Reviews & Analysis

In patients with stroke, weekend hospitalization has been associated with increased mortality and worse outcomes compared with hospitalization on weekdays, prompting calls for interventions to improve off-hours hospital care. New data suggest that organized systems of stroke care could be one means of attenuating this so-called weekend effect.

The pathological underpinnings of Alzheimer disease (AD) are now known to begin up to two decades before manifestation of clinical disease, and intervention during preclinical AD stages is increasingly recognized as key to therapeutic success. Here, Eric Reiman and colleagues discuss strategies to study changes in the brain and bodily fluids that precede clinical AD, focusing in particular on genetic at-risk individuals, who might be suitable candidates for secondary prevention trials.

Decompressive craniectomy (DC)—a surgical procedure that involves removal of part of the skull—has been used for many years in the management of patients with brain oedema and/or intracranial hypertension; however, the risk of post-surgery disability has raised important ethical issues. Here, Kolias et al. outline the history of DC, and review current considerations and evidence with regard to the use of this procedure in stroke, traumatic brain injury and other indications. The direction of future studies of DC is also discussed.

Race is a recognised risk factor for intracerebral haemorrhage (ICH). A new study reports that the incidence of ICH is higher in black individuals than white people in young populations. In elderly populations, however, ICH incidence is higher in white than black individuals, suggesting ethnicity-related variation in ICH aetiology.

Uhthoff's phenomena are transient disturbances in neurological functioning that can be triggered by an increase in core body temperature in patients with multiple sclerosis (MS). In this article, Frohman et al. discuss the pathophysiological underpinnings of Uhthoff's phenomena, and highlight the importance of differentiating between these events and true MS exacerbations in the clinic.

Daclizumab is a humanized monoclonal antibody that targets the IL-2 receptor α chain, and has shown promise as a novel treatment for multiple sclerosis (MS). Here, Wiendl and Gross provide an overview of clinical experience with daclizumab in MS, including results from phase II trials. They also discuss the putative mechanisms of action of this drug in MS pathogenesis, which involve dampening of early T-cell activation and upregulation of immunomodulatory natural killer cells.

The average age of the elderly population is increasing. In this Review, the authors examine the prevalence of dementia in the elderly population, and especially in the oldest old. Furthermore, they discuss the absence of dementia in the oldest old as a model of successful ageing, and the impact of genetic and environmental factors on various dementia phenotypes.

A recent study of a large multi-ethnic cohort has shown that cumulative serum titres of antibodies against five common microbial pathogens are associated with an increased risk of cognitive impairment. Further studies are needed to better understand this risk and enable translation of the findings into effective preventative strategies.

Surgical intervention in children with hypothalamic chiasmatic low-grade glioma, diffuse intrinsic pontine glioma, or high-grade glioma is controversial, and randomized controlled trials are lacking. A recent consensus statement provides recommendations for optimal management of these patients, including situations in which surgical resection represents the preferred option.

Definition, evaluation and management of concussion are contentious issues in sports medicine, and evidence-based guidelines on these issues are urgently needed. An updated practice parameter on sport-related concussion from the AAN is, therefore, timely. However, these new guidelines fail to meet the needs of clinicians in this field.

Primary CNS lymphoma (PCNSL) is a rare malignancy with an aggressive course, for which treatment has yet to be optimized. Here, Korfel and Schlegel discuss diagnostic approaches in patients with suspected PCNSL, and review the latest studies on new and more established therapeutic interventions. Ongoing issues—including the utility of whole-brain radiotherapy, and treatment of young, elderly and immunocompromised patients—are also considered.

This Review focuses on the growing area of telemedicine in the diagnosis and treatment of patients with acute stroke who are geographically remote from primary care centres. Hess and Audebert discuss telemedicine as a means to improve the availability of care for stroke patients, and the financial implications of this approach. Furthermore they highlight technological advances that might help to overcome barriers to the wider application of telemedicine in acute stroke care.

Systemic inflammation has been associated with dementia. A new study has found an increased rate of autoimmune disorders and elevated serum levels of tumour necrosis factor in patients with frontotemporal dementia associated with TDP-43 pathology. These findings help in understanding disease mechanisms and in identification of biomarkers of this heterogeneous disease.

Psychogenic movement disorders (PMDs) manifest with physical symptoms but currently lack a clear pathology and standard of care. However, some physicians support the use of placebo therapy for patients with PMDs. In this Perspectives article, Karen Rommelfanger outlines the need for further research and international dialog regarding the treatment of patients with PMDs, and the use of placebo therapy in these patients.

Susac syndrome is an important differential diagnosis in many neurological disorders, but our understanding of this rare disease has largely been limited to reports of single cases and small case series. In this article, Dörr and colleagues review all reported cases of Susac syndrome to provide a comprehensive overview of demographic, clinical and diagnostic data on this disorder. On the basis of their findings, Dörr et al. make recommendations for diagnosis and management of individuals with suspected Susac syndrome.

Genetic diagnosis of frontotemporal lobar degeneration has become challenging since the identification of a number of autosomal dominantly inherited causative mutations. As highlighted in a new paper, careful characterization of the clinical picture associated with a specific gene defect is mandatory to guide genetic screening, and to establish a diagnostic algorithm for neurology practice.

Cerebrovascular disease is the most frequently identified antecedent of new-onset seizures or epilepsy in industrialized countries. Three recent publications have evaluated the risk of epilepsy following a first cerebrovascular insult. Each report provides evidence for a substantial risk in some patient subgroups, although the study methodologies and definitions of epilepsy vary.

Neurological diseases that result from an underlying genetic mutation or from acquired genetic and/or epigenetic changes are prevalent in the population, and might be amenable to gene therapy. In this Review, Simonato et al. discuss new developments in gene therapy vector design and delivery for the treatment of neurological diseases, including sensorineural disorders, Parkinson disease and epilepsy. The authors also outline the most promising therapeutic approaches to date.

Multiple sclerosis and neuromyelitis optica—two inflammatory demyelinating diseases with different pathogenesis and treatment regimens—have overlapping clinical manifestations, which can make diagnosis at initial presentation difficult. Now, a new guideline aims to improve early treatment decisions in patients with these disorders, particularly in the Asia–Pacific region.

Accumulating evidence suggests that statin therapy could assist functional recovery following acute ischaemic stroke. A recent study indicates that early administration of statins after intravenous thrombolysis for stroke improves functional outcomes, and reduces mortality and neurological deterioration. Owing to the observational study design, however, these findings should be interpreted cautiously.