[Body Integrity Identity Disorder (BIID): interrogation of patients and theories for explanation]. / Body Integrity Identity Disorder (BIID): Befragung von Betroffenen und Erklärungsansätze.

Apotemnophilia, Amputee Identity Disorder or Body Integrity Identity Disorder (BIID) is the intensive feeling that the body will be "more complete" after amputation of a limb. The article disputes the question of matching personality characteristics of these subjects and asks for motives. Based on reports of nine individuals, triggering experiences are referred. In contrast to other children, often these subjects were fascinated by the sight of a handicapped person. In the article is investigated, whether the concerned limb showed more affections. Described is typical pretending behavior. Parallels to body dysmorphic disorder (BDD), fetishism, or delusions are investigated. These were minor, in most cases the wish was fixated on a specific limb, the subjects were aware of the abnormity of their desire and quarreled with the pros and cons. Sexual motives were found in one third. Some of the interviewed persons were in medical or psychological therapy; this did not let the desire disappear. In several BIID sufferers the wish for amputation changed, e. g. from the left to the right leg. This finding is not in accordance with the brain-dysfunction-theory. These people rather have an ideal of a "perfect" body minus one arm or leg. Most admire the beauty of a stump, and see amputees as "heroes" who still master their life in spite of their handicap. BIID is not a homogenous disturbance, one should separate three axes: 1. Strength of neuronal dysfunction, 2. Psychic components (e. g. secondary morbid gain) and 3. Intensity of sexual interests.

[Do patients undergo changes of their personality due to visual field defects? An investigation with FPI-R and NEI-VQ]. / Zeigen Patienten aufgrund ihres Gesichtsfeldausfalls Veränderungen in ihrer Persönlichkeit? Eine Untersuchung mittels FPI-R und NEI-VFQ.

BACKGROUND: Visual restrictions can lead to anxiety and possibly to social retirement. Therefore it makes sense to assess the patients' degree of handicap. The goal of the present study was to investigate if patients show changes in their personality or a reduced quality of life as a result of their visual field defect. METHODS: 15 patients with visual field defects were asked to fill out the revised version of the Freiburger Persönlichkeitsinventar (FPI-R) and the National Eye Institute Visual Function Questionnaire (NEI-VFQ). The FPI-R encompasses the standardised recording of many personality traits whereas the NEI-VFQ addresses the visual quality of life. RESULTS: In the total sample all FPI-R scales were appropriate for the study in the inconspicuous standard range. Slight shifts resulted toward increased willingness to make contacts (scale 4, ST 4.2), reduced physical strain (scale 7, ST 4.3) and lower physical discomfort (scale 8, ST 3.7). The size of the visual field defect does not correlate with the satisfaction with life, with the physical discomfort and with the state of health but with the dependency on others (p = 0.047) and with the exertion of their social roles (p = 0.043). The scale "satisfaction with life" of the FPI-R correlated with the scale "psychic condition" of the NEI-VFQ (p = 0.028) and the physical discomfort showed a significant correlation with the scale "eye strain" (p = 0.006) in the NEI-VFQ. DISCUSSION: Contrary to our presumptions, patients with visual field defects did not show any changes in their personality. It is supposed that they have learned to compensate for their reduced visual functions.

Visual hallucinations during spontaneous and training-induced visual field recovery.

Visual hallucinations after post-geniculate visual system lesions were shown to be associated with spontaneous recovery of visual functions. We investigated the occurrence of hallucinations during spontaneous recovery and additionally tested whether hallucinations were re-instated in a phase of vision restoration therapy (VRT). Nineteen patients with post-geniculate lesions and homonymous visual loss participated in a prospective study, and 121 patients with various lesions were included in a retrospective study using a questionnaire including verbal descriptions as well as drawings of hallucinations experienced by the patients. In both samples, visual-field size was determined before and after 6 months of VRT. Many patients in both groups experienced post-lesion hallucinations (mostly colors, objects, motion) which subsided after spontaneous recovery of visual functions (increase of visual field size, recovery of more complex visual function) was ended. Hallucinations re-emerged during training. However, the majority of patients reported simple, unformed visual hallucinations (uncolored phosphenes, spots, flashes), especially when visual field recovery was most intense. Hallucinations were mainly found in patients with large shifts of the visual field border. They occurred in blind areas, particularly in areas of residual vision where recovery was predominantly observed. Hallucinations may reflect functional recovery in partially lesioned brain areas. While the colored/formed hallucinations during spontaneous recovery may represent non-specific activation of higher visual areas, the simple, unformed training-related hallucinations may indicate recovery in the primary visual cortex during treatment. Hallucinations should not generally be discarded as pathological or unimportant symptoms, but they may be functional indicators of visual system plasticity.

