Ulrich Schiefer

Does visual restitution training change absolute homonymous visual field defects? A fundus controlled study

Aim: To examine whether visual restitution training (VRT) is able to change absolute homonymous field defect, assessed with fundus controlled microperimetry, in patients with hemianopia. Methods: 17 patients with stable homonymous visual field defects before and after a 6 month VRT period were investigated with a specialised microperimetric method using a scanning laser ophthalmoscope (SLO). Fixation was controlled by SLO fundus monitoring. The size of the field defect was quantified by calculating the ratio of the number of absolute defects and the number of test points; the training effect E was defined as the difference between these two ratios before and after training. A shift of the entire vertical visual field border by 1° would result in an E value of 0.14. Results: The mean training effect of all right eyes was E = 0.025 (SD 0.052) and all left eyes E = 0.008 (SD 0.034). In one eye, a slight non-homonymous improvement along the horizontal meridian occurred. Conclusions: In one patient, a slight improvement along the horizontal meridian was found in one eye. In none of the patients was an explicit homonymous change of the absolute field defect border observed after training.

Stereotactic fractionated irradiation of optic nerve sheath meningioma: a new treatment alternative

Background: Primary optic nerve sheath meningioma (ONSM) is a rare but almost invariably blinding tumour when its natural history is observed in a “wait and see” strategy. Surgery has hitherto only been advocated in case of progressive disease involving intracranial structures, as it leads to iatrogenic blindness in the overwhelming majority of cases. Therefore, treatment options bearing lesser risk of functional deterioration are highly desirable, both in cases of intracranial involvement as well as during earlier phases of the disease which are currently generally left untreated. The authors report the outcome of the largest series of patients to date treated by stereotactic fractionated irradiation as a new treatment approach in ONSM at all stages. Methods: 15 patients (16 nerves) underwent stereotactic fractionated conformal irradiation with a total dose of 54 Gy, using standard fractionation. Main outcome parameters included visual acuity and visual field, as well as three dimensional remission as documented by imaging. Results: Tumour control was confirmed in all 15 patients undergoing stereotactic fractionated conformal irradiation (mean follow up 37 (range 12–71) months). No patient developed functional deterioration during or after treatment. Moreover, visual acuity improved by more than two lines in one patient and the visual field improved in six cases. Visual outcome in the other patients remained unchanged. There were no significant side effects of radiation therapy. Conclusion: These data provide convincing evidence that stereotactic fractionated conformal irradiation is an effective treatment option for primary ONSM with minimal treatment related morbidity. It should therefore be considered as therapeutic option both in early stage ONSM where surgery cannot be justified as well as in later stages, where surgery is so far considered the first line approach.

Stereotactic fractionated radiotherapy in patients with optic nerve sheath meningioma.

PURPOSE To evaluate the effectiveness of stereotactic fractionated radiotherapy (SFRT) in the treatment of optic nerve sheath meningioma (ONSM). METHODS AND MARERIALS: Between 1994 and 2000, a total of 39 patients with either primary (n = 15) or secondary (n = 24) ONSM were treated with SFRT and received a median total tumor dose of 54 Gy using 1.8 Gy/fraction. RESULTS The radiographic response to SFRT was documented in all patients as stable disease (no change) except for 1 patient with a partial response. After a median follow-up of 35.5 months, all patients with ONSM were alive without recurrence. The visual fields and visual acuity were improved in 6 of 15 and 1 of 16 examined eyes in patients with primary ONSM, respectively, and in 6 of 24 and 7 of 26 examined eyes in patients with secondary ONSM, respectively. Stable visual fields and visual acuity was observed in 8 of 14 and 15 of 16 patients with primary ONSM, respectively, and in 17 of 24 and 19 of 26 patients with secondary ONSM, respectively. Except for reversible alopecia and erythema, no other SFRT-related toxicity was observed. CONCLUSION SFRT represents a very effective and low-toxic treatment modality for ONSM. Despite a median follow-up of 3 years, this series of primary ONSM holds promise for future studies. It adds substantial evidence that SFRT may definitely become a standard treatment approach in selected cases of ONSM.

A mathematical description of nerve fiber bundle trajectories and their variability in the human retina

We developed a mathematical model wherein retinal nerve fiber trajectories can be described and the corresponding inter-subject variability analyzed. The model was based on traced nerve fiber bundle trajectories extracted from 55 fundus photographs of 55 human subjects. The model resembled the typical retinal nerve fiber layer course within 20 degrees eccentricity. Depending on the location of the visual field test point, the standard deviation of the calculated corresponding angular location at the optic nerve head circumference ranged from less than 1 degrees to 18 degrees , with an average of 8.8 degrees .

