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Homonymous hemianopia is the hallmark of postchiasmal brain damage and a sign of serious underlying neurological disease. Patients are often unaware of their visual deficit and may present with vague symptoms. However, there are various neuro-ophthalmological signs, which will alert the clinician to the possibility of a homonymous visual field defect. First, precise history taking and questions about quality of life are of outmost importance in order to proceed to the appropriate diagnostic investigations. Although perimetry is the mainstay for diagnosis in patients with suspected homonymous visual field defects, there are numerous additional tests, which provide useful information regarding the etiology, extent, localization, and functional significance of the underlying brain lesion. Even in the era of modern neuroimaging, the clinician should assess visual acuity, color vision, ocular motility, reading ability, pupil responses, neuropsychological status, and also perform funduscopy and optical coherence tomography, in order to make an individualized assessment and choose the most appropriate therapeutic and rehabilitation interventions.

Retrochiasmal brain damage can lead to various types of homonymous visual field defects (HVFDs), with the occipital lobe being the most common lesion location, followed by the optic radiation, the optic tract, and lateral geniculate nucleus (LGN). The etiology depends on the age of the patient. Older patients tend to have vascular lesions, whereas younger patients may have tumors, traumatic injuries, and arteriovenous malformations. HVFDs can be classified according to their size, shape, macular sparing, and congruency, giving rise to a variety of perimetric findings. Complete homonymous hemianopias are nonlocalizing and may be caused by lesions in any part of the retrochiasmal visual pathway, including the optic tract, LGN, optic radiation, and occipital lobe. However, a highly congruous complete homonymous hemianopia is usually due to an occipital lobe lesion. Homonymous quadrantanopias usually result from lesions of the occipital lobe and the optic radiation. Less frequent types of HVFDs are paracentral circumscribed homonymous scotomas due to small lesions at the tip of the occipital lobe, homonymous sectoranopias in lesions of the LGN, and peripheral HVFDs due to lesions in the intermediate striate cortex. A unique HVFD is the “temporal crescent,” after damage to the most anterior portion of the occipital lobe. Bilateral homonymous visual field defects due to bilateral postchiasmatic lesions may present as checkerboard visual fields or bilateral altitudinal defects. Such deficits may pose a diagnostic dilemma, as they have to be differentiated from anterior ischemic optic neuropathy, ischemic retinal lesions, choroiditis, choroidal colobomas, glaucoma, optic nerve hypoplasia, tilted discs, or drusen.

Nystagmus is an involuntary rhythmic oscillation of the eyes, which leads to reduced visual acuity due to the excessive motion of images on the retina. Nystagmus can be grouped into infantile nystagmus (IN), which usually appears in the first 3-6 months of life, and acquired nystagmus (AN), which appears later. IN can be idiopathic or associated to albinism, retinal disease, low vision, or visual deprivation in early life, for example due to congenital cataracts, optic nerve hypoplasia, and retinal dystrophies, or it can be part of neurological syndromes and neurologic diseases. It is important to differentiate between infantile and acquired nystagmus. This can be achieved by considering not only the time of onset of the nystagmus, but also the waveform characteristics of the nystagmus. Neurological disease should be suspected when the nystagmus is asymmetrical or unilateral. Electrophysiology, laboratory tests, neurological, and imaging work-up may be necessary, in order to exclude any underlying ocular or systemic pathology in a child with nystagmus. Furthermore, the recent introduction of hand-held spectral domain optical coherence tomography (HH SD-OCT) provides detailed assessment of foveal structure in several pediatric eye conditions associated with nystagmus and it can been used to determine the underlying cause of infantile nystagmus. Additionally, the development of novel methods to record eye movements can help to obtain more detailed information and assist the diagnosis. Recent advances in the field of genetics have identified the FRMD7 gene as the major cause of hereditary X-linked nystagmus, which will possibly guide research towards gene therapy in the future. Treatment options for nystagmus involve pharmacological and surgical interventions. Clinically proven pharmacological treatments for nystagmus, such as gabapentin and memantine, are now beginning to emerge. In cases of obvious head posture, eye muscle surgery can be performed to shift the null zone of the nystagmus into the primary position, and also to alleviate neck problems that can arise due to an abnormal head posture.

