Eye Movement Evaluation in Multiple Sclerosis and Parkinson's Disease Using a Standardized Oculomotor and Neuro-Ophthalmic Disorder Assessment (SONDA)

Evaluating the state of the oculomotor system of a patient is one of the fundamental tests done in neuro-ophthalmology. However, up to date, very few quantitative standardized tests of eye movements' quality exist, limiting this assessment to confrontational tests reliant on subjective interpretation. Furthermore, quantitative tests relying on eye movement properties, such as pursuit gain and saccade dynamics are often insufficient to capture the complexity of the underlying disorders and are often (too) long and tiring. In this study, we present SONDA (Standardized Oculomotor and Neurological Disorder Assessment): this test is based on analyzing eye tracking recorded during a short and intuitive continuous tracking task. We tested patients affected by Multiple Sclerosis (MS) and Parkinson's Disease (PD) and find that: (1) the saccadic dynamics of the main sequence alone are not sufficient to separate patients from healthy controls; (2) the combination of spatio-temporal and statistical properties of saccades and saccadic dynamics enables an identification of oculomotor abnormalities in both MS and PD patients. We conclude that SONDA constitutes a powerful screening tool that allows an in-depth evaluation of (deviant) oculomotor behavior in a few minutes of non-invasive testing

How visually impaired cyclists ride regular and pedal electric bicycles

The present study investigates whether visually impaired cyclists compensate for their vision limitations by maintaining a lower speed or a larger distance to the kerb than normally sighted cyclists when riding a regular bicycle or pedal electric bicycle (pedelec). A normally sighted control group (n = 10), a peripheral visual field loss group (n = 9), and a low visual acuity group (n = 12) rode a fixed route (7.5 km) in the Netherlands on a regular bicycle and on a pedelec. Speed and lateral position were measured when participants cycled a (I) one-way cycle path, (II) two-way cycle path, (III) residential area, and (IV) shared space zone. With regard to both the regular bicycle and the pedelec, no significant speed or lateral position differences were found between the three groups. In conclusion, for some people with severe and permanent visual impairments, and under certain circumstances, regular bicycle and pedelec riding may be possible without noticeable speed reduction or adapted lane position to compensate for their functional impairment. The present findings may further optimise the cycling advice provided by mobility trainers of vision rehabilitation centres and the independent mobility of visually impaired people

The Birthday Party Test (BPT); A new picture description test to support the assessment of simultanagnosia in patients with acquired brain injury

There is broad consensus on the utility of complex pictures in the assessment of simultanagnosia in patients with acquired brain injury (ABI). To overcome various shortcomings of current instruments, we have developed the Birthday Party Test (BPT); a picture description test that contains a neutral scene, a balanced representation of events, and provides clear instructions and a scoring-aid. We have applied the BPT in a large group of patients with ABI (n = 502) and in an age-matched healthy control group (n = 194). Our results show that performance on the BPT was associated with a range of descriptive, neuropsychological and clinical characteristics and that poor test performance appeared to be more common in patients with etiologies that have an increased risk of bilateral damage. Furthermore, we assume a high correspondence between test performance on the BPT and the assessor’s clinical judgment of likely having simultanagnosia in preliminary analyses. This study shows the potential usefulness of the BPT to support diagnostic decision making in simultanagnosia. The BPT is made freely available to facilitate its broad application in the clinical assessment of patients with visual impairment and to enable a further evaluation of its utility and validity in future studies

Drosophila DJ-1 Mutants Are Selectively Sensitive to Environmental Toxins Associated with Parkinson’s Disease

SummaryParkinson’s disease (PD) is a common neurodegenerative disorder that displays both sporadic and inherited forms [1]. Exposure to several common environmental toxins acting through oxidative stress has been shown to be associated with PD [2]. One recently identified inherited PD gene, DJ-1, may have a role in protection from oxidative stress [3–10], thus potentially linking a genetic cause with critical environmental risk factors. To develop an animal model that would allow integrative study of genetic and environmental influences, we have generated Drosophila lacking DJ-1 function. Fly DJ-1 homologs exhibit differential expression: DJ-1β is ubiquitous, while DJ-1α is predominantly expressed in the male germline. DJ-1α and DJ-1β double knockout flies are viable, fertile, and have a normal lifespan; however, they display a striking selective sensitivity to those environmental agents, including paraquat and rotenone, linked to PD in humans. This sensitivity results primarily from loss of DJ-1β protein, which also becomes modified upon oxidative stress. These studies demonstrate that fly DJ-1 activity is selectively involved in protection from environmental oxidative insult in vivo and that the DJ-1β protein is biochemically responsive to oxidative stress. Study of these flies will provide insight into the critical interplay of genetics and environment in PD

