Articles in 2016

A new study reports that the incidence of dementia in one population has declined by over 40% between the 1970s and 2010s. The finding could have implications for the future prevalence of dementia, and illustrates the importance of environmental factors in the risk of dementia disorders.

The characteristic muscle weakness in myasthenia gravis (MG) is caused by antibodies against target molecules the neuromuscular junction. Here, Nils Gilhus and colleagues review how these antibodies and other biomarkers can be used to guide MG subgroup classification and treatment, as well as the best tests to detect these antibodies. Moreover, they discuss how the various antibodies are involved in MG pathogenesis.

A recent study of clinical and genetic characteristics in patients with hereditary spastic paraplegia highlights the difficulties of making clinicogenetic correlations in a heterogeneous group of diseases. Genetic analysis beyond the causative variants, and independent of the core clinical symptoms (pyramidal signs versus ataxia), might offer a more pertinent way to approach phenotypic variability.

The end of 2015 saw the passing of John F. Kurtzke and Richard T. Johnson, two legends in neurology, and founding fathers of neuroepidemiology, neurovirology and neuroimmunology. Here, we pay tribute to these two giants by discussing their impact on the genesis of these profoundly important disciplines.

Two new studies highlight the potential of neuroimaging to aid the differential diagnosis of neurodegenerative disease, for both clinical practice and emerging trials. Although this approach holds great promise, meaningful implementation of neuroimaging as part of a tailored precision medicine strategy may require additional imaging and non-imaging biomarkers.

In addition to the functional impairments that can arise after stroke, about one-third of stroke survivors experience neuropsychiatric disorders such as depression, anxiety or apathy. In this Review, Ferro and colleagues provide an update on the diagnosis and pathophysiology of the acute and long-term psychiatric consequences of stroke, and discuss available treatments and management strategies for these stroke-associated disorders.

Nonconvulsive status epilepticus has subtle symptoms and can be difficult to treat, meaning it is associated with considerable morbidity and mortality. In this Review, Sutter and colleagues discuss the epidemiology, clinical features and diagnosis of nonconvulsive status epilepticus, and discuss current recommendations for treatment.

The limb-girdle muscular dystrophies (LGMDs) comprise more than 30 genetically defined neuromuscular diseases that share the determining features of weakness and wasting of the pelvic and shoulder girdle muscles. Next-generation sequencing (NGS) technologies are continuing to expand the range of genes and phenotypes associated with the LGMDs. In this article, Thompson and Straub review the international collaborations that are addressing translational research issues in the LGMDs, and the lessons learned from large-scale NGS programmes.