We thank Lee et al. for the insightful comments on our Teaching Video NeuroImage on pulsatile proptosis and wall-eyed bilateral internuclear ophthalmoplegia (WEBINO).1 The causative lesions for bilateral WEBINO can be located in the tegmentum of pons, pons–midbrain, or midbrain, with some injuries almost bisecting the brainstem.2,3 As the MRI presented by Lee et al. discloses multiple hyperintense foci at the pontomesencephalic level, we reviewed the diffusion-weighted MRI, which shows a focal area of restricted diffusion at the midline of the pontine tegmentum beneath the floor of the fourth ventricle, which involves the medial longitudinal fasciculus (MLF) bilaterally (figure). The patient had presented 2 days after onset of diplopia and this lesion was consistent with an acute ischemic stroke. Although we indicated in the text and figure legend of the T2-weighted MRI that the infarction was located at the midline of the midbrain tegmentum,1 the arrow on the MRI had been placed more anteriorly by mistake. Hence, we agree with Lee et al. that, at the pontine level, the MLF is located just ventral to the fourth ventricle, and lesions of this area can lead to bilateral WEBINO.4

A 37-year-old woman presented to the department of otolaryngology with a 15-day history of frontal headache. The patient had been treated for sinusitis with a 10-day course of amoxicillin and clavulanic acid without any improvement. Five days before presentation, a well-defined, fluctuating forehead

Acute retinal necrosis (ARN) is a rare, potentially blinding condition typically affecting immunocompetent individuals. It is defined by the clinical triad of vitreous inflammation, occlusive vasculopathy, and progressive retinal necrosis, usually located in peripheral retina with circumferential extension. Varicella zoster virus (VZV), herpes simplex virus (HSV), Epstein-Barr virus (EBV) and occasionally cytomegalovirus (CMV) are the common causative agents of ARN. Reports of human herpesvirus 6 (HHV6) infection of the central nervous system (CNS) associated with ocular inflammatory disease are extremely rare. We here report the case of a 22-year-old immunocompetent male who presented with acute bilateral ARN and fourth nerve palsy, following HHV6 infection of the CNS and EBV infectious mononucleosis.

A 56-year-old postmenopausal obese woman presented with a 6-month history of right-sided rhinorrhoea. There was no history of head trauma or surgery. On clinical examination drops of clear fluid were falling from her right nostril when bending forward (online supplementary video 1). Nasal endoscopy identified no pathology. The fluid was positive for beta-2 transferrin. There was no evidence of papilloedema and the opening pressure was normal.1 Imaging demonstrated a right cribriform plate defect (figure 1). Endoscopic …

Macular Hole from a Laser Pointer A 9-year-old boy presented with decreased vision in his left eye after gazing into the beam of a laser pointer. Funduscopy and optical coherence tomography revealed a full-thickness macular hole.