[Comparison of early and late rehabilitation of stroke and cerebral trauma patients with visual field defects]. / Vergleich von Früh- und Spätrehabilitation bei zerebral geschädigten Patienten mit Gesichtsfelddefekten.

BACKGROUND: Most rehabilitation studies on visual field deficits after stroke or trauma are conducted after completion of the spontaneous recovery phase. However, the question arises whether more extensive visual field improvements can be reached when the training starts very soon after the lesion. METHODS: In this study, the results of 26 patients who began visual restoration therapy within the first 12 months after the lesion were compared with an age-related group whose lesions were more than 1 year old. RESULTS: The early-onset group showed an improvement of 8% in computer campimetry and 10-15% in conventional automated perimetry. The late-onset group had 13.5% improvement in campimetry and 20% in perimetry. CONCLUSION: In contrast to our assumptions, there was no significant difference between the groups. Furthermore, the late-onset group showed considerably greater improvement than the early-onset group. It is proposed that pronounced attention deficits soon after brain damage may complicate the training.

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 degrees 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.

Stability of visual field enlargements following computer-based restitution training -- results of a follow-up.

In a previous randomized placebo-controlled clinical trial, we observed significant visual field enlargements induced by computer-based restitution training in patients with cerebral lesions (Kasten et al., Nature med., 4, 1998, 1083-87). Now we asked the question whether this effect is stable after training was discontinued? Here we report data of a follow-up study after a training-free interval (mean 23.5 +/- 2.3 months after end of therapy). 16 patients of the original restitution group and 6 patients of the placebo group were re-examined. On average, in high resolution computer campimetry (stimulus detection: PeriMa, form recognition: PeriForm, color perception: PeriColor) as well as in conventional automatic perimetry (TAP-2000) both groups showed no significant decline in the number of correctly detected stimuli after training was discontinued. However, cluster analysis revealed three different types of patients, who showed either increase (Type-I), decrease (Type-II) or stability (Type-III) in performance. We propose that many patients learn to use the regained visual capacities not only in the setting of a computer training but also in every day life, while other patients do not use the areas of restored vision and show a decrease of visual functions after the end of training. The Type-I group does not need continuous training, while the Type-II group may benefit from phases of refreshment exercises.

Unusual spontaneous and training induced visual field recovery in a patient with a gunshot lesion.

Over a period of more than 3 years, changes in visual and neuropsychological functions were examined in a patient with a visual field defect caused by a cerebral gunshot lesion. Initially, the patient had been completely blind, but after 6 months of spontaneous recovery, he showed a homonymous bilateral lower quadrantanopia and impairment of higher visual functions. Unexpectedly, recovery still continued after the first 6 months. This process was documented in detail by visual field examinations using high resolution perimetry. When visual field size had stabilised almost 16 months after the lesion, further improvement could be achieved by visual restitution training. The duration and extent of spontaneous recovery were unusual. In spontaneous as well as in training induced recovery, progress was mainly seen in partially defective areas (areas of residual vision) along the visual field border. Thus, it is speculated that modulation of perceptual thresholds in transition zones of visual field defects contributes to spontaneous and training induced recovery.

Computer-based training of stimulus detection improves color and simple pattern recognition in the defective field of hemianopic subjects.

