Midregional proatrial naturetic peptide (MRproANP) and copeptin (COPAVP) as predictors of all-cause mortality in recently diagnosed mild to moderate COPD-results from COSYCONET.

BACKGROUND: MRproANP and COPAVP are prognostic markers for mortality in chronic obstructive pulmonary disease (COPD). Furthermore, these biomarkers predict mortality due to cardiovascular diseases, which are important prognostically determining comorbidities in patients with COPD. However, less is known about these biomarkers in recently diagnosed mild to moderate COPD. Therefore, we analyzed these biomarkers as potential predictors of mortality in recently diagnosed mild to moderate COPD. METHODS: The blood biomarkers considered were copeptin (COPAVP), midregional adrenomedullin (MRproADM), midregional proatrial naturetic peptide (MRproANP), and fibrinogen. Analyses were performed in patients with stable "recently diagnosed mild to moderate COPD" defined by GOLD grades 0-2 and diagnosis of COPD ≤ 5 years prior to inclusion into the COSYCONET cohort (COPD and Systemic Consequences-Comorbidities Network), using Cox regression analysis with stepwise adjustment for multiple COPD characteristics, comorbidities, troponin and NT-proBNP. RESULTS: 655 patients with recently diagnosed mild to moderate COPD were included. In the initial regression model, 43 of 655 patients died during the 6-year follow-up, in the final model 27 of 487. Regression analyses with adjustment for confounders identified COPAVP and MRproANP as statistically robust biomarkers (p < 0.05 each) of all-cause mortality, while MRproADM and fibrinogen were not. The fourth quartile of MRproANP (97 pmol/L) was associated with a hazard ratio of 4.5 (95%CI: 1.6; 12.8), and the fourth quartile of COPAVP (9.2 pmol/L) with 3.0 (1.1; 8.0). The results for MRproANP were confirmed in the total cohort of grade 0-4 (n = 1470 finally). CONCLUSION: In patients with recently diagnosed mild to moderate COPD, elevated values of COPVP and in particular MRproANP were robust, independent biomarkers for all-cause mortality risk after adjustment for multiple other factors. This suggests that these markers might be considered in the risk assessment of early COPD.

Interplay of tau and functional network connectivity in progressive supranuclear palsy: a [18F]PI-2620 PET/MRI study.

PURPOSE: Progressive supranuclear palsy (PSP) is primary 4-repeat tauopathy. Evidence spanning from imaging studies indicate aberrant connectivity in PSPs. Our goal was to assess functional connectivity network alterations in PSP patients and the potential link between regional tau-burden and network-level functional connectivity using the next-generation tau PET tracer [18F]PI-2620 and resting-state functional MRI (fMRI). MATERIAL AND METHODS: Twenty-four probable PSP patients (70.9 ± 6.9 years, 13 female), including 14 Richardson syndrome (RS) and 10 non-RS phenotypes, underwent [18F]PI-2620 PET/MRI imaging. Distribution volume ratios (DVRs) were estimated using non-invasive pharmacokinetic modeling. Resting-state fMRI was also acquired in these patients as well as in thirteen older non-AD MCI reference group (64 ± 9 years, 4 female). The functional network was constructed using 141 by 141 region-to-region functional connectivity metrics (RRC) and network-based statistic was carried out (connection threshold p < 0.001, cluster threshold pFDR < 0.05). RESULTS: In total, 9870 functional connections were analyzed. PSPs compared to aged non-AD MCI reference group expressed aberrant connectivity evidenced by the significant NBS network consisting of 89 ROIs and 118 connections among them (NBS mass 4226, pFDR < 0.05). Tau load in the right globus pallidus externus (GPe) and left dentate nucleus (DN) showed significant effects on functional network connectivity. The network linked with increased tau load in the right GPe was associated with hyperconnectivity of low-range intra-opercular connections (NBS mass 356, pFDR < 0.05), while the network linked with increased tau load in the left cerebellar DN was associated with cerebellar hyperconnectivity and cortico-cerebellar hypoconnectivity (NBS mass 517, pFDR < 0.05). CONCLUSIONS: PSP patients show altered functional connectivity. Network incorporating deep gray matter structures demonstrate hypoconnectivity, cerebellum hyperconnectivity, while cortico-cortical connections show variable changes. Tau load in the right GPe and left DN is associated with functional networks which strengthen low-scale intra-opercular and intra-cerebellar connections and weaken opercular-cerebellar connections. These findings support the concept of tau load-dependent functional network changes in PSP, by that providing evidence for downstream effects of neuropathology on brain functionality in this primary tauopathy.