Purpose The goal of this work was to (i) determine patterns of progression in glaucomatous visual field loss, (ii) compare the detection rate of progression between locally condensed stimulus arrangements and conventional 6° × 6° grid, and (iii) assess the individual frequency distribution of test locations exhibiting a local event (i.e., an abrupt local deterioration of differential luminance sensitivity (DLS) by more than -10dB between any two examinations). Methods The visual function of 41 glaucomatous eyes of 41 patients (16 females, 25 males, 37 to 75 years old) was examined with automated static perimetry (Tuebingen Computer Campimeter or Octopus 101-Perimeter). Stimuli were added to locally enhance the spatial resolution in suspicious regions of the visual field. The minimum follow-up was four subsequent sessions with a minimum of 2-month (median 6-month) intervals between each session. Progression was identified using a modified pointwise linear regression (PLR) method and a modified Katz criterion. The presence of events was assessed in all progressive visual fields. Results Eleven eyes (27%) showed progression over the study period (median 2.5 years, range 1.3–8.6 years). Six (55%) of these had combined progression in depth and size and five eyes (45%) progressed in depth only. Progression in size conformed always to the nerve fiber course. Seven out of 11 (64%) of the progressive scotomata detected by spatially condensed grids would have been missed by the conventional 6° × 6° grid. At least one event occurred in 64% of all progressive eyes. Five of 11 (46%) progressive eyes showed a cluster of events. Conclusions The most common pattern of progression in glaucomatous visual fields is combined progression in depth and size of an existing scotoma. Applying individually condensed test grids remarkably enhances the detection rate of glaucomatous visual field deterioration (at the expense of an increased examination time) compared to conventional stimulus arrangements.

Die rhinoorbitozerebrale Mukormykose ist eine seltene invasive Pilzinfektion, die unbehandelt todlich verlauft. Vorgestellt wird der Fall einer immunsupprimierten Patientin, die bei Z. n. allogener Stammzelltransplantation bei chronisch lymphatischer Leukamie eine schwere rhinoorbitozerebrale Mukormykose entwickelte. Aufgrund des fulminanten und moglicherweise todlichen Verlaufs ist bei entsprechenden klinischen Zeichen auch ohne eines histopathologischen Nachweises eine radikale Entfernung der gesamten nekrotischen Areale in Kombination mit antifungaler Therapie unerlasslich.

Purpose: To characterize the time course of normal foveal development in vivo in term infants and young children using handheld spectral-domain optical coherence tomography (HH-SDOCT). Methods: We obtained 534 HH-SDOCT scans from 261 infants, children, and young adults with a mean age of 4.9 years (range, 0-27 years). Each retinal layer was manually segmented in ImageJ and correlated with gestational age (GA) and visual acuity (VA). The developmental trajectories of each retinal layer at the fovea, parafovea, and perifovea were calculated using fractional polynomial modeling. Results: The central macular thickness (CMT) increases logarithmically between birth and 48.6 months GA. The foveal ganglion cell (GCL), inner plexiform, inner nuclear (INL), and outer plexiform layers decrease in thickness exponentially until 18 months GA. Interestingly, the parafoveal and perifoveal GCL and INL thicknesses initially decrease until 17 months GA and then increase in thickness until 65.5 GA. The foveal outer nuclear layer, inner segment, and outer segment of the photoreceptors increase in thickness logarithmically until 32.4, 26.9, and 45.3 months GA, respectively. The parafoveal and perifoveal outer retinal layers increase in thickness more gradually until 146 months GA. The thickness of the outer retinal layers and CMT were strongly correlated with VA, with r = 0.54 (P Conclusions: We have modeled for the first time the complex, nonlinear developmental trajectories for each retinal layer and demonstrate that development continues until adolescence. Our description of normal development will be helpful in diagnosing, monitoring, and understanding pediatric retinal disease.

Background: In the present study, we evaluated the association of platelet reactivity with vascular function in patients after percutaneous coronary intervention receiving clopidogrel treatment. Methods: We enrolled 150 patients with stable CAD receiving clopidogrel regimen (75 mg/d), 1 month after percutaneous coronary intervention. Carotid-femoral pulse wave velocity (PWV) was measured as an index of aortic stiffness and augmentation index (AIx) as an index of arterial wave reflections. High on treatment platelet reactivity (HPR) was evaluated using Verify Now Assay. Verify Now reports its results in P2Y12 reaction units (PRU), and the diagnostic cutoff value is 230 PRU. Patients were evaluated prospectively up to 24 months. The primary end point was a composite of death from cardiovascular causes, nonfatal major cardiovascular events and hospitalization for cardiovascular causes. Results: There was no difference in the basic clinical and demographic characteristics between subjects with HPR and non-HPR. Subjects with high on treatment platelet reactivity and PRU > 230 had significantly increased PWV (8.81 +/- 2.25 m/s vs. 7.69 +/- 1.95 m/s, p = 0.001) and AIx (25.27 +/- 8.67% vs. 20.87 +/- 10.57%, p = 0.04) compared to subjects with PRU