In a previously conducted randomized placebo-controlled trial, we were able to demonstrate significant visual field enlargement induced by restitution therapy in patients with cerebral lesions [Kasten, E., Wuest, S., Behrens-Bamann, W., & Sabel, B. A. (1998c). Computer-based training for the treatment of partial blindness. Nature Medicine, 4, 1083-1087.]. Visual field training was performed on a computer monitor for 1 hr per day over a period of 6 months. Since the procedure included only stimulation with white light, in the present study we investigated if this simple detection training had a transfer effect on color or form recognition in the trained area (i.e., in the absence of modality specific training). Answering this question would be crucial for planning optimal restitution therapy: In case there is no transfer of training effects to other visual modalities, a specific treatment of each visual function must be performed in order to achieve maximum benefit. Therefore, we analyzed the data from 32 patients with visual field defects who had participated in the original trial and whose form and color recognition had been investigated. The experimental group (n = 19, restitution training) experienced not only an increase of 12.8% correctly detected stimuli (PeriMa program, p <.05), but also an improvement of 5.6% in pattern recognition (PeriForm) and of 6.1% in color perception (PeriColor), respectively. In contrast, the placebo group (n = 13, fixation training) showed no significant changes from baseline to final outcome in any of the visual modalities (PeriMa: 0.3%; PeriForm: -0.3%; PeriColor: 0.4%). Conventional perimetry yielded an increase of 7.8% detected stimuli in the experimental group, but only of 1.2% in the placebo group (p <.05). For form recognition and color perception, the differences between the results of the experimental and the placebo groups narrowly missed significance. However, correlations of diagnostic results showed that mainly those patients who had achieved visual field enlargement also improved in color and form perception: r =.67 (p <.05) between PeriMa and PeriForm and r =.32 between PeriMa and PeriColor. We conclude that visual restitution training using a simple white light stimulus has at least some influence on improving other visual functions such as color and pattern recognition. This result supports the "bottleneck theory" of visual restitution, i.e., training effects can be explained as a process of perceptual learning and increased processing of information by residual structures surviving lesions of the primary visual pathways.

Restoration of vision by training of residual functions.

A new paradigm emerges: visual field defects after optic nerve or brain injury are partially reversible. Using high-resolution visual field tests, areas of residual vision can be identified which are characterized by impaired vision (relative defect) with some residual capacities. By repetitively stimulating these partially damaged areas with daily computer-based visual restitution training it is now possible to enlarge the visual field. Average border shifts of 5 degrees (range, 0 to 20 degrees) have been found in clinical trials, and training is effective even when started years after the injury. Visual restitution training is useful for the treatment of patients with stroke, head injury, or partial optic nerve damage, as long as the patient presents some residual vision. The improved vision is maintained in most patients after training is discontinued. Brain plasticity is likely to provide the substrate for restoration of vision, opening new opportunities to treat partial blindness, which has been considered irreversible.

Automobile driving performance of brain-injured patients with visual field defects.

The purpose of this study was to examine whether patients with visual field defects resulting from cerebral injury are handicapped in their driving ability, because visual field loss as assessed in standard perimetry is often the basis for withdrawal of a person's driving license. Driving performance was tested on a driving simulator to obtain standardized results and for safety reasons. The visual field was assessed both with standard automated perimetry and computer-based, high-resolution, qualitative perimetry. We investigated nine patients with purely cerebral field defects (mostly homonymous binocular defects) who had no further neuropsychological or ophthalmological deficits. Their performance (driving speed, reaction time, and driving error rate) was compared with that of a control group of ten subjects. We found no differences in any of the tested parameters between the visually impaired subjects and the normal participants. This suggests that individuals with visual field defects, including those who suffer from homonymous hemianopia, may perform as adequately as normal individuals in realistic driving scenarios. The perimetrically assessed visual field may, thus, be of limited value for the prediction of driving safety, and we conclude that patients who have field defects should not summarily be denied a driving license.

Restoration of vision II: residual functions and training-induced visual field enlargement in brain-damaged patients.

