One typical use of negation is to refer to exceptions. In a visual display showing several similar items (majority) and one exception, referring to the exception by negating the majority should therefore be pragmatically felicitous. We investigated whether comprehenders are sensitive to these pragmatic aspects when processing negative sentences and having to identify the according items in the visual display. In Experiment 1, participants read affirmative and negative sentences referring to either the exception or the majority object in strongly biased displays. Additionally, unbiased displays were implemented, showing equal numbers of objects of each type. Identification times of the correct referent were shorter with the biased display independent of sentence polarity. Also, picking the exceptional item in the biased display was faster than picking a majority item, independent of sentence polarity. Thus, participants did not specifically profit from pragmatically felicitous conditions when processing negation. Critically, in the biased displays, the exceptional object was highly salient, which might have initially drawn the participant’s attention to this object, resulting in a general speed-up. Therefore, in Experiment 2, we used a biased display with reduced saliency of the exceptional object. Again negation did not result in a specific speed-up due to pragmatically correct negation use. Thus, negation does not seem to facilitate the identification of an exceptional object.

Frontotemporal dementia (FTD) is a devastating neurodegenerative condition for which there is currently no effective treatment. Although it is much less common than Alzheimer’s disease, the impact of FTD is increased by its relatively early onset and high heritability. Clinical heterogeneity and overlap with other neurodegenerative and psychiatric syndromes complicate diagnosis. However, recent advances in our understanding of the molecular basis of FTD provide a foundation for the development of much-needed biomarkers and targeted therapies. This review provides a summary of the recently revised clinical criteria for FTD, highlights diagnostic challenges, briefly summarizes recent molecular discoveries and then focuses on promising developments in biomarkers and clinical trials.

There are suggestions that large evening meals are associated with greater BMI. This study reviewed systematically the association between evening energy intake and weight in adults and aimed to determine whether reducing evening intake achieves weight loss. Databases searched were MEDLINE, PubMed, Cinahl, Web of Science, Cochrane Library of Clinical Trials, EMBASE and SCOPUS. Eligible observational studies investigated the relationship between BMI and evening energy intake. Eligible intervention trials compared weight change between groups where the proportion of evening intake was manipulated. Evening intake was defined as energy consumed during a certain time – for example 18.00–21.00 hours – or self-defined meal slots – that is ‘dinner’. The search yielded 121 full texts that were reviewed for eligibility by two independent reviewers. In all, ten observational studies and eight clinical trials were included in the systematic review with four and five included in the meta-analyses, respectively. Four observational studies showed a positive association between large evening intake and BMI, five showed no association and one showed an inverse relationship. The meta-analysis of observational studies showed a non-significant trend between BMI and evening intake (P=0·06). The meta-analysis of intervention trials showed no difference in weight change between small and large dinner groups (−0·89 kg; 95 % CI −2·52, 0·75, P=0·29). This analysis was limited by significant heterogeneity, and many trials had an unknown or high risk of bias. Recommendations to reduce evening intake for weight loss cannot be substantiated by clinical evidence, and more well-controlled intervention trials are needed.

Feature Inheritance (Richards 2007) entails that uninterpretable features originate on the phase head C or v* and are then transferred to the associated Agree head, T or V. In the present article, it is argued that the French que–qui alternation is the locus of a Case contrast, implying that nominative Case originates on the complementiser and only becomes associated with T as a consequence of feature transfer. Que–qui thus provides new, Case-based empirical support for the theory of Feature Inheritance. The article also suggests that the que–qui alternation has an important implication for Chomsky's recent application of dynamic antisymmetry, reinterpreted in terms of labelling, to the issue of subject extraction failure. Specifically, the alternation appears to indicate that Case-matching is required, in addition to phi-feature agreement, in order for extraction to be blocked by labelling.

Human rabies encephalitis is rare in Canada, with only five cases reported in the past 30 years. The first and only patient who contracted rabies encephalitis in British Columbia died in 2003. Here we provide the first detailed clinical and pathological description of that case, which had several unusual features, including preexisting immunosuppression, paralytic presentation, prolonged survival, focal lesions on neuroimaging and severe neuropathology with focal necrosis, intense inflammation, and abundant viral inclusion bodies.

Mutations in the progranulin gene (GRN) are a common cause of familial frontotemporal dementia. We used a comprehensive neuropsychological battery to investigate whether early cognitive changes could be detected in GRN mutation carriers before dementia onset. Twenty-four at-risk members from six families with known GRN mutations underwent detailed neuropsychological testing. Group differences were investigated by domains of attention, language, visuospatial function, verbal memory, non-verbal memory, working memory and executive function. There was a trend for mutation carriers (n=8) to perform more poorly than non-carriers (n=16) across neuropsychological domains, with significant between group differences for visuospatial function (p<.04; d=0.92) and working memory function (p<.02; d=1.10). Measurable cognitive differences exist before the development of frontotemporal dementia in subjects with GRN mutations. The neuropsychological profile of mutation carriers suggests early asymmetric, right hemisphere brain dysfunction that is consistent with recent functional imaging data from our research group and the broader literature. (JINS, 2014, 20, 1–10)

Iodine deficiency affects an estimated 241 million school-aged children in the world. Little is known about iodine deficiency in relation to auditory function, except for the fact that deaf–mutism is one of the features of cretinism. In the present review, we documented the scientific knowledge on the role of iodine and hypothyroidism in the auditory system. We found that ear development and hearing function depend on thyroid hormones. Multiple pathways are involved in this, including both inner ear morphology as well as neurological processes. Conductive as well as sensorineural hearing loss is found in studies with animals that were rendered hypothyroidic. In humans, auditory impairment is reported frequently in relation to hypothyroidism, ranging from mild disturbances to severe handicap. Auditory impairment has been related more explicitly to congenital hypothyroidism than to acquired hypothyroidism. The critical period for thyroid function-related hearing maturation is the first and second trimesters of pregnancy. Although only a limited number of studies have directly investigated the relationship between iodine deficiency and auditory function, most studies point toward an association. However, evidence from good randomised controlled trials is lacking. Inclusion of auditory outcomes in iodine supplementation studies is therefore to be recommended, especially for trials in pregnancy. Hearing deficit is an invisible abnormality, but has major consequences for educational and social skills if not detected. In view of this, auditory impairment should be mapped in iodine-deficient areas in order to realistically estimate the magnitude of the problem.

Population modelling is an invaluable tool for identifying effective management strategies for threatened species whose populations are too small for experimental manipulation. Recently developed Bayesian approaches allow us to combine deterministic models with probability distributions to create stochastic models that account for uncertainty. We illustrate this approach in the case of the takahe Porphyrio hochstetteri, an Endangered flightless rail, which is supported by one of New Zealand's costliest recovery programmes. Using mark–recapture and logistic regression models implemented in a Bayesian framework we calculated demographic parameters for a fully stochastic population model based on 25 years of data collected from the last wild population of takahe in the Murchison Mountains, Fiordland. Our model results show that stoat trapping, captive rearing and cross-fostering of eggs/chicks in wild pairs all have a positive effect on takahe demography. If it were not for these management actions the Fiordland population would probably be declining (λ = 0.985; confidence interval, CI = 0.39–1.08), with a non-negligible risk of quasi-extinction (P = 16%) within 20 years. The captive rearing of eggs and chicks has been the main factor responsible for the positive growth observed during the last decade but in the future expanding stoat trapping to cover the entire Murchison Mountains would be the single most beneficial management action for the takahe population (λ = 1.038; CI = 0.86–1.10), followed by captive rearing (λ = 1.027; CI = 0.85–1.09).