Bei einer akuten Makulablutung bei einem sonst gesunden jungen Patienten sollte die seltene Valsalva-Retinopathie in Betracht gezogen werden. Meist stellt sich der Patient wegen einer plotzlichen, schmerzlosen Sehverschlechterung und eines Zentralskotoms vor. Essenziell ist eine sorgfaltige Anamneseerhebung, da der Betroffene zuvor typischerweise eine anstrengende korperliche Tatigkeit ausgeubt hat.

Injury to the primary visual cortex (V1) typically leads to loss of conscious vision in the corresponding, homonymous region of the contralateral visual hemifield (scotoma). Several studies suggest that V1 is highly plastic after injury to the visual pathways, whereas others have called this conclusion into question. We used functional magnetic resonance imaging (fMRI) to measure area V1 population receptive field (pRF) properties in five patients with partial or complete quadrantic visual field loss as a result of partial V1+ or optic radiation lesions. Comparisons were made with healthy controls deprived of visual stimulation in one quadrant [ldquo;artificial scotomardquo; (AS)]. We observed no large-scale changes in spared-V1 topography as the V1/V2 border remained stable, and pRF eccentricity versus cortical-distance plots were similar to those of controls. Interestingly, three observations suggest limited reorganization: (i) the distribution of pRF centers in spared-V1 was shifted slightly toward the scotoma border in 2 of 5 patients compared with AS controls; (ii) pRF size in spared-V1 was slightly increased in patients near the scotoma border; and (iii) pRF size in the contralesional hemisphere was slightly increased compared with AS controls. Importantly, pRF measurements yield information about the functional properties of spared-V1 cortex not provided by standard perimetry mapping. In three patients, spared-V1 pRF maps overlapped significantly with dense regions of the perimetric scotoma, suggesting that pRF analysis may help identify visual field locations amenable to rehabilitation. Conversely, in the remaining two patients, spared-V1 pRF maps failed to cover sighted locations in the perimetric map, indicating the existence of V1-bypassing pathways able to mediate useful vision.

In this study we investigated macular morphology, including individual retinal layers, in patients with optic nerve head drusen (ONHD) and optic disc edema (ODE) compared with healthy participants, using high-resolution spectral domain optical coherence tomography (OCT).Prospective, cross-sectional, observational study.A total of 67 patients with ONHD, 36 patients with ODE, and 57 healthy participants.High-resolution spectral domain OCT (Copernicus [OPTOPOL Technology S.A., Zawiercie, Poland] 3-μm resolution, 7 × 7 × 2-mm volumetric scans) was used to image macula morphology. Average retinal nerve fiber layer (RNFL) thickness was measured using a semiautomated method with manual correction of the internal limiting membrane, RNFL, and retinal pigment epithelium (RPE). Retinal and RNFL thicknesses were measured and analyzed in 3 circular zones (Early Treatment Diabetic Retinopathy Study protocol). Individual retinal layers at the macula were quantified by analyzing tomograms using ImageJ (http://rsbweb.nih.gov/ij/; Accessed June 1, 2013).Average retinal and individual retinal layer thickness in patients with ODE or ONHD, and healthy controls.Patients with ONHD had thicker retinae in the inner annulus compared with patients with ODE and controls (significant in the temporal segment compared with those with ODE [P = 0.013] and in the superior segment compared with controls [P = 0.05]). Patients with ONHD had a significantly thinner inner plexiform layer (IPL) (P = 0.02), nerve fiber layer (P = 0.05), and RPE (P = 0.0001), and thicker ganglion cell layer (P = 0.003) and outer plexiform layer (OPL) (P0.001) compared with controls. Patients with ODE demonstrated the thickest retina and RNFL in the outer annulus (significant in the inferior segment compared with controls, P = 0.02 for both) with significant thickening in the IPL (P = 0.004), OPL (P0.003), and outer segment layer (P ≤ 0.02), and severe ganglion cell loss (P = 0.004) and RPE (P = 0.0001) thinning compared with healthy volunteers.Our study shows that optic nerve diseases are associated with selective changes in different retinal layers in patients with ODE and ONHD. These findings may be of diagnostic value and could be taken into consideration in assessing patients and studying the pathogenesis of these conditions.