Spatial pattern of glaucomatous visual field loss obtained with regionally condensed stimulus arrangements.

PURPOSE To assess the spatial distribution of glaucomatous visual field defects (VFDs) obtained with regionally condensed stimulus arrangements. METHODS Sixty-three eyes of 63 glaucoma subjects were examined with threshold-estimating automated static perimetry (full threshold 4-2-1 dB strategy with at least three reversals) on an automatic campimeter or a full-field perimeter. Stimuli were added by the examiner to regionally enhance spatial resolution in regions that were suspicious for a glaucomatous VFD. These regions were characterized by contiguous local VFDs, attributable to the retinal nerve fiber bundle course according to the impression of the examiner. The added stimulus locations were subsets of a predefined, dense perimetric grid. All VFD locations with P < 0.05 (total deviation plots) were assessed by superimposing the visual field records of all participants. RESULTS Glaucomatous VFD loss occurred more frequently in the upper than in the lower hemifield, with a typical retinal nerve fiber-related pattern and a preference of the nasal step region. More than 50% of the eyes with predominantly mild to moderate glaucomatous field loss showed defective locations in the immediate superior paracentral region within an eccentricity of 3°. CONCLUSIONS Conventional thresholding white-on-white perimetry with regionally enhanced spatial resolution reveals that glaucomatous visual field loss affects the immediate paracentral area, especially the upper hemifield, in many eyes with only mild to moderate glaucomatous visual field loss. Detailed knowledge about the spatial pattern and the local frequency distribution of glaucomatous VFDs is an essential prerequisite for creating regionally condensed stimulus arrangements for adequate detection and follow-up of functional glaucomatous damage.

Visual field constriction and electrophysiological changes associated with vigabatrin

Purpose: We investigated functional, morphological and electrophysiological changes in patients under anti-epileptic therapy with vigabatrin (VGB), a GABA aminotransferase inhibitor. Methods: 20 epileptic patients treated with vigabatrin (age range 25–66 years) were enrolled in this study. The referrals were made by the treating neurologist, based on suspected or known visual field changes in these patients. Two patients had vigabatrin monotherapy, 18 patients were treated with vigabatrin in combination with other antiepileptic drugs. None of the patients reported visual complaints. Patients were examined with psychophysical tests including colour vision (Farnsworth D15), dark adaptation threshold, Goldmann visual fields and Tuebingen Automated Perimetry (90°). A Ganzfeld ERG and an EOG following the ISCEV standard protocol were also obtained. Additionally, all patients were examined with the VERIS multifocal ERG including recordings of multifocal oscillatory potentials. Results: Visual acuity, anterior and posterior segments, colour vision and dark adaptation thresholds were normal in all patients. Of 20 patients, 18 presented visual field constriction. All patients with visual field defects revealed altered oscillatory potentials waveforms in the ERG, especially in those patients with marked visual field defects. Multifocal oscillatory potentials were also delayed in those patients. In some patients a delayed cone single flash response (6/20), a reduced mERG amplitude (12/20) and a reduced Arden ratio (9/20) were found. Conclusions: The present data indicate an effect of vigabatrin on the inner retinal layers. Since abnormalities of the oscillatory potentials were seen in all patients with visual field defects a dysfunction of GABA-ergic retinal cell transmission might be assumed.

Vigabatrin and epilepsy: lessons learned.

Summary:  Purpose: The risk factors for visual field loss attributable to vigabatrin (VAVFL) are equivocal. This multinational, prospective, observational study aimed to clarify the principal/major factors for VAVFL.

