. There are indications that in multiple sclerosis the slowly progressive cases present with more receptor damage than the acute cases.
Two brothers, 20 and 21 years of age, are reported who had a circumscribed area of atrophy of the pigment epithelium, choriocapillaris and part of the choroidal vessels in the posterior pole; they were otherwise in good health. In addition, the optic discs, particularly in the older brother had hypoplastic characteristics. Both patients were known to have had low visual acuity and nystagmus since early childhood. Electro-oculography was normal. Electroretinography and color vision tests were subnormal in both patients. Although the morphological picture showed features similar to central areolar choroidal dystrophy, familial bilateral macular colobomata, serpiginous choroiditis and some cases of cone dystrophy, there are clinical and electrophysiological reasons for differentiating the cases presented here from these other diseases.
The Berson test for blue cone monochromatism discriminates X-linked blue cone monochromatism from achromatopsia but not from X-linked progressive c dystrophy.
With a routine clinical colour vision test battery we found scotopization in 32% of retinal diseases presenting with pseudoprotanomaly as sign of an acquired type III blue-yellow colour vision defect. In blue-yellow colour vision defects of retinal origin scotopization is a transient phenomenon, present in early stages of the disease, but it is not an obligatory finding. There is no evident relationship between visual acuity and scotopization.
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The term scotopization refers to the intrusion of rod activity in colour vision when assessed under photopic observation conditions. Scotopization is an important symptom of cone dystrophies. The detection of scotopization is not easy. With the Nagel-II anomaloscope scotopization can be detected in two ways. One method is new and this method is described in the present paper.
The authors report on a case of congenital bilateral protruding corneal opacities. Clinical and histological examination was consistent with a diagnosis of Peters' anomaly with a defect in the endothelium and Descemet's membrane but without abnormalities of iris and lens. Corneal grafting was performed on both eyes. During a follow-up period of one year the corneas have remained clear. However, surgical intervention for glaucoma has been necessary.
The authors examined a family in which father and son presented with pars planitis. Both of them and the paternal grandparents were HLA typed; no association between HLA antigens and pars planitis was found. Present and previous data suggest a relation between pars planitis and allergic predisposition.
More than half of the cases with complaints of chromatopsia had recent-onset retinal pathology. Erythropsia due to bright (sun-)light is a relatively common finding in aphakia and pseudophakia. UV-coated intraocular lenses do not provide complete protection. Cerebrovascular chromatopsia usually occurs in transient attacks.
The authors describe a patient with bilateral severe disruption of the epithelium while wearing MMA/VP70 contact lenses. Withdrawal of the contact lenses and the contact lens care system resulted in complete recovery. Later on Boston IV contact lenses with another care system were fitted successfully.
The electro-oculogram (EOG) in the 64 patients with a melanoma of the choroid or ciliary body was compared to the EOG in 11 patients with choroidal metastasis, 11 patients with choroidal naevi and 27 patients with a rhegmatogenous retinal detachment. Using the Dt and the Lp/Dt-ratio, 87.5% of the melanomas could be diagnosed correctly whereas choroidal naevus and retinal detachment were diagnosed correctly in 72.7% and 70.4% of the cases respectively. Choroidal metastases never were classified correctly. Accompanying retinal detachment, tumour volume or a break through Bruch's membrane had no influence on the EOG in the melanoma patients. An important advantage of the method is that it can be used irrespective of the condition of the other eye. Combined with ophthalmoscopy, ultrasonography and fluorescein-angiography the EOG can be an additional aid in the differential diagnosis of malignant melanoma of the choroid and ciliary body.
The authors examined a patient presenting with congenital sensory neuropathy with selective loss of small myelinated nerve fibres. The appearance of (bilaterial) keratitis or corneal ulceration in early childhood is strongly suggestive of congenital corneal anaesthesia. Concomitant symptoms such as anisocoria, abnormal pupillary reaction, diminished tear production and disturbed sensibility to pain and temperature point to a generalized disease: one of the hereditary sensory and autonomic neuropathies. In order to establish a definite diagnosis, elaborate neurological examination, including ultrastructural study of a muscle-nerve biopsy, is required. Tarsorrhaphy, therapeutic flushfitting PMMA scleral lenses and hydrophilic HEMA contact lenses are advocated, in order to protect the cornea. The results with high-water-content hydrophilic contact lenses are promising, those of keratoplasty limited.
Pigmentations and depigmentations, accompanied or not by yellow subretinal lipofuscin accumulations, are the ophthalmologically visible manifestations of dystrophies of the retinal pigment epithelium. The pigmentations may or may not become confluent and form concentric, butterfly-shaped or reticular configurations. Different patterns of pigment migration may occur in a family and even in one individual. Hence these dystrophies are called pattern(ed) dystrophies of the retinal pigment epithelium. The visual functions are relatively well-preserved, although severe visual impairment is not excluded. The pattern(ed) dystrophies are inherited as an autosomal dominant, an autosomal recessive or an X-linked recessive trait. The author suggests that some cases diagnosed as atypical pigmentary dystrophies with near-normal visual functions probably are dystrophies of the retinal pigment epithelium.
We describe six related patients presenting with an autosomal dominantly inherited pattern dystrophy of the retinal pigment epithelium, significantly abnormal electro-oculogram and minor colour vision abnormalities. There is a continuum of variable phenotypic expression within the pattern dystrophies of the retinal pigment epithelium.
Whitish dots in the stroma of the cornea resembling the cloudy dystrophy were observed in 4 patients wearing HEMA contact lenses; a lattice-like corneal pattern was seen in another patient wearing HEMA contact lenses. There were no complaints. Visual acuity was normal. Corneal sensitivity was normal or reduced. The pseudo-dystrophies vanished after replacement of the HEMA lenses by Boston IV material.