PURPOSE: Brain damage is often accompanied by visual field defects which have been considered to be non-treatable. In recent years, however, new diagnostic methods have revealed hitherto unknown residual vision, which was found, for instance, in transition zones near the blind visual field sectors and in spared islands of vision within the blind regions ("blindsight"). Furthermore, animal studies revealed a high degree of plasticity in the visual system suggesting the possibility that recovery of vision may be induced by systematic visual training. METHODS: Here we summarize a series of studies with patients suffering from visual field defects after brain lesion using some most recently developed computer-based programs for the diagnosis and treatment of visual field defects. Specifically, high-resolution perimetry (HRP) was applied to first diagnose residual function in or near the "blind" sector of the visual field. Thereafter, visual restitution training (VRT, see Kasten et al., Nature med. 4, 1998, p. 1083) was used daily for 6 months to provide systematic stimulation of these areas of residual vision. RESULTS: In a number of studies, we have observed not only residual visual functions within or near the field defect, but we were also able to follow the course of spontaneous recovery of visual functions within weeks or months after visual system damage. Furthermore, even long after spontaneous recovery is complete, computer-based visual restitution training (VRT) in or near the areas of residual vision results in a significant enlargement of intact areas, both after optic nerve damage and postchiasmatic lesions. Using VRT, we found a border shift of about 5 degrees of visual angle which cannot be explained by eye movements or eccentric fixation. We observed a transfer of this training effects to other tasks such as form and color detection, as well as to tests of visual exploration which were not specifically trained. Moreover, 72 % of the patients reported subjective improvements of vision. Training-induced visual field enlargement persisted for at least one year, even in the absence of training beyond 6 months of treatment. CONCLUSIONS: The visual system possesses a remarkable plasticity which becomes apparent in visual field enlargement during spontaneous recovery and specific visual training. Animal studies indicate that a minimum number of residual neurons surviving the lesion, in the order of 10%, provides a sufficient substrate for recovery of vision. Though the precise mechanisms of training-induced visual field enlargement need to be further explored, VRT can be introduced for routine clinical treatment of patients with visual field defects.

Computer-based training for the treatment of partial blindness.

Partial blindness after brain injury has been considered non-treatable. To evaluate whether patients with visual-field defects can profit from computer-based visual restitution training (VRT), two independent clinical trials were conducted using patients with optic nerve (n = 19) or post-chiasmatic brain injury (n = 19). In post-chiasma patients, VRT led to a significant improvement (29.4%) over baseline in the ability to detect visual stimuli; in optic nerve patients, the effects were even more pronounced (73.6% improvement). Visual-field enlargements were confirmed by the observation of a visual-field expansion of 4.9 degrees-5.8 degrees of visual angle and improved acuity in optic nerve patients. Ninety five percent of the VRT-treated patients showed improvements, 72.2% confirmed visual improvements subjectively. Patients receiving a placebo training did not show comparable improvements. In conclusion, VRT with a computer program improves vision in patients with visual-field defects and offers a new, cost-effective therapy for partial blindness.

Mantle-cell lymphomas have more widespread disease and a slower response to chemotherapy compared with follicle-center lymphomas: results of a prospective comparative analysis of the German Low-Grade Lymphoma Study Group.

PURPOSE: To compare mantle-cell lymphomas (MCLs) and follicle-center lymphomas (FCLs) for their features of clinical presentation, response to chemotherapy, and prognosis on the basis of a prospective randomized clinical trial. PATIENTS AND METHODS: Patients with MCL and FCL who entered onto the prospective randomized comparison of cyclophosphamide, vincristine, and prednisone (COP) versus prednimustine and mitoxantrone (PmM) followed by a second randomization for interferon (IFN) maintenance versus observation only. RESULTS: One hundred sixty-five of 234 patients had FCL and 45 of 234 patients had MCL. With FCL, both sexes were equally affected (men, 47%); patients with MCL were predominantly men (78%; P < .0004) and had a higher median age (64 v 53 years; P < .0001). Patients with MCL also had more widespread disease, reflected by the proportion of patients with two or greater extranodal manifestations (43% v 21%; P < .005) and nine or greater involved nodal areas (64% v 45%; nonsignificant [NS]). Response to chemotherapy was significantly lower in patients with MCL (complete remission [CR] + partial remission [PR], 69% v 88%; P < .05) and occurred at a slower pace. Patients with MCL also had a shorter event-free interval (median, 8 v 24 months; P < .0001) and overall survival (median, 28 v 77 months; P < .0001). In both subtypes, however, patients with less than two residual lymphoma manifestations in remission experienced a relatively good prognosis with an estimated 5-year survival of greater than 60% for MCL and greater than 75% for FCL. CONCLUSION: MCL and FCL differ substantially in their features of presentation, response to chemotherapy, and long-term prognosis. The extent of residual disease after completion of chemotherapy discriminates patients with different prognosis and may be used for the stratification of postremission strategies.

