[Functional visual explorations of Bardet-Biedl syndrome. A study of three cases]. / Exploration fonctionnelle visuelle dans le syndrome de Bardet-Biedl. A propos de 3 cas.

Laurence-Moon syndrome, which is very rare, and Bardet-Biedl syndrome, which is more frequent are now well-recognized as two distinct entities in pediatric neurology. Bardet-Biedl syndrome includes a number of common clinical signs it shares with Laurence-Moon syndrome but also with other syndromes, particularly Alströme syndrome. These signs are retinitis pigmentosa, mental retardation, obesity, and hypogonadism. Ophthalmological and electrophysiological examinations are essential for confirmation and correct diagnosis of Bardet-Biedl syndrome. We present three case histories. Our third case illustrates the possibility of below normal yet discernable electroretinogram amplitudes which do not infirm the diagnosis of Bardet-Biedl syndrome.

Functional assessment of macular hole surgery by scanning laser ophthalmoscopy.

OBJECTIVE: This study aimed to evaluate more precisely the benefit of macular hole surgery. DESIGN: The design was a prospective study of 40 eyes in 40 patients examined before and after surgery of full-thickness macular holes with a scanning laser ophthalmoscope (SLO). PARTICIPANTS: Forty patients with full-thickness macular holes participated. Three of the holes were stage 2, 23 were stage 3, and 14 were stage 4. INTERVENTION: The SLO examination consisted of macular imaging and assessment of macular function using tests produced by an acousto-optical modulator. MAIN OUTCOME MEASURES: Preferred retinal locus (PRL) was determined, visual acuity was measured, and scotoma was detected by microscotometry and by the line test (modified Watzke-Allen test). RESULTS: Anatomic success was achieved in 32 of 40 cases as assessed biomicroscopically. On SLO examination before surgery, macular holes were seen as a central bright round disc outlined by a thin dark edge surrounded by a dark ring and a less dark area with ill-defined limits. In all cases, the PRL was located on the upper edge of the hole, a scotoma was always detected inside the hole, and the line was seen as broken in 26 of 32 cases. After surgery, the hole closed completely in 25 of 32 eyes; it disappeared from 14 of these 25 eyes and was replaced by a dark or clear disc in 11. In the other seven successful cases, its size shrank and its edge flattened but remained faintly visible. The hole remained unchanged in eight cases. Eccentric PRL became central in 28 of 32 cases. The scotoma disappeared in 23 of 32 cases. The line was seen as continuous in 24 of 32 cases. Complete anatomic and functional successes were achieved in 19 of the 32 cases of macular hole closure. CONCLUSION: The SLO examination allows accurate assessment of the anatomic and functional results of macular hole surgery. Various degrees of functional success were recorded, depending on the test used.

Scanning laser ophthalmoscope imaging of fluorescein-labelled blood cells.

PURPOSE: To demonstrate the feasibility of a technique for the visualization by scanning laser ophthalmoscope (SLO) of fluorescein-labelled autologous leukocytes and platelets in retinal vessels. METHOD: Individual blood samples from rats and rabbits were centrifuged to isolate platelets and leukocytes, then passively labelled with fluorescein and reinjected into the same animal. An SLO was used to visualize and record cell displacement in the retinal circulation. Labelled platelets were analysed by flow cytometry. RESULTS: By SLO, platelets appeared as a heterogeneous particle flow, and individual leukocytes appearing as brighter spots could easily be traced. Flow cytometry showed that after labelling platelets were well individualized and their size was slightly increased. CONCLUSION: Circulating blood cells can be visualized in retinal vessels by a simple method consisting of passive labelling of autologous platelets and leukocytes by fluorescein. No platelet toxicity was detected. This method could be applied to the study of blood cell movement in human retinal vascular diseases.

[Static contrast sensitivity in idiopathic Parkinson disease]. / Sensibilité au contraste statique dans la maladie de Parkinson idiopathique.

During Parkinson's disease static contrast sensitivity (C.S.) abnormalities are linked to an impairment of the sensitive visual function. C.S. was tested in twelve parkinsonians and 12 controls without neurological and/or ophthalmological pathology, using a Colored Stripes Electronic Generator (GEPCO). Results for parkinsons showed a general deficiency over the spectrum of spatial frequencies, which was statistically significant and particularly pronounced for intermediate frequencies. This study was repeated for three patients: it showed threshold deterioration for two of them, correlated by evolution in the disease, and an improvement for the third patient after introduction of dopatherapy. C.S. is subjected to dopaminergic control. Among parkinsonians. C.S. deterioration may result in an operating failure of both the visual cortex and retina, and is improved by dopatherapy. The Static Gepco contrast sensitivity test is easy to reproduce and can be used easily to monitor the sensory visual defect in parkinsonian patients under treatment.

