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Two Greek female patients with Aicardi's syndrome are presented. The clinical picture, electroencephalogram, computer axial tomography scan, and the ophthalmological findings in these patients are described. The ocular features are emphasised because they can establish the diagnosis of this rare syndrome.
Of 76 children with histiocytosis X 18 had orbital involvement, and four developed additional neuro-ophthalmic complications. No instance of intraocular involvement was detected. Among those patients with ophthalmic involvement the main problems were bilateral or unilateral proptosis, ptosis, papilloedema, optic atrophy, and seventh nerve palsy. Only one patient developed a severe visual defect. Management of the ophthalmological complications depends not only on the extent of the orbital disease but also on the degree of systemic involvement. Overall management by a paediatric oncologist is mandatory.
In reports on ophthalmological research the results of measurements on the eye are often expressed as mean and standard deviation based on m patients, n eyes (n greater than m). This approach leads to t tests that are invalid because the measurements on the two eyes of one subject are usually related, not independent. In a simulation study involving intraocular pressure data analysed in this way, the null hypothesis of no difference between groups was rejected at a nominal alpha = 0.05 level in 39 out of 200 simulations; thus the true alpha was nearly 0.2. This approach is excessively prone to produce false positive results.
This paper describes the ophthalmological features of 150 patients with idiopathic retinal vasculitis, 67 of whom had isolated retinal vasculitis (RV) and 83 had RV associated with systemic inflammatory disease (RV + SID). The diagnosis of retinal vasculitis was made by ophthalmoscopy and fluorescein angiography, and patients with any identifiable cause (infection, ischaemia, or malignancy) were excluded from the study. Patients with isolated RV tended to have peripheral vascular sheathing, macular oedema, and diffuse capillary leakage. Those with RV accompanying Behçet's disease often had branch vein retinal occlusions and retinal infiltrates together with macular oedema and diffuse capillary leakage; the retinal infiltrates were pathognomonic for Behçet's disease. In sarcoidosis the retina typically showed features of periphlebitis associated with focal vascular leakage. Patients with uveomeningitis, multiple sclerosis, arthritis, or systemic vasculitis showed diffuse retinal capillary leakage associated with a mixture of the other features. Poor visual function was particularly associated with macular oedema and branch vein retinal occlusion, while the retina appeared to 'withstand' the impact of vascular sheathing, periphlebitis, or neovascularisation alone. Within the limitations of a point prevalence study it was concluded that different patterns of retinal vasculitis occur in different systemic inflammatory diseases, and that in isolated retinal vasculitis there is a particular association between peripheral vascular sheathing, macular oedema, and diffuse capillary leakage. In Part 2 we describe the results of examining the sera of these patients for the presence of antiretinal antibodies and circulating immune complexes.
BACKGROUND: Numerous studies have reported an increased risk of refractive errors in prematurely born infants, but only few have been long-term and strictly population based. METHODS: A 3.5 year ophthalmological long term follow up of 248 preterm infants was performed. The infants had been included in a previous epidemiological study of retinopathy of prematurity (ROP) (birth weight < or = 1500 grams). The incidence of refractive errors and development of refraction were studied, based on retinoscopies at 6 and 30 months of corrected age. RESULTS: The overall incidence of myopia was 8% at 6 months, of which 35% was transient, and 10% at 30 months. Of the cryotreated infants, 30-40% were myopic at both retinoscopies. The incidence of astigmatism was 52% at 6 months and 26% at 30 months. Astigmatism was associated with ROP, but not with cryotreated ROP itself. Astigmatism "against the rule" was commoner than astigmatism "with the rule". Anisometropia occurred in 6.5% of the infants at 6 months and in 8.4% at 30 months. The incidence of anisometropia was higher in eyes with ROP, particularly in cryotreated eyes, which tended to have high and persistent anisometropia. CONCLUSION: The risk of refractive errors is higher in preterm infants than in infants born at term, and also prematurely born infants without ROP do run an increased risk of having myopia and anisometropia. We recommend follow up examinations with retinoscopy for all infants included in screening programmes for ROP.