Testing for food-specific antibodies in saliva and blood of food allergic and healthy dogs.

An elimination diet (ED) followed by re-challenge has been the reference standard to diagnose adverse food reactions (AFR) in dogs, but can be challenging to conduct. This study investigated the accuracy of a saliva-based test for food-specific IgA and IgM and an ELISA serum test for food-specific IgE. Three groups of dogs were tested. Group 1 (n=11) included dogs with previously diagnosed and controlled AFR; group 2 (n=15) comprised dogs with allergic dermatitis at the beginning of their ED; and group 3 (n=16) was composed of clinically healthy research dogs. Saliva samples were collected from all groups and blood samples from group 1 and group 3. The results of clinical re-challenges with individual food components were compared with the test results. Specificity, sensitivity, positive and negative predictive values and likelihood ratios were determined. Forty-one dogs completed the study; one dog was lost to follow up. There was a total of 163 re-challenges. Sensitivity, positive predictive value and likelihood ratio, specificity, negative predictive value and likelihood ratios were unsatisfactory for both tests in most instances, except for IgM testing in group 2, which had moderate specificity. There was no clear difference in the number of positive reactions between the allergic dogs and healthy dogs from a research population. Based on these results, the saliva test for food specific IgA and IgM and the ELISA serum test for food specific IgE were not reliable to diagnose adverse food reactions in dogs. Until more data are available, elimination diets remain the reference standard in the diagnosis of this disease.

The associative brain at work: Evidence from paired associative stimulation studies in humans.

The original protocol of Paired Associative Stimulation (PAS) in humans implies repetitive cortical and peripheral nerve stimuli, delivered at specific inter-stimulus intervals, able to elicit non-invasively long-term potentiation (LTP)- and long-term depression (LTD)-like plasticity in the human motor cortex. PAS has been designed to drive cortical LTP/LTD according to the Hebbian rule of associative plasticity. Over the last two decades, a growing number of researchers have increasingly used the PAS technique to assess cortical associative plasticity in healthy humans and in patients with movement disorders and other neuropsychiatric diseases. The present review covers the physiology, pharmacology, pathology and motor effects of PAS. Further sections of the review focus on new protocols of "modified PAS" and possible future application of PAS in neuromorphic circuits designed for brain-computer interface.

Preliminary evaluation of cytosine-phosphate-guanine oligodeoxynucleotides bound to gelatine nanoparticles as immunotherapy for canine atopic dermatitis.

Cytosine-phosphate-guanine oligodeoxynucleotides (CpG ODN) are a promising new immunotherapeutic treatment option for canine atopic dermatitis (AD). The aim of this uncontrolled pilot study was to evaluate clinical and immunological effects of gelatine nanoparticle (GNP)-bound CpG ODN (CpG GNP) on atopic dogs. Eighteen dogs with AD were treated for 8 weeks (group 1, n=8) or 18 weeks (group 2, n=10). Before inclusion and after 2 weeks, 4 weeks, 6 weeks (group 1+2), 8 weeks, 12 weeks and 16 weeks (group 2) 75 µg CpG ODN/dog (bound to 1.5 mg GNP) were injected subcutaneously. Pruritus was evaluated daily by the owner. Lesions were evaluated and serum concentrations and mRNA expressions of interferon-γ, tumour necrosis factor-α, transforming growth factor-ß, interleukin (IL) 10 and IL-4 (only mRNA expression) were determined at inclusion and after 8 weeks (group 1+2) and 18 weeks (group 2). Lesions and pruritus improved significantly from baseline to week 8. Mean improvements from baseline to week 18 were 23 per cent and 44 per cent for lesions and pruritus, respectively, an improvement of ≥50 per cent was seen in six out of nine and three out of six dogs, respectively. IL-4 mRNA expression decreased significantly. The results of this study show a clinical improvement of canine AD with CpG GNP comparable to allergen immunotherapy. Controlled studies are needed to confirm these findings.