Electrophysiological exploration of visual function in mitochondrial diseases.

In a group of 10 children (ranging from 5 months to 15 years old) affected by diseases with mitochondrial dysfunction, 4 suffered from mitochondrial myopathy, 4 from mitochondrial encephalopathy and 2 from Friedreich's ataxia. The clinically detectable visual impairment consisted of 3 grey ocular fundi (the other 7 were normal) associated, in 2 subjects, with a mild nystagmus. Electrophysiological assessment, consisting of ERGs and flash VEPs, was systematically performed. The normal ERGs in all subjects confirmed the normal functioning of retinal electrogenesis. In contrast, the VEPs of 6 out of 10 subjects were modified: in 2 of the 4 subjects with mitochondrial myopathy, the VEPs had a hyperamplitude; in the 2 subjects with Friedreich's ataxia, the implicit time of the principal VEP peaks was increased, together with a hyperamplitude in 1 case; lastly, in 2 of the 4 subjects with mitochondrial encephalopathy, the VEPs were altered. These modifications reflected visual pathway conduction disorders with no clinical expression. Various underlying pathophysiological mechanisms possibly responsible for these modifications are discussed.

Eye fixations near corners: evidence for a centre of gravity calculation based on contrast, rather than luminance or curvature.

Subjects scanned line drawings of polygons in order to count the number of corners. The positions their eyes fixated were studied as a function of the size of the angle and whether the apex of the angle was present or absent. The results showed that the eyes tended to land at a position near the centre of gravity of the corner configurations. The observed landing positions were coherent with the hypothesis that the centre of gravity was calculated within an attentional spotlight centered on the apex of the corners, and that the calculation was based not on the total luminance distribution, nor on the distribution of energy in a neurophysiologically motivated curvature detector, but simply on the basis of a contrast detector.

Contribution of scanning laser ophthalmoscopy to the functional investigation of subjects with macular holes.

A study was designed to validate a functional investigation performed with the scanning laser ophthalmoscope before surgery for macular holes in 12 eyes: The assessment included fundus examination, a functional examination resulting in evaluation of the preferred retinal lows, visual acuity and recording of visual evoked potentials. The preferred retinal locus was evaluated by presenting a small square area, and visual acuity was determined by means of calibrated figures. The visual evoked potentials were evoked by three alternating checkerboards (check size, 30', 2 Hz) centered over the hole and seen at an angle of 6.5 x 6.5 degrees, 2.5 x 2.5 degrees and 6.5 x 6.5 degrees with central exclusion of 2.5 x 2.5 degrees. The appearance of the fundus visualized by scanning laser ophthalmoscopy consisted of a clear central disk corresponding to the hole, surrounded by a very dark ring, associated with a second, less dark ring with unclear margins. Fixation was unstable in one case with a visual acuity of 20/70. In 11 cases, fixation was localized to the superior retina with a visual acuity superior to 20/70. The visual evoked potentials evoked by 6.5 x 6.5 degrees were discernible in all 12 eyes; visual evoked potential by annular stimuli were discernible in 11 cases. The 2.5 x 2.5 degrees stimulus evoked no response in eight cases, proving the area of the hole was nonfunctional. A response was recorded in the four other cases, where the dimension of the holes was less than 2 degrees. The results of this scanning laser ophthalmoscopic assessment demonstrated a precise evaluation of the residual macular function in the cases of full-thickness macular holes.

Topographical characterization of normal visual evoked responses.

A study of visual evoked responses in a normal population was carried out using a high-precision programmable stimulator and an innovative brain mapping system which presents the overall activity on a three dimensional surface representing the human head. The interpolation method used gives a more precise computation of the potential value between electrodes. Two normal distributions of visual responses are presented which were identified while trying to topographically characterize visual evoked responses in order to construct a comparative data base. The proposed classification of two normal groups was verified by means of a statistical topographic analysis with the significance probability mapping technique.

Focal visual evoked potentials generated by scanning laser ophthalmoscope in patients with age-related macular degeneration treated by perifoveal photocoagulation.