PURPOSE To present the unusual case of multiple retinal arterial macroaneurysms (RAMs) associated with an extensive submacular hemorrhage. METHODS A 64-year-old hypertensive woman is presented including the clinical findings, fluorescein angiography, and optical coherence tomography. RESULTS The patient presented with 20/200 vision due to a dense submacular hemorrhage in the right eye. Fluorescein angiography revealed 4 hyperfluorescent lesions along the inferotemporal arteriole corresponding to RAMs. Optical coherence tomography confirmed RAM rupture with no choroidal abnormality. The patient refused surgical intervention. At 3-month follow-up, vision had improved to 20/70 with partial resolution of the hemorrhage. However, vitreoretinal traction with neurosensory retinal detachment involving the macula had developed. CONCLUSIONS Acquired RAMs are typically found as unilateral solitary lesions in older hypertensive women. The presence of 4 RAMs along the same arteriole has not been reported previously. Hypertensive patients should be examined regularly in order to detect potential RAMs early enough to permit treatment and prevent vision loss.

Aim of the present study was to identify efficient compensatory gaze patterns applied by patients with homonymous visual field defects (HVFDs) under virtual reality (VR) conditions in a dynamic collision avoidance task. Thirty patients with HVFDs due to vascular brain lesions and 30 normal subjects performed a collision avoidance task with moving objects at an intersection under two difficulty levels. Based on their performance (i.e. the number of collisions), patients were assigned to either an “adequate” (HVFDA) or “inadequate” (HVFDI) subgroup by the median split method. Eye and head tracking data were available for 14 patients and 19 normal subjects. Saccades, fixations, mean number of gaze shifts, scanpath length and the mean gaze eccentricity, were compared between HVFDA, HVFDI patients and normal subjects. For both difficulty levels, the gaze patterns of HVFDA patients (N = 5) compared to HVFDI patients (N = 9) were characterized by longer saccadic amplitudes towards both the affected and the intact side, larger mean gaze eccentricity, more gaze shifts, longer scanpaths and more fixations on vehicles but fewer fixations on the intersection. Both patient groups displayed more fixations in the affected compared to the intact hemifield. Fixation number, fixation duration, scanpath length, and number of gaze shifts were similar between HVFDA patients and normal subjects. Patients with HVFDs who adapt successfully to their visual deficit, display distinct gaze patterns characterized by increased exploratory eye and head movements, particularly towards moving objects of interest on their blind side. In the context of a dynamic environment, efficient compensation in patients with HVFDs is possible by means of gaze scanning. This strategy allows continuous update of the moving objects’ spatial location and selection of the task-relevant ones, which will be represented in visual working memory.