Comparison of ultrasound imaging and video otoscopy with cross-sectional imaging for the diagnosis of canine otitis media.

Ultrasound imaging (US) of the tympanic bulla (TB) for diagnosis of canine otitis media (OM) is less expensive and less invasive than cross-sectional imaging techniques including computed tomography (CT) and magnetic resonance imaging (MRI). Video otoscopy (VO) is used to clean inflamed ears. The objective of this study was to investigate the diagnostic value of US and VO in OM using cross-sectional imaging as the reference standard. Client owned dogs with clinical signs of OE and/or OM were recruited for the study. Physical, neurological, otoscopic and otic cytological examinations were performed on each dog and both TB were evaluated using US with an 8 MHz micro convex probe, cross-sectional imaging (CT or MRI) and VO. Of 32 dogs enrolled, 24 had chronic otitis externa (OE; five also had clinical signs of OM), four had acute OE without clinical signs of OM, and four had OM without OE. Ultrasound imaging was positive in three of 14 ears, with OM identified on cross-sectional imaging. One US was false positive. Sensitivity, specificity, positive and negative predictive values and accuracy of US were 21%, 98%, 75%, 81% and 81%, respectively. The corresponding values of VO were 91%, 98%, 91%, 98% and 97%, respectively. Video otoscopy could not identify OM in one case, while in another case, although the tympanum was ruptured, the CT was negative. Ultrasound imaging should not replace cross-sectional imaging for the diagnosis of canine OM, but can be helpful, and VO was much more reliable than US.

[Deep brain stimulation using simultaneous stereotactic electrode placement: an alternative to conventional functional stereotaxy?]. / Tiefe Hirnstimulation mittels simultaner stereotaktischer Elektrodenplatzierung : Eine Alternative zur konventionellen funktionellen Stereotaxie?

BACKGROUND: Deep brain stimulation (DBS) has become a reliable method in the treatment of movement disorders, e.g. idiopathic Parkinson's disease (IPD) and is technically based on stereotaxy. The Starfix® platform is a new type of stereotactic frame that allows an individualized and patient-optimized therapeutic regimen in IPD. OBJECTIVES: The aim of this study was to retrospectively compare the outcomes of IPD patients who underwent surgery with the use of conventional stereotactic frames (31 patients) to those who underwent implantation of DBS with the use of Starfix® frames (29 patients). MATERIAL AND METHODS: Surgery time, the unified Parkinson's disease rating scale III (UPDRS/III) score, L-dopa and L-dopa equivalent doses (LED) were compared prior to surgery as well as 4 weeks, 12 weeks, 6 months and 1 year postoperatively. RESULTS: The IPD-related symptoms improved significantly in both groups with respect to the UPDRS III score (conventional 69.6% vs. 72.4% Starfix®). After surgery significant reductions of L-dopa and LED were seen in both groups. Inherent advantages of the Starfix® platform included simultaneous positioning of the stimulating electrodes and a significant reduction in surgical time. CONCLUSION: In summary, both stereotactic procedures are reliable and safe procedures for the placement of stimulating electrodes as well as the stimulation effect achieved. The logistical uncoupling of presurgical planning from surgical therapy emphasizes the benefits of the individualized stereotactic procedure.