Perifoveal laser photocoagulation has been proposed for the treatment of subfoveal neovascular membranes in age-related macular degeneration. We evaluated residual function in seven eyes of six treated patients by means of transient focal visual potentials evoked with a scanning laser ophthalmoscope. The site of the preferred retinal locus was determined. The modulation of the helium-neon laser beam generated three tests (a homogeneous 6 x 6 degrees square--offset and onset--and two alternating pattern checkerboards 6 x 6 degrees and 2.5 x 2.5 degrees 60', 2 Hz) projected onto the preferred retinal locus. The focal visual evoked potentials were recorded. One eye had an unstable fixation with no discernible focal visual evoked potentials. The other six eyes had a stable fixation located in the superior retina, temporally for the right eyes and nasally for the left eyes. The homogeneous 6 x 6 degrees square evoked discernible responses in all six patients. The two checkerboards evoked discernible responses in five of six patients. These results were compared with those recorded in four controls in whom the three tests were projected onto the same retinal areas as in the patients. Evoked responses were more often recorded in the preferred retinal locus of the treated patients with age-related macular degeneration than in the corresponding retinal areas of the controls. The scanning laser ophthalmoscope allowed us to control the site of stimulation in the patients' and controls' retinas. These preliminary results suggest that there may be a functional plasticity of the visual system after therapeutic laser-induced central scotoma.

Colored focal visual evoked potentials by cathode ray tube versus scanning laser ophthalmoscope.

We compared the focal visual evoked potentials obtained in 52 young subjects with normal vision, evoked by means of three alternating black/color checkerboards generated by a trichromic cathode ray tube (dominant wavelength, 514 nm; colorimetric purity, 0.45) and by means of a scanning laser ophthalmoscope (argon laser beam, 514 nm; colorimetric purity, approximately 1). These three checkerboards, with an area of 3.5 degrees x 3.5 degrees (stimulating the fovea), then with an area of 3.5 degrees x 3.5 degrees with a central exclusion of 1.5 degrees x 1.5 degrees (stimulating the perifoveola) and finally with an area of 1.5 degrees x 1.5 degrees (stimulating the foveola) were presented within a field (8 degrees x 8 degrees) of homogeneous luminance of 170 cd/m2 and 1500 cd/m2, respectively. Their check sizes were 30', with a reversal temporal frequency of 0.75 Hz. The transient focal visual evoked potentials recorded with these three stimuli generated by the two types of stimulators were clearly detected for at least 85% of subjects. Their characteristics (waveform, amplitude and culmination times of the different waves) were comparable, regardless of the stimulator used (cathode ray tube or scanning laser ophthalmoscope). These results suggest that, under these various conditions of luminance and colorimetric purity, the neurophysiologic circuits tested function in identical ways. The focal visual evoked potential signs, now clearly defined by means of stimuli generated by cathode ray tubes, therefore apparently can be applied to the focal visual evoked potential evoked by stimuli generated by the scanning laser ophthalmoscope.

[Statistical results of 700 electrophysiologic tests (ERG) in patients without ophthalmologic manifestations treated with synthetic antimalarials for rheumatologic or dermatologic disease]. / Bilan statistique de 700 examens électrophysiologiques (ERG) chez des patients sans signe ophtalmologique traités par antipaludéens de synthèse pour maladie rhumatologique ou dermatologique.

737 adapto-electroretinograms are registered on patients treated by chloroquine for a rheumatoid arthritis (RA) or for a lupus. Their ophthalmological examinations are completely normal. In term of the cumulative doses, the statistical results of the electrological characteristics of the different waves of the ERG issued from white, red and blue stimulations show: a great stability of the latencies of the "a" and "b" waves; a gradual decrease of the "b" wave amplitudes for the RA; a gradual increase of the "b" wave amplitudes till a level dose reached between 800 and 900 g and then a decrease of the "b" wave amplitudes, for the lupus. We discuss the electrophysiological criterions for a beginning intoxication. As a standard, for the R.A., it seems that a certain amount of "b" wave amplitude decrease could be admitted but the "a" and "b" wave latencies must be very constant. For the lupus, the hyper normality or at least stability of the "b" wave amplitude can be admitted till a dose around 800 g, associated to a good stability of the "a" and "b" wave latencies. Beyond these statistically established limits a beginning intoxication should be suspected.

[Visual electrophysiology in 101 children with encephalopathy]. / Etude de l'électrophysiologie visuelle chez 101 enfants encéphalopathes.