OBJECTIVE: To evaluate the nature and incidence of pathological ocular conditions in boxing. METHODS: A group of 20 active, elite, amateur, asymptomatic Turkish boxers were examined and compared with a control group composed of 20 age matched sportsmen who were not boxers. The boxers had been actively boxing for 5-20 years (mean 9.65), were aged 16-34 (mean 22.25), and weighed 51-91 kg (mean 73.07). They had been involved in 67-500 fights (mean 143.8), with 5-40 losses (mean 17.75). All were championship title holders at the national, European, Olympic, or World level. RESULTS: An atrophic retinal hole was found in one boxer, which was treated with laser prophylaxis. In this series, the incidence of traumatic eye injuries was much lower than in the literature. Possible reasons are their young age, their division being mainly lightweight or middleweight, few fight losses, being elite boxers, their fighting distance being near or distant, protective methods, and racial factors. CONCLUSION: Boxing does not appear to be as hazardous to the eyes as previously reported, but prospective, longitudinal studies need to be carried out, including boxers of all divisions, ages, boxing experience, and level, and using various methods of fighting and protection. It should be made mandatory for all boxers to have a complete ophthalmological examination at the beginning of their careers and periodically thereafter.
OBJECTIVE: To assess neurotological status and its ophthalmological correlates in Behçet's disease. STUDY DESIGN: A cross sectional study in which 17 patients with Behçet's disease and 13 healthy controls were included. PATIENTS AND METHODS: The patients were divided into two groups: those with and without end stage ocular involvement. Neurotological status was evaluated with audiological tests (pure tone and speech audiometry, tympanometry, short increment sensitivity index, tone decay, and evoked response audiometry), and Dix-Halpike positional testing. RESULTS: The positional test was normal. The audiological test results of the patients with and without end stage ocular involvement were not significantly different (p>0.05). A sloping audiogram with bilateral symmetric and mild sensorineural hearing loss was the main audiogram obtained. There was no relationship between the presence or absence of the end stage ocular involvement and the otological parameters studied excluding I-III interval on evoked response audiometry (p>0.05). CONCLUSION: Although the main underlying pathogenetic factor in Behçet's disease is the autoimmune vasculitis, the mechanisms involved in the pathogenesis of neurological and ocular damage may be different. Alternatively, the differential involvement of certain organ systems may not reflect the nature of the disease process itself, but rather the manner in which each organ responds to injury.
Fifty-three orbital lesions of various types were studied by computer tomography to assess the capabilities and limitations of this technique. Although CT scanning in ophthalmology is in its infancy, results showed that it is useful in the investigation of a number of pathological conditions, including congenital anomalies, arteriovenous malformations, bone lesions, trauma, inflammation, granulomas, pseudotumors, epidermoid cysts, and tumors arising in or near the optic nerve, behind the optic bulb, or in the lacrimal region, as well as changes following eye surgery.
The present status of laser application in clinical ophthalmology is breifly reviewed. The potentials of tunable dye lasers as retinal photocoagulators are discussed. Selective irradiation of ocular tissues over the full visible spectrum and the simplicity and reliability of recently developed waveguide lasers are the most attractive features of these lasers. Waveguide dye lasers have permitted to set up very compact and simple retinal photocoagulators, with improved output intensity stability and nearfield distribution uniformity. Preliminary results obtained with pulsed Rhodamine 6G laser show that good retinal photocoagulations are obtained at very low output energies.
On the basis of his studies on Fabry's disease, the dyscephalic syndrome of François, ligneous conjunctivitis and macular dystrophy of the cornea, the author stresses the importance of heredity in ophthalmology.
A case of a 6-year-old boy is referred. Ophthalmological findings suggested subacute sclerosing panencephalitis. Only very distinct neurological alterations could be found. Positive measle titers in serum and cerebrospinal fluid and positive subacute sclerosing panencephalitis factors proved the diagnosis. While the first eye showed a macular scar and mild optic neuritis the second eye developed the acute stage of retinal lesion and immediate visual loss. Differential diagnosis and possible therapeutical aspects are discussed.