Predicting intracerebral hemorrhage by baseline magnetic resonance imaging in stroke patients undergoing systemic thrombolysis.

OBJECTIVES: Intracerebral hemorrhage (ICH) remains a serious complication in ischemic stroke patients undergoing systemic thrombolysis. Here, we examined whether the risk of treatment-associated hemorrhage can be predicted from magnetic resonance imaging (MRI) using fluid-attenuated inversion recovery (FLAIR) and diffusion-weighted imaging (DWI) within 3 h after symptom onset. METHODS: In this single-center observational study involving 122 ischemic stroke patients between January 2005 and December 2008, the incidence of FLAIR-positive lesions within diffusion-restricted areas was determined on baseline MRI, which was carried out prior to treatment with tissue plasminogen activator (Actilyse(®) ) within 3 h from symptom onset. The rate of ICH was assessed by computed tomography performed within 24 h after treatment. Relationships between FLAIR-positive lesions, DWI lesion size, proportion of FLAIR/DWI-positive lesions, and occurrence of bleeding were explored. RESULTS: Data from 97 patients were evaluated. FLAIR-positive lesions were present in 25 patients (25.8%) and ICH occurred in 32 patients (33.0%). FLAIR-positive lesions were associated with a bleeding rate of 80.0% compared with 16.7% in FLAIR-negative patients (P < 0.001; odds ratio 20.0, positive predictive value 0.8). DWI lesion size was significantly correlated with the rate of ICH (P = 0.001). In contrast, FLAIR/DWI proportion was not associated with ICH (P = 0.788). CONCLUSIONS: In ischemic stroke patients within 3 h from symptom onset, the existence of FLAIR-positive lesions on pretreatment MRI is significantly associated with an increased bleeding risk due to systemic thrombolysis. Therefore, considering FLAIR-positive lesions on baseline MRI might guide treatment decisions in ischemic stroke.

Whole-brain diffusion tensor imaging in correlation to visual-evoked potentials in multiple sclerosis: a tract-based spatial statistics analysis.

BACKGROUND AND PURPOSE: Functional correlates of microstructural damage of the brain affected by MS are incompletely understood. The purpose of this study was to evaluate correlations of visual-evoked potentials with microstructural brain changes as determined by DTI in patients with demyelinating central nervous disease. MATERIALS AND METHODS: Sixty-one patients with clinically isolated syndrome or MS were prospectively recruited. The mean P100 visual-evoked potential latencies of the right and left eyes of each patient were calculated and used for the analysis. For DTI acquisition, a single-shot echo-planar imaging pulse sequence with 80 diffusion directions was performed at 3T. Fractional anisotropy, radial diffusivity, and axial diffusivity were calculated and correlated with mean P100 visual-evoked potentials by tract-based spatial statistics. RESULTS: Significant negative correlations between mean P100 visual-evoked potentials and fractional anisotropy and significant positive correlations between mean P100 visual-evoked potentials and radial diffusivity were found widespread over the whole brain. The highest significance was found in the optic radiation, frontoparietal white matter, and corpus callosum. Significant positive correlations between mean P100 visual-evoked potentials and axial diffusivity were less widespread, notably sparing the optic radiation. CONCLUSIONS: Microstructural changes of the whole brain correlated significantly with mean P100 visual-evoked potentials. The distribution of the correlations showed clear differences among axial diffusivity, fractional anisotropy, and radial diffusivity, notably in the optic radiation. This finding suggests a stronger correlation of mean P100 visual-evoked potentials to demyelination than to axonal damage.

Plasticity of the motor system in multiple sclerosis.