Car Driving Performance in Hemianopia:An On-Road Driving Study

PURPOSE. To study driving performance in people with homonymous hemianopia (HH) assessed in the official on-road test of practical fitness to drive by the Dutch driver's licensing authority (CBR).METHODS. Data were collected from a cohort (January 2010-July 2012) of all people with HH following the official relicensure trajectory at Royal Dutch Visio and the CBR in the Netherlands. Driving performance during the official on-road tests of practical fitness to drive was scored by professional experts on practical fitness to drive, using the visual impairments protocol and a standardized scoring of visual, tactical and operational aspects. Age ranged from 27 to 72 years (mean = 52, SD = 11.7) and time since onset of the visual field defect ranged from 6 to 41 months (mean = 15, SD = 7.5).RESULTS. Fourteen (54%) participants were judged as fit to drive. Besides poor visual scanning during driving, specific tactical, and operational weaknesses were observed in people with HH that were evaluated as unfit to drive. Results suggest that judgement on practical fitness to drive cannot be based on solely the visual field size. Visual scanning and operational handling of the car were found to be more impaired with longer time not driven, while such an effect was not found for tactical choices during driving.CONCLUSIONS. Training programs aimed at improving practical fitness to drive in people with HH should focus on improving both visual scanning, as well as driving aspects such as steering stability, speed adaptation, and anticipating environmental changes.</p

Acute toxicity of organic pesticides to Daphnia magna is unchanged by co-exposure to polystyrene microplastics

Daphnia magna were exposed to two pesticides in the presence or absence of microplastics (300 000 particles ml−1 1 µm polystyrene spheres) and to microplastics alone. The pesticides were dimethoate, an organophosphate insecticide with a low log Kow, and deltamethrin, a pyrethroid insecticide with a high log Kow. Daphnia were exposed to a nominal concentration range of 0.15, 0.31, 0.63, 1.25, 2.5, 5 mg l−1 dimethoate and 0.016, 0.08, 0.4, 2, 5 and 10 µg l−1 deltamethrin. Exposure to polystyrene microplastics alone showed no effects on Daphnia magna survival and mobility over a 72 h exposure. In the dimethoate exposures, mobility and survival were both affected from a concentration of 1.25 mg l−1, with effects were seen on mobility from 28 h and survival from 48 h, with greater effects seen with increasing concentration and exposure time. In deltamethrin exposures, survival was affected from a concentration of 0.4 µg l−1 and mobility from a concentration of 0.08 µg l−1. Effects of deltamethrin on mobility were seen from 5 h and on survival from 28 h, with greater effects on survival and mobility seen with increasing concentration and exposure time. Contrary to expectations, pesticide toxicity to Daphnia magna was not affected by the presence of microplastics, regardless of chemical binding affinity (log Kow). This therefore suggests that polystyrene microplastics are unlikely to act as a significant sink, nor as a vector for increased uptake of pesticides by aquatic organisms

Mutation Frequency of the Major Frontotemporal Dementia Genes, MAPT, GRN and C9ORF72 in a Turkish Cohort of Dementia Patients

‘Microtubule-associated protein tau’ (MAPT), ‘granulin’ (GRN) and ‘chromosome 9 open reading frame72’ (C9ORF72) gene mutations are the major known genetic causes of frontotemporal dementia (FTD). Recent studies suggest that mutations in these genes may also be associated with other forms of dementia. Therefore we investigated whether MAPT, GRN and C9ORF72 gene mutations are major contributors to dementia in a random, unselected Turkish cohort of dementia patients. A combination of whole-exome sequencing, Sanger sequencing and fragment analysis/Southern blot was performed in order to identify pathogenic mutations and novel variants in these genes as well as other FTD-related genes such as the ‘charged multivesicular body protein 2B’ (CHMP2B), the ‘FUS RNA binding protein’ (FUS), the ‘TAR DNA binding protein’ (TARDBP), the ‘sequestosome1’ (SQSTM1), and the ‘valosin containing protein’ (VCP). We determined one pathogenic MAPT mutation (c.1906C>T, p.P636L) and one novel missense variant (c.38A>G, p.D13G). In GRN we identified a probably pathogenic TGAG deletion in the splice donor site of exon 6. Three patients were found to carry the GGGGCC expansions in the non-coding region of the C9ORF72 gene. In summary, a complete screening for mutations in MAPT, GRN and C9ORF72 genes revealed a frequency of 5.4% of pathogenic mutations in a random cohort of 93 Turkish index patients with dementia