16 healthy and ophthalmologically normal truck drivers are tested with a battery of tests according to the routine described in the following paper. This includes isopter perimetry, colour discrimination, recovery of sensitivity during dark adaptation and refraction at both far and near points, in order to record night myopia as well as night presbyopia. The ranges of individual variability are reported. As far as refraction is concerned, it seems of interest to stress the individual differences in a sort of "transient anisometropia" under incoming twilight.
The subjects of the study are 74 patients with craniopharyngiomas treated at the Clinic of Neurosurgery, Bulgarian Medical Academy, within the period 1960-1989. Fifty of the patients are children and 24 adults. The diagnosis of all patients is made on the base of clinical, X-ray and histological studies. The ophthalmologic state was studied in all patients, prior to operation, via routine methods. Reduced visual acuity was established in 69.6% of the children and 81.8% of the adults. The presence of congestive optic papilla was diagnosed in 22% of the children and 12.5% of the adults. Optic atrophy was established in 62.5% of the adults and 52% of the children. Various defects in the visual field were established in 67.7% of the children and 81.8% of the adults with the predomination of bitemporal defects. The use of highly informative conventional and modern X-ray methods is recommended in all patients suspected of compression of the visual pathways.
Biotinidase deficiency is an autosomal recessively inherited metabolic disorder characterized by neurological and cutaneous manifestations and metabolic abnormalities. We studied 78 symptomatic children and found that 51% had ophthalmologic abnormalities. These include infections (30%), optic neuropathies and visual disturbances (13%), motility disturbances (13%), retinal pigment changes (4%) and pupillary findings (1%). The most commonly reported findings are optic atrophy and keratoconjunctivities. Although the disorder can be effectively treated with biotin therapy, untreated children are at risk of developing permanent neuro-ophthalmic damage.
The authors analysed accidents of ophthalmological interest obtaining information from the database of the INAIL (National Insurance Institute for Professional Casualties) concerning the period of 1986-1991. The INAIL registers all casualties that cause work disabilities exceeding 3 days. The investigation included all job types grouped into two major categories: agriculture (16% of all insured labour) and industry/craftsmanship (84%). Over 78% of the cases examined were in the industry/craftsmanship category. Approximately 22% of the cases were in the agriculture category. Every year in Italy, about 6% of regularly employed workers suffer casualties. The incidence of casualties of ocular interest has been a stable 0.37% in the years examined; 2.88% of these casualties produce permanent consequences (1/10,000 workers per year). The risk in 3 times higher in agriculture.
The authors report two patients with prolonged metabolic delirium presumably initiated by systemic absorption of homatropine from a topical ophthalmologic solution. Anticholinergic toxicity was confirmed by muscarinic radioreceptor assay in both cases and by quantitative EEG in one case.
Patients with Parkinson's disease (PD) commonly complain of impaired visual function and difficulty reading, despite normal visual acuity. Although previous studies have evaluated contrast sensitivity, color vision, visuospatial processing, visual hallucinations, and ocular movements, none has systematically evaluated the ocular complaints and ocular findings of PD patients. Thirty patients with early untreated PD and 31 control subjects without neurologic or known ocular diseases were ophthalmologically evaluated for the frequency of visual complaints, dry eyes, blepharitis, visual hallucinations, reduced blink rate, blepharospasm, and convergence insufficiency. Ocular complaints suggesting ocular surface irritation, altered tear film, visual hallucinations, blepharospasm, decreased blink rate, and decreased convergence amplitudes were more common in PD patients than in control subjects. These findings likely account for many of the visual difficulties commonly encountered by PD patients. These ocular abnormalities frequently respond to treatment.
Four cases in two generations of a Japanese family are described as affected by an autosomal dominant-inherited cerebellar ataxia, pyramidal and extrapyramidal signs, and abnormal ocular movements, resembling Machado-Joseph disease. The neuro-ophthalmologic examination suggests the involvement of multiple motor control systems. The neuropathologic examination of one case showed neuronal loss in the subthalamic nuclei, pallidum, dentate nuclei of cerebellum, and red nuclei, which is distinct from Machado-Joseph disease.