Multiple sclerosis (MS) is a chronic neurological disease characterized by inflammation and degeneration within the CNS. Over the course of the disease, most MS patients successively accumulate inflammatory lesions, axonal damage, and a rather diffuse CNS pathology, along with an increasing degree of disability. Pharmacological treatment options which are currently approved for MS aim at limiting inflammation and decreasing the relapse rate, or at simply relieving symptoms. Established disease-modifying and immunosuppressive treatments are unable to prevent the accumulation of pathology in most patients over long-term. Therefore, therapies promoting the innate ability of the CNS to compensate for dysfunction resulting from brain injury might be highly beneficial in MS. As a precondition, however, development of such strategies requires well-grounded knowledge about the extent to which central plasticity is intact and accessible in MS patients, and whether it is functionally relevant at all. This review will focus on plasticity of the motor system in patients with MS. A number of functional imaging studies have assessed patterns of brain activation during simple motor tasks in MS patients and their relationship with CNS damage and motor function. Deeper insights about causal and functional relationships were gained by neurophysiological techniques, predominantly by transcranial magnetic stimulation. In addition, and probably closest to rehabilitative approaches, practice-induced plasticity has been probed in a few studies. Altogether, there is growing evidence for a preservation of rapid-onset motor plasticity and for functionally relevant chronic reorganization processes, which might be limited by high CNS injury in advanced stages of the disease. Clinical implications of these findings with regard to the development and optimization of rehabilitative treatments in MS are discussed, as well as open questions which need to be addressed by future studies.

A new temporal window for inducing depressant associative plasticity in human primary motor cortex.

OBJECTIVE: Spike-timing dependent plasticity (STDP) usually refers to synaptic plasticity induced by near-synchronous activation of neuronal input and neuronal firing. However, some models of STDP predict effects that deviate from this tight temporal synchrony. We aimed to characterise the induction of STDP using paired associative stimulation (PAS) when the pre-synaptic input arrives in primary motor cortex (M1) at (i) intermediate intervals (50-80 ms; PAS(50),..PAS(80)) before the post-synaptic neuron is activated and (ii) long intervals (100-450 ms; PAS(-100),..PAS(-450)) after the post-synaptic neuron is activated. PAS at near-synchronicity (PAS(25)) was applied for comparison. METHODS: To characterise the physiological effects of the different PAS protocols, we examined short- and long-interval intra-cortical inhibition; intra-cortical facilitation and short- and long-latency afferent inhibition, in addition to recording MEPs in 45 healthy individuals. RESULTS: MEP amplitude was reduced at PAS intervals between -250 and -450 ms, increased with PAS(25), and unaltered at the remaining intervals. There was no change in intra-cortical inhibitory or facilitatory circuits following any PAS protocol. CONCLUSIONS: These findings provide evidence of a previously unreported temporal window in which PAS induces a depression of corticospinal excitability in human M1. SIGNIFICANCE: Establishing new temporal rules for STDP broadens its applicability for therapeutic usage in future.

Maintaining success, reducing treatment burden, focusing on survivorship: highlights from the third European consensus conference on diagnosis and treatment of germ-cell cancer.

In November 2011, the Third European Consensus Conference on Diagnosis and Treatment of Germ-Cell Cancer (GCC) was held in Berlin, Germany. This third conference followed similar meetings in 2003 (Essen, Germany) and 2006 (Amsterdam, The Netherlands) [Schmoll H-J, Souchon R, Krege S et al. European consensus on diagnosis and treatment of germ-cell cancer: a report of the European Germ-Cell Cancer Consensus Group (EGCCCG). Ann Oncol 2004; 15: 1377-1399; Krege S, Beyer J, Souchon R et al. European consensus conference on diagnosis and treatment of germ-cell cancer: a report of the second meeting of the European Germ-Cell Cancer Consensus group (EGCCCG): part I. Eur Urol 2008; 53: 478-496; Krege S, Beyer J, Souchon R et al. European consensus conference on diagnosis and treatment of germ-cell cancer: a report of the second meeting of the European Germ-Cell Cancer Consensus group (EGCCCG): part II. Eur Urol 2008; 53: 497-513]. A panel of 56 of 60 invited GCC experts from all across Europe discussed all aspects on diagnosis and treatment of GCC, with a particular focus on acute and late toxic effects as well as on survivorship issues. The panel consisted of oncologists, urologic surgeons, radiooncologists, pathologists and basic scientists, who are all actively involved in care of GCC patients. Panelists were chosen based on the publication activity in recent years. Before the meeting, panelists were asked to review the literature published since 2006 in 20 major areas concerning all aspects of diagnosis, treatment and follow-up of GCC patients, and to prepare an updated version of the previous recommendations to be discussed at the conference. In addition, ∼50 E-vote questions were drafted and presented at the conference to address the most controversial areas for a poll of expert opinions. Here, we present the main recommendations and controversies of this meeting. The votes of the panelists are added as online supplements.