[Chronic visual hallucinations and illusions following brain lesions. A single case study]. / Chronische visuelle Halluzinationen und Illusionen nach Hirnschädigung. Eine Einzelfallstudie.

Lesions of the visual system do not necessarily lead to deficits in visual function. In some cases, there may even occur Positive Spontaneous Visual Phenomena (PSVP) following cerebral damage. We present data from a male patient with continuous, long-term visual illusions after having experienced cerebral infarction at the age of 56. Basing on conventional Magnetic Resonance Imaging, lesions could be located in areas supported by the lateral and medial occipital artery. Initially, homonymous hemianopsia of the right visual field was found in perimetric examinations, but in the course of six months, visual function recovered completely. Ever since the incident, the patient has been suffering from permanent photopsia, intense colourful visual hallucinations and perseverations located in the former defective area which continued unabated even after the remission of his visual field defects. While many authors have published data on PSVP lasting for several seconds, usually vanishing completely within days or weeks after cerebral lesion, in our patient the symptoms continued over a period of so far nine months. Surprisingly, he was even able to make drawings of his illusions so that we were able to include some of his pictures.

Residual vision in transition zones in patients with cerebral blindness.

Using high resolution perimetry in repeated sessions, we investigated 27 patients with homonymous visual field defects in order to detect islands of vision within the damaged area, and to determine color- and form-recognition abilities within these zones of residual vision. In most patients we found circumscribed areas within the "blind" field in which the stimulus was detected in about 50% of presentations. Only one patient had an island of vision greater than 5 degrees within the defective area. We also found an area of variable performance between the blind and the intact field. Borders of field defects were classified as being: (a) sharp (small transition zone), (b) medium, or (c) fuzzy (scattered deficits). We propose that transition zones are functional representations of partially spared neuronal structures in areas of the brain which are only partially injured.

Visual field enlargement after computer training in brain-damaged patients with homonymous deficits: an open pilot trial.

Brain damage is often accompanied by homonymous hemianopia, but few therapeutic approaches exist for visual field deficits. In this open pilot study we describe a computerized training program which may possibly reduce the size of the 'blind' visual field in patients with homonymous visual field deficits. Various stimuli to test light perception and discrimination of colors and shapes were presented on a monitor which permitted the examination or training of the central section of the visual field up to about 25° vertical and 40° horizontal eccentricity. Eleven patients trained at home for 1 h each day for a total of 80-300 h. Their results were compared with those of three patients who opted not to participate in the training procedure or those with very little therapy. These latter subjects had a slight decrease in the visual field size after about 1 year. In contrast, the treatment group displayed a reliable enlargement of visual field size. This was revealed by a significant improvement in the detection of small light stimuli, an increase in the ability to discriminate colors and a minor, but notable, improvement of shape discrimination in the blind areas of the visual field. Additional training of shape recognition led to further improvement of shape discriminations, even when the patients trained with very different kinds of shapes, e.g. lines or letters. Outcome depended on age of the patients and the size of the lesion, but it was independent of on-set of treatment and cause of the lesion. Only two of the 11 patients with treatment showed no significant improvement. This study suggests that regular home training of the 'blind' visual field with computer-controlled stimuli may lead to improvement in vision. However, because of the following methodological limitations results are only preliminary: (1) the trial did not contain a true placebo group, (2) the patients were not assigned randomly to a control or treatment condition, (3) the lack of defined inclusion criteria considerably increased the variance in neuropsychological performance, (4) because the experimental design was not double blind, experimenter bias cannot be ruled out, and (5) the conditions of the home training could not be standardized. The results warrant a larger randomized, double-blind controlled trial.