Reviews & Analysis

The implementation of array comparative genomic hybridization in clinical practice has helped to identify syndromes in patients that were previously unsuspected. This Case Study describes a patient with mental retardation who was found to have an interstitial deletion of chromosome 5 encompassing the adenomatous polyposis coli tumor suppressor locus, with major implications for her care and management.

A range of impulse control disorders (ICDs), including pathological gambling, compulsive shopping and impulsive aggression, has been recognized in patients with Parkinson's disease (PD). This Review provides the neurologist with an understanding of the clinical and biological features of ICDs and the nature of their relationship with PD. The authors also offer guidance on the management of PD patients with an ICD.

Despite advances in treatment approaches for patients with high-grade glioma, the overall prognosis remains poor. In this article, Castellino and Durden highlight the important role of angiogenesis in the pathogenesis of brain tumors, and discuss the development of new therapies that target angiogenesis in high-grade glioma by regulating the activity of the PI3K–Akt–PTEN signaling node.

It is estimated that around one-third of patients with new-onset epilepsy will eventually develop medically intractable seizures. Many patients with intractable epilepsy are likely to benefit from surgery, which, by controlling the seizures, can substantially improve quality of life and reduce the costs of medical care. In this article, Kuzniecky and Devinsky review the current surgical management of epilepsy, including identification of candidates, localization of the seizure focus, and surgical techniques.

Despite decades of debate, no consensus has been reached on the role of carotid endarterectomy in patients with asymptomatic carotid stenosis. Anne Abbott argues that the time for deliberation is over. In this Viewpoint, she makes a case for the suspension of surgery in favor of medical interventions.

The question of whether carotid endarterectomy should be performed on patients with asymptomatic carotid stenosis is one of the big medical controversies of our time. Uncertainty over surgical benefits hasn't stopped people from operating, however, with surgery rates soaring in some countries. Vincent Thijs reminds clinicians of the open questions in the field and calls for a more selective and cautious approach to surgery.

The search for the perfect pain reliever continues, with several drugs targeting pain-specific pathways in clinical and preclinical development. Pain is a protective mechanism, however, so complete pain control might also have its downsides. This Viewpoint aims to launch a debate on how the use of a perfect pain blocker can be implemented in a safe and effective fashion.

In this Case Study, the authors describe how sulfonylurea therapy not only restored glucose homeostasis, but also alleviated some of the neurological symptoms in a patient with intermediate DEND syndrome. Single-photon emission CT provided evidence of increased CNS blood flow and function that correlated with mental and motor improvements.

The extent to which white matter lesions affect brain function in elderly individuals has important implications for the development of drugs to treat cognitive impairment and dementia. In this article, Frisoni et al. argue that white matter lesions can contribute to cognitive impairment in elderly individuals, although their effects are minor when compared with those of other causes of cognitive deterioration, such as Alzheimer's disease.

Migraine is a common disabling brain disorder that—considering its clinical and economic impact—is understudied and in need of additional management options. In this Review, Peter Goadsby outlines new pharmaceutical and device-based approaches to migraine treatment by describing their potential mechanisms of action and the clinical data that are currently available.

Surgery is becoming a viable treatment option for an increasing proportion of patients with low-grade gliomas, but there is a lack of class I evidence to support its efficacy. In this Review, Pouratianet al. critically analyze the utility of surgery in low-grade glioma. They highlight recent technological advances that have made this approach safer and more accessible, and consider the challenges involved in designing trials to assess the extent to which aggressive resection improves prognosis.

Prematurely born infants are at high risk of brain injury, but although structural and volumetric alterations often persist into later life, there is strong evidence of recovery from cognitive impairments associated with preterm birth. In this Review, Ment and Constable discuss how functional MRI techniques are providing important insights into the adaptive mechanisms of the developing preterm brain, including the engagement of alternative neural networks for language and memory.

Small-fiber neuropathy is a condition that has been underdiagnosed in the past, partly because small nerve fibers cannot be observed using routine neurophysiological tests. Skin biopsy is a safe, minimally invasive tool that can provide important diagnostic information on small nerve fibers, and in this article Lauria and Devigili discuss how this approach is being applied to the diagnosis and monitoring of peripheral neuropathies.

Findings in animal models of Huntington's disease and levodopa-induced dyskinesia indicate that corticostriatal long-term potentiation is abnormally stable in these conditions, causing persistent inhibition of the output nuclei of the basal ganglia, which leads to involuntary movements. In this article, Centonze et al. review evidence that repetitive transcranial magnetic stimulation can interfere with the maintenance of corticostriatal long-term potentiation in humans, and might, therefore, be useful as an antidyskinetic treatment.

There is strong evidence that clinical trials with a positive outcome are more likely to be published than are trials with negative results. In this Viewpoint, Richard Johnson and Kay Dickersin explore the reasons for this phenomenon and discuss possible solutions.

Huntington's disease (HD) accounts for around 90% of cases of chorea of genetic etiology, but a number of other distinct genetic disorders can present with a clinical picture indistinguishable from HD. In this article, Schneider et al. review the clinical features and genetic bases of these HD-like syndromes, discussing the clinical clues that should prompt further investigations in patients presenting with these conditions.

Activation of the ligand-inducible transcription factor peroxisome proliferator-activated receptor-γ (PPARγ) has been shown to suppress inflammation in peripheral macrophages and in models of human autoimmune disease. As Heneka et al. discuss in this Review, PPARγ is now being investigated as a potential therapeutic target in CNS disorders with an inflammatory component, including Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis and multiple sclerosis.

In children, nonconvulsive status epilepticus (NCSE) is associated with various conditions, including acute neurological injuries and specific childhood epilepsy syndromes, and it can also be observed in individuals with learning difficulties. In this article, Korff and Nordli review various aspects of NCSE, including clinical and electroencephalographic features, prognosis, and treatment recommendations. They also highlight the need for further research into NCSE in pediatric populations.