The aim of the present study was to examine the effect of homonymous visual field defects (HVFDs) on collision avoidance of dynamic obstacles at an intersection under virtual reality (VR) conditions. Overall performance was quantitatively assessed as the number of collisions at a virtual intersection at two difficulty levels. HVFDs were assessed by binocular semi-automated kinetic perimetry within the 90° visual field, stimulus III4e and the area of sparing within the affected hemifield (A-SPAR in deg 2 ) was calculated. The effect of A-SPAR, age, gender, side of brain lesion, time since brain lesion and presence of macular sparing on the number of collisions, as well as performance over time were investigated. Thirty patients (10 female, 20 male, age range: 19–71 years) with HVFDs due to unilateral vascular brain lesions and 30 group-age-matched subjects with normal visual fields were examined. The mean number of collisions was higher for patients and in the more difficult level they experienced more collisions with vehicles approaching from the blind side than the seeing side. Lower A-SPAR and increasing age were associated with decreasing performance. However, in agreement with previous studies, wide variability in performance among patients with identical visual field defects was observed and performance of some patients was similar to that of normal subjects. Both patients and healthy subjects displayed equal improvement of performance over time in the more difficult level. In conclusion, our results suggest that visual-field related parameters per se are inadequate in predicting successful collision avoidance. Individualized approaches which also consider compensatory strategies by means of eye and head movements should be introduced.

Background: Lesions affecting the pretectum or the brachium of the superior colliculus (brachium) and sparing the optic tract cause a contralateral relative afferent pupil defect (RAPD) but no visual field loss. It has been assumed that the pupillomotor pathways within the brachium are a continuation of the pupillomotor pathways traveling in the optic tract. To investigate this assumption, we looked for hemihypokinesia by means of pupil perimetry. Methods: Pupillary hemifield stimulation was performed in a 65-year-old woman with normal visual fields and an isolated left RAPD due to a cerebral hemorrhage affecting the right dorsal midbrain. The pupil responses from light stimulation of the nasal inferior, nasal superior, and temporal inferior and temporal superior quadrants of both eyes were recorded using computerized binocular infrared pupillography. Each stimulus was presented 5 times and the mean amplitude of the pupil response was calculated for each stimulus location. Results: Pupil perimetry demonstrated a marked hemihypokinesia (reduced light reaction) in the hemifield contralateral to the site of the lesion. Conclusions: Our experiment suggests that the brachium is indeed a continuation of the afferent pupillary fibers traveling in the optic tract. (J Neuro-Ophthalmol 2009;29:33–36)

Purpose The aim of this study was to assess whether visual field-related parameters per se are able to predict driving performance of patients with homonymous visual field defects (HVFDs) and healthy controls in a standardized virtual reality (VR) environment. Methods Thirty-two patients with HVFDs due to cerebro-vascular lesions and 32 healthy control subjects underwent testing on a driving simulator under two levels of traffic density. Driving performance was defined as the frequency of accidents under VR-conditions. From the binocular visual field, as measured with kinetic perimetry, we assessed the area of sparing within the affected hemifield (A-SPAR) and the perimetric reaction time (RT). Results Frequency of traffic accidents was best explained by traffic density, age of individuals, A-SPAR and RT. This model explained 78% of the total variability (Radj2 = 0.78). All factors were significant. The effect of traffic density explained 63.4 % of the total variance. Age, A-SPAR and RT were minor effects explaining only 2.7%, 2.0% and 0.9% respectively. Conclusion The extent of the visual field loss is weakly related to driving performance. This finding suggests that – at least for this group of patients – the visual field-related parameters should not be taken as the sole indicator of fitness to drive. Some patients with HVFDs demonstrated sufficient compensatory driving behavior during the simulated test ride, presumably due to eye and head movements. Driving performance declined slightly with age; these changes were exacerbated in the presence of cerebro-vascular disease.