Focal hand dystonia: lack of evidence for abnormality of motor representation at rest.

OBJECTIVE: To examine whether the corticospinal system emanating from the primary motor cortex may be organized to facilitate generation of dystonic movements. METHODS: In this cross-sectional observational study, finger movement (FM) representations were assessed in 10 patients with focal hand dystonia (FHD) and 10 matched healthy controls by transcranial magnetic stimulation during rest. Evoked finger movements of the right hand were recorded using an instrumented data glove. Patterns of finger joint movements were analyzed using cluster analysis. Principal component analysis and centers of gravity for finger movement representations and motor evoked potentials recorded from the abductor pollicis brevis and abductor digiti minimi muscles were computed. For comparison, high-resolution somatosensory evoked potentials (SSEP) were recorded after electrical stimulation of the thumb (D1) or little finger (D5) in the same patients. Source reconstruction for the N20 SSEP component was performed using a dual-dipole model. RESULTS: Stimulation of the resting motor cortex did not reveal overt abnormalities in FHD, neither with respect to finger joint movement patterns nor with respect to the topologic organization of finger movements or intrinsic hand muscle representations. However, in line with previous reports, the distance between the dipole sources of D1 and D5 in the somatosensory cortex (S1) was smaller in patients with FHD, suggesting disruption of homuncular finger representations in S1. CONCLUSIONS: Our findings may imply that abnormality of motor organization in focal hand dystonia arises principally only during activation, when abnormal somatosensory representations are functionally integrated.

[Critical illness myopathy and neuropathy (CRIMYN). Electroneurographic classification]. / Critical-illness-Myopathie und -Neuropathie (CRIMYN). Elektroneurographische Klassifikation.

BACKGROUND: Critical Illness Myopathy and Neuropathy (CRIMYN) frequently coexist with severe sepsis and is associated with prolonged weaning from mechanical ventilation and prolonged ICU length of stay. We aimed to classify different levels as well as patterns of impairment with regard to electrophysiological disturbances in CRIMYN patients by cluster analysis. METHODS: A total of 30 patients with sepsis/SIRS were studied prospectively. Motor and sensory conduction studies were performed from six motor and four sensory nerves on a weekly basis from admission until discharge and finally after 6 months. A control group of 63 healthy persons was examined simultaneously using the same criteria. Different patterns of electrophysiological disturbances were classified by cluster analysis based on differences to reference values of 20 parameters, compound muscle action potential (CMAP), sensory nerve action potential (SNAP) and motor and sensor conduction velocity (NCV). RESULTS: Four different clusters were identified: cluster 1 showing normal values for CMAP, SNAP and NCV in all nerves (3 patients and all test persons), cluster 2 showing pathological values for CMAP in the lower extremities and the other parameters were normal (5 patients), cluster 3 showing moderately pathological values for CMAP, SNAP and sensory NCV in upper and lower extremities and motor NCV in lower extremities (12 patients) and cluster 4 showing severe disturbances of CMAP, SNAP and NCV in upper and lower extremities (10 patients). CONCLUSION: A total of four different clusters of electrophysiological impairment can be identified in patients with sepsis/SIRS, which enables further differentiation of the severity of neuromuscular disturbances in sepsis-associated organ failure. This might be useful as a prognostic parameter and can be correlated with additional clinical and paraclinical parameters related to sepsis.