Comparison of the new perimetric GATE strategy with conventional full-threshold and SITA standard strategies

PURPOSE A new, fast-threshold strategy, German Adaptive Thresholding Estimation (GATE/GATE-i), is compared to the full-threshold (FT) staircase and the Swedish Interactive Thresholding Algorithm (SITA) Standard strategies. GATE-i is performed in the initial examination and GATE refers to the results in subsequent examinations. METHODS Sixty subjects were recruited for participation in the study: 40 with manifest glaucoma, 10 with suspected glaucoma, and 10 with ocular hypertension. The subjects were evaluated by each threshold strategy on two separate sessions within 14 days in a randomized block design. RESULTS SITA standard, GATE-i, and GATE thresholds were 1.2, 0.6, and 0.0 dB higher than FT. The SITA standard tended to have lower thresholds than those of FT, GATE-i, and GATE for the more positive thresholds, and also in the five seed locations. For FT, GATE-i, GATE, and SITA Standard, the standard deviations of thresholds between sessions were, respectively, 3.9, 4.5, 4.2, and 3.1 dB, test-retest reliabilities (Spearmans rank correlations) were 0.84, 0.76, 0.79, and 0.71, test-retest agreements as measured by the 95% reference interval of differences were -7.69 to 7.69, -8.76 to 9.00, -8.40 to 8.56, and -7.01 to 7.44 dB, and examination durations were 9.0, 5.7, 4.7, and 5.6 minutes. The test duration for SITA Standard increased with increasing glaucomatous loss. CONCLUSIONS The GATE algorithm achieves thresholds that are similar to those of FT and SITA Standard, with comparable accuracy, test-retest reliability, but with a shorter test duration than FT.

Visual field loss in patients with refractory partial epilepsy treated with vigabatrin: final results from an open-label, observational, multicentre study.

Background: Use of the antiepileptic drug vigabatrin is associated with an elevated risk of visual field loss.Objective: To determine the frequency of, and risk factors for, vigabatrin-attributed visual field loss (VAVFL) in the setting of a large-scale, multinational, prospective, observational study.Study design: A comparative, open-label, parallel-group, multicentre study.Setting: Hospital outpatient clinics at 46 centres in five countries.Patients: 734 patients with refractory partial epilepsy, divided into three groups and stratified by age (8–12 years; >12 years) and exposure to vigabatrin. Group I comprised patients treated with vigabatrin for ≥6 months. Group II comprised patients previously treated with vigabatrin for ≥6 months who had withdrawn from the drug for ≥6 months. Group III comprised patients never treated with vigabatrin. Patients underwent perimetry at either 4- or 6-month intervals, for up to 36 months. Visual field outcome was evaluated masked to drug exposure.Intervention: Perimetry.Main outcome measure: The visual field outcome at each of four analysis points: (i) at enrolment (i.e. baseline, all patients); (ii) for patients exhibiting a conclusive outcome at the initial visual field examination; (iii) for patients exhibiting at least one conclusive outcome to the visual field examinations; and (iv) at the last conclusive outcome to the visual field examinations.Results: Of the 734 patients, 524 yielded one or more conclusive visual field examinations. For Group I, the frequency of VAVFL at the last conclusive examination was 10/38 (26.3%) for those aged 8–12 years and 65/150 (43.3%) for those aged >12 years. For Group II, the respective frequencies were 7/47 (14.9%) and 37/151 (24.5%). One case resembling VAVFL was present amongst the 186 patients in Group III at the last conclusive examination. The frequency of VAVFL in Groups I and II combined was 20.0% for those aged 8–12 years and 33.9% for those aged >12 years. VAVFL was associated with duration of vigabatrin therapy (odds ratio [OR] up to 15.2; 95% CI 4.4, 51.7), mean daily dose of vigabatrin (OR up to 26.4; 95% CI 2.4, 291.7) and male gender (OR 2.51; 95% CI 1.5, 4.1). VAVFL was more frequently detected with static than with kinetic perimetry (OR up to 0.43; 95% CI 0.24, 0.75).Conclusions: Since the probability of VAVFL is positively associated with treatment duration, careful assessment of the risk-benefit ratio of continuing treatment with vigabatrin is recommended in patients currently receiving this drug. All patients continuing to receive vigabatrin should undergo visual field examination at least every 6 months for the duration of treatment. We recommend two-level (three-zone), gradient-adapted, suprathreshold static perimetry of the peripheral field together with threshold perimetry of the central field out to 30° from fixation. The frequency of ophthalmological and perimetric examinations should be increased in the presence of VAVFL.

Effect of visual restitution training on absolute homonymous scotomas.

The authors examined 16 patients with stable homonymous visual field defects (HVFDs) with static automated perimetry (SAP). Training effect E was defined as difference of the proportions of absolutely defective locations in all test locations, before and after visual restitution training (VRT). E was 0.05 ± 0.05 (mean ± SD). The authors observed a relevant training effect (E ≥ 0.12) in two subjects, but only monocularly. VRT has little effect on absolute HVFDs in SAP.