Objective: The anatomy of the human pupillary light reflex (PLR) pathway is a matter of debate. The aim of this study was twofold: namely, to investigate the association of a relative afferent pupillary defect (RAPD) in acquired suprageniculate lesions with the location and extent of the cerebral lesions. Further, we suggest a new strategy of lesion analysis by combining established techniques with the stereotaxic probabilistic cytoarchitectonic atlas developed by the Julich group. Methods: Twenty-three patients with homonymous visual field defects participated in this study. The RAPD was quantified clinically by two independent examiners with graded neutral density filters (swinging flashlight test). Using MRI in each individual, cerebral regions commonly affected in patients with a RAPD but spared in patients without a RAPD were determined and subsequently assessed by using cytoarchitectonic probabilistic maps. Results: A RAPD was present in 10/23 patients. Comparison of patients showing a RAPD vs those not showing a RAPD revealed that a region including the course of the optic radiation at its early beginning in the temporal white matter is commonly associated with a RAPD. Conclusions: It was demonstrated that the pupillary light reflex (PLR) depends on the input of suprageniculate neurons, thus supporting the involvement of a cortical pathway also. The site of integration of cortical signals in relation to the PLR into the pupillomotor pathway may be located suprageniculately in the vicinity of the lateral geniculate nucleus. Moreover, the suggested combination of established lesion analysis techniques with the probabilistic cytoarchitectonic atlas turned out to be a very helpful amelioration of stroke data analyses.

Retinal development normally involves migration of the inner retinal layers away from the fovea, migration of the cone photoreceptors into the fovea, and elongation of the photoreceptors over time. This process is arrested prematurely in albinism. However, because retinal development continues at least until the age of 4 years, when development arrests in albinism is uncertain. In this study we outlined the time course of retinal development in children with albinism.We studied 44 children with a diagnosis of albinism and 223 control participants. All participants were aged between 0 and 6 years. We obtained 219 mixed cross-sectional and longitudinal optical coherence tomography examinations in the albinism group and compared them with 558 control examinations. Retinal layer segmentation was performed with ImageJ software. Generalised linear mixed regression modelling was used to analyse group differences in retinal development.In the albinism group, inner retinal layer migration from the fovea was delayed and arrested prematurely, resulting in a significantly thicker central macular thickness than in the control group (p0·0001). Whereas the central macular thickness increased with age in the control group, in the albinism group it initially decreased with age as a result of continuing regression of the inner retinal layers (p=0·041). The perifoveal retinal thickness was significantly decreased in albinism from a reduction of both inner (p0·0001) and outer (p0·0001) retinal layer thicknesses. There was evidence that the photoreceptor layers across the fovea were elongating in albinism, albeit at a reduced rate, compared with the control group. This difference was most apparent for the foveal photoreceptor inner segment (p=0·001).Our findings show that perturbations exist in several aspects of retinal development including the migration and differentiation of the neuronal cells within the retina. We showed continuing regression of the inner retinal layers and elongation of the photoreceptor layers suggesting residual plasticity of the developing albino retina. This finding is important because treatment at the earliest stages of the condition might normalise retinal development and optimise vision.UK Medical Research Council (grant number MR/J004189/1), Ulverscroft Foundation, National Eye Research Centre, Nystagmus Network UK.

Homonymous visual field defects (HVFDs) are among the most common disorders that occur in the elderly after vascular brain damage and can have a major impact on quality of life (QOL). Aims of this study were to describe the vision-targeted, health-related QOL in patients with HVFDs after cerebrovascular lesion, and to determine the relationship between patients' self-reported difficulties and the characteristics of HVFDs in the binocular visual field.The German version of the 25-item National Eye Institute Visual Functioning Questionnaire (NEI-VFQ-25) was used. NEI-VFQ-25 scores for patients were compared to reference values of healthy German subjects from Franke (Z Med Psychol 7:178-184, 1999). Extent and location of absolute HVFDs were assessed by binocular semi-automated kinetic perimetry (SKP) within the 90 degrees visual field. Correlations of the NEI-VFQ-25 scores of patients with the area of sparing within the affected hemifield (A-SPAR) were estimated by Spearman's r (s).The mean NEI-VFQ-25 composite score for 33 patients (time span after brain injury at least 6 months) was 77.1, which was significantly lower (p0.0001) than the reference value for 360 healthy subjects (composite score = 90.6), and this was also the case for general vision, near activities, vision specific mental health, driving, colour, and peripheral vision. The score for general health was also significantly lower in patients than in reference subjects (p0.0001). A weak correlation of the composite score with A-SPAR (r (s) = 0.38) was observed.Our findings indicate that detectable decrements in vision-targeted, health-related QOL are observed in patients with homonymous visual field loss. A relationship of the perceived visual functioning with objective parameters is by definition difficult; however, understanding what components of visual function affect certain visual tasks, would help in developing more efficient, clinical assessment strategies. The results reveal a tendency for increasing QOL with advancing size of the area of sparing within the affected hemifield (A-SPAR). The lack of a strong correlation between NEI-VFQ-25 subscales and A-SPAR suggests that an assessment of the visual field may not accurately reflect patients' perceived difficulty in visual tasks. Additional consideration of visual exploration via eye and head movements may improve the correlation between visual function and its perception.