The electrophysiological recordings (E.R.G. associated with C.V.E.P.) performed on 101 children suffering from encephalopathy of different origins demonstrate a trend towards retinal integrity for subjects with encephalopathy and no suspicion of acid-base disorders. However a functional macular deficiency is frequently observed in encephalopathy with suspicion of acid-base disorders (encephalopathy due to intracranial hypertension or cardio-vascular disorders or metabolic deficiencies with acid-base disturbance). In these cases, a common pathophysiological substratum with disturbance of the acute or chronic acid-base equilibrium has been assumed. Over a period of time, in fixed encephalopathy, the electrophysiological results reflect a real but slow maturation of the visual pathways.

Data processing software for electrophysiological visual exploration.

The software we have developed originated after careful study of a routine functional visual processing session. It has three objectives: (i) to have available a large library of data processing programs; (ii) to add to these electrophysiological results, automatically transferred into a file, a clinical record card, creating a personalized file for study of visual characteristics of each patient; (iii) to allow programs providing statistical studies, creation of groups based on the same clinical features and various comparisons between files or groups. The database now contains 5000 different files and the results of its use are very encouraging. Some results are presented, especially those of a study on multiple sclerosis. The goal of the operation is the creation, in the future, of an expert system.

[Retinal protection using glasses filtering short wave lengths in patients with hereditary degenerative diseases. First electrophysiologic results]. / Protection rétinienne par des verres filtrant les courtes longueurs d'ondes chez des patients atteints de maladies hérédo-dégénératives. Premiers résultats électrophysiologiques.

The electrophysiological action of the ORMA RT glasses filtering the short and middle wavelengths are exposed through the first results of this work by retinitis pigmentosa patients. In 90% of the tested patients A.E.R.G. and V.E.P. are improved.

[Methods of stimulation in visual electrophysiology]. / Méthodes de stimulation en électrophysiologie visuelle.

After introducing physical and physiological stimulation parameters, the authors present different techniques and discuss their clinical usefulness for the investigation of the visual system: flashes: chiefly 'ganzfeld' stimulators (whole field or local stimulation). A solution using optic fibres incorporated in a sclero-corneal electrode built in our laboratory is presented; patterns: bars or chekerboards, especially on TV screens. Advantages and inconveniences of video images are discussed; other techniques: essentially endocular laser interference fringes, electrical stimulations, high level flashes for ERP and binocular studies, particularly dynamic random dot pattern EVP. In conclusion, the necessity for the careful control of stimulation characteristics and for a strategy suitable for clinical examination is discussed.

[Effect of fundus vagotomy on the gastric emptying of the solids and liquids of a meal in patients with and without postoperative duodenal ulcer recurrence]. / Effet de la vagotomie fundique sur la vidange gastrique des solides et des liquides d'un repas chez des patients avec et sans récidive ulcéreuse duodénale post-opératoire.

The effects of fundic vagotomy (FV) on gastric emptying in the solid and liquid phases of a meal were studied by an isotopic technique in 12 patients with duodenal ulcer. Postoperative results were compared with those obtained in the same subjects before FV and with control values obtained in an identical group of healthy subjects. Early gastric emptying (perprandial) of the two phases of the meal was enhanced by FV but the results failed to reach statistical significance. Gastric emptying of liquids, measured during the 3 h following the end of the meal, was not significantly modified by FV (half-emptying times: 62 +/- 5 min before FV, 75 +/- 8 min after and 65 +/- 7 min for controls). That of solids was significantly delayed by FV (per cent emptied by min: 0.50 +/- 0.02 p. 100 before FV, 0.40 +/- 0.03 p. 100 after, and 0.49 +/- 0.02 p. 100 for controls); this delay was found in all but one patient, but the difference was very slight. Eleven of the 12 investigated patients cured by the operation, were compared with 6 subjects presenting with post FV recurrence: gastric emptying rates of solids (0.41 +/- 0.02 p. 100/min and 0.47 +/- 0.07 p. 100/min) and half emptying times of liquids (72 +/- 10 min and 61 +/- 10 min) were not significantly different. Thus, the gastric emptying rate of an ordinary meal remains practically unchanged by FV and postoperative recurrences of duodenal ulcer cannot be explained by alteration of gastric evacuation.

Correlation functions in the analysis of visual evoked potentials.

Our "vision" laboratory has been working for several years on stimulation methods and data processing. We present here the results of an analysis of correlation functions between VEPs obtained by flashes and patterned stimulations. This study provides additional information about possible interpretations.