Rapid-onset central motor plasticity in multiple sclerosis.

OBJECTIVE: To study rapid-onset central motor plasticity, and its relationship to motor impairment and CNS injury in patients with multiple sclerosis (MS). METHODS: In this cross-sectional observational study, motor plasticity was examined neurophysiologically and behaviorally in 22 patients with moderately severe (median Expanded Disability Status Scale score 2.5 [0-6]) stable MS and matched healthy controls. First, plasticity was assessed using paired associative stimulation (PAS), a protocol modeling long-term synaptic potentiation in human cortex. PAS combines repetitive electric nerve stimulation with transcranial magnetic stimulation (TMS) of the contralateral motor cortex. Second, motor learning was tested by a force production task. Motor impairment was assessed by functional tests. CNS injury was evaluated by obtaining normalized N-acetyl-aspartate (NAA/Cr) spectra using magnetic resonance spectroscopy and by the corticomuscular latency (CML) to the abductor pollicis brevis muscle as tested by TMS. RESULTS: Patients with MS performed worse than controls in functional motor tests, CMLs were prolonged, and NAA/Cr was decreased. PAS-induced enhancement of corticospinal excitability and training-induced increments of motor performance were comparable between patients with MS and controls. Neither PAS-induced plasticity nor motor learning performance correlated with motor impairment or measures of CNS injury. Patients with high CNS injury and good motor performance did not differ significantly from those with high CNS injury and poor motor performance with respect to PAS-induced plasticity and motor learning success. CONCLUSIONS: Despite motor impairment and CNS injury in patients with multiple sclerosis (MS), rapid-onset motor plasticity is comparable to that in healthy subjects. Compensation of MS-related CNS injury is unlikely to be constrained by insufficient rapid-onset neuroplasticity.

[Early symptoms and findings in optic nerve meningiomas]. / Erstsymptome und Befunde bei Meningeomen des N. opticus.

PURPOSE OF THE STUDY: While evaluating radiotherapy results in patients with primary and secondary optic nerve sheath meningiomas (ONSM) treated between 1993 and 2002, a large amount of data about early signs and symptoms has been collected which might be helpful for establishing an early diagnosis. METHODS: We have reviewed the charts of the patients available at the Centre of Ophthalmology, collecting especially pretreatment data. RESULTS: 112 patients, 4 with bilateral tumours, 83 % female were included. Mean age was 51.7 years. Visual acuity loss with relative afferent pupillary defect was the main symptom and sign in primary ONSM (38 patients). Median interval between first symptoms and diagnosis was 12 months. Optic discs were in approximately one half atrophic, the other half were swollen, and only rarely normal (3 cases only). Retinociliary shunt vessels were seen in 10 cases. Nerve fibre bundle defects were the major visual field finding (including constriction and central scotoma). Visual acuity was better than 0.5 (20 / 40) in 46 % and worse than 0.1 (20 / 200) in 30 %. In secondary ONSM, the interval to diagnosis was with a median of 6 months shorter than in primary ONSM. Approximately half of the optic discs were atrophic, only 6 % were swollen. Even here nerve fibre bundle defects were dominating, only 7 % had vertical hemianopic defect. Visual acuity was better than 0.5 (20 / 40) in 30 % and in worse than 0.1 (20 / 200) 22 %. 45 % had ocular motility disorders. DISCUSSION: Vertical hemianopic defects were surprisingly rare. The high rate of nerve fibre bundle defects and the relatively high number of patients with good visual acuity might explain why this disorder is occasionally mistaken for glaucoma. A typical clinical appearance can be outlined: mainly mid-aged women, slowly progressing visual loss, frequently motility disorders, relative afferent pupillary defect, nerve fibre bundle defects and atrophic or--mainly in primary ONSM--swollen optic disc form the characteristic picture.