Gastrointestinal symptoms such as nausea, abdominal pain, and bloating are frequent complaints in patients with Parkinson disease (PD) and in patients with multiple system atrophy (MSA), and may be associated with delayed gastric emptying (GE). Although several GE studies in patients with PD have been performed, scant data exist in patients with MSA.We assessed GE half-times (T50) in 12 patients with MSA and compared them with those of 12 patients with PD and 12 age-matched healthy controls.GE was estimated scintigraphically using the left anterior oblique method after ingestion of a (99m)Tc colloid-labeled balanced semi-solid meal (yogurt). GE data were obtained every 15 minutes until there was complete emptying of the stomach. Blood pressure, heart rate, plasma glucose and glucosylated hemoglobin were regularly determined.Reproducibility of the GE technique was excellent (Bland-Altman analysis, limits of agreement: -2.3 to 2.8). T50 was longer in MSA (82+/-3.4 min) and in PD (90.6+/-3.9 min) patients compared with controls (46.2+/-0.7) (two-way ANOVA, p0.0001). T50 did not differ between patients with MSA and those with PD. No correlation existed between T50 and age, duration of the disease, magnitude of postprandial hypotension, levels of plasma glucose and glucosylated hemoglobin (Kendall's tau, p0.05).Our results suggest that patients with MSA have GE rates similar to those of patients with PD, but slower than healthy age matched individuals. It remains to be investigated whether gastrointestinal dysfunction in MSA is related to both brain and peripheral pathology, as is presumed for PD.

To assess the spatial distribution of glaucomatous visual field defects (VFDs) obtained with regionally condensed stimulus arrangements.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 P0.05 (total deviation plots) were assessed by superimposing the visual field records of all participants.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°.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.

We investigated the task-specific role of eye and head movements as a compensatory strategy in patients with homonymous visual field deficits (HVFDs) and in age-matched normal controls. All participants were tested in two tasks, i.e. a dot counting (DC) task requiring mostly simple visual scanning and a cognitively more demanding comparative visual search (CVS) task. The CVS task involved recognition and memory of geometrical objects and their configuration in two test fields. Based on task performance, patients were assigned to one of two groups, "adequate" (HVFD(A)) and "inadequate" (HVFD(I)); the group definitions based on either task turned out to be identical. With respect to the gaze related parameters in the DC task we obtained results in agreement with previous studies: the gaze pattern of HVFD(A) patients and normal controls did not differ significantly, while HVFD(I) patients showed increased gaze movement activity. In contrast, for the more complex CVS task we identified a deviating pattern of compensatory strategy use. Adequately performing subjects, who had used the same gaze strategies as normals in the DC task, now changed to increased gaze movement activity that allowed coping with the increasing task demands. Inadequately performing patients switched to a novel pattern of compensatory behavior in the CVS task. Different compensatory strategies are discussed with respect to the task-specific demands (in particular working memory involvement), the specific behavioral deficits of the patients, and the corresponding brain lesions.