Review Articles in 2011

Brain tumors are among the most common childhood cancers and are the leading cause of cancer-related childhood mortality. In this Review, Pollack and Jakacki describe the clinical presentation, diagnosis and management of childhood brain tumors, with a particular focus on how treatment of the most common subtypes of childhood brain tumors has evolved over time, and the future approaches that could lead to improved outcome for these pediatric patients.

Monitoring for secondary brain injury is central to neurocritical care. Hemphill and colleagues provide an overview of multimodal monitoring in neurocritical care and discuss how bioinformatics tools for data acquisition, analysis and storage could be used in the clinical management of patients with acute brain injuries.

In this Review, Hoppe and Elger discuss the etiological factors underlying depression as a comorbidity of epilepsy, comprising psychological factors such as learned helplessness and the 'burden of epilepsy', and neurobiological factors directly related to the seizures. The authors highlight the need for more clinical studies of antidepressants and psychotherapy, and emphasize the importance of an integrated approach to treatment that addresses both the psychological and the neurobiological factors.

Research in neurodegenerative disease is generating vast neuroimaging data sets, necessitating the development of powerful new e-infrastructures for data collection, storage, access and analysis. In this article, Frisoni et al. provide an overview of the currently available e-infrastructures—LONI, neuGRID and CBRAIN—and consider how computational neuroscience in neurodegenerative disease might evolve in the future.

Malignant brain tumors such as glioma have a poor prognosis. Large-scale efforts to compile and store genome-wide data on gliomas have recently been initiated. Riddick and Fine describe these key initiatives and how these genomic approaches aim to improve tumor classification and elucidate the underlying biology of glioma. Use of these genomic technologies in the development of new diagnostic and therapeutic strategies in personalized medicine will also be discussed.

In this article, Carsten Bönnemann describes the clinical and diagnostic features of the entire spectrum of collagen VI-related myopathies, ranging from severe Ullrich congenital muscular dystrophy to mild Bethlem myopathy. He also considers the genetic and molecular mechanisms that underlie this group of diseases, and discusses current and future approaches to treatment.

Thrombolysis was first shown to be effective for treating acute ischemic stroke around 15 years ago, but therapeutic uptake worldwide has been modest to date. Here, Donnan et al. outline the various strategies that are being explored to optimize the use of thrombolysis, including enhancement of thrombolytic efficacy, extension of the therapeutic time window, reduction in the risk of intracerebral hemorrhage, and improvements in prehospital and in-hospital patient management.

The autoimmune myopathies, the best known of which are polymyositis and dermatomyositis, share a number of clinical features, including progressive proximal muscle weakness, and evidence of inflammation on muscle biopsy. Here, Mammen reviews the typical clinical presentations of the autoimmune myopathies, and discusses the relationships between distinct clinical phenotypes and myositis-specific autoantibodies.

Lack of the tumor suppressor protein merlin results in the development of benign nervous system tumors—most commonly schwannomas, which characterize neurofibromatosis type 2, but also meningiomas and ependymomas. Such tumors engender considerable morbidity owing to the high tumor burden and the lack of effectiveness of current treatment options. Here, Ammoun and Hanemann discuss emerging preclinical and clinical data in support of targeting four key families of receptor tyrosine kinases, as well as their downstream signaling pathways, for the treatment of schwannomas and related tumors.

Ischemic stroke is a major public health concern, and research to determine the underlying genetics of this disease is ongoing. Meschia and colleagues describe the single-gene disorders associated with stroke and progress in understanding the complex genetics of this condition, as well as discussing the relevance of pharmacogenomics and endophenotypes to stroke risk and treatment.

Neuralgic amyotrophy is a distinct, painful focal neuropathy with a broad spectrum of clinical phenotypes and a complex pathophysiology. The extent of disability experienced varies from patient to patient, but many individuals are left with residual disabilities affecting their everyday lives. In this Review, Nens van Alfen provides an overview of the current clinical and pathophysiological concepts and research topics in neuralgic amyotrophy.

Cognitive impairment is commonly observed in patients with multiple sclerosis (MS), often with a heterogeneous neuropsychological presentation among patients. Benedict and Zivadinov describe the clinical features and neuropsychological tests for cognitive dysfunction in MS with a particular focus on the risk factors for this disorder. Protective factors and potential therapy for cognitive impairment in MS are also discussed.

Given the aging population, understanding the link between depression and dementia could be crucial to the potential treatment and prevention of dementia in the elderly. In this Review, Byers and Yaffe describe the evidence supporting a role for both earlier-life and late-life depression in dementia. A particular focus is given to the putative biological mechanisms that underlie this association and the implications of depression treatment and prevention for the development of dementia.

Corticobasal degeneration is a rare, progressive neurodegenerative disorder with highly variable clinical presentation, and is difficult to identify in living patients. The classic clinical presentation of this disease—corticobasal syndrome—often occurs in conjunction with other neurological diseases. Kouri et al. discuss the use of imaging and biomarkers to improve diagnosis, and examine the genomic data that should provide insights into novel pathways involved in the pathogenesis of tauopathy.

Numerous neurological consequences of alcoholism have been identified, including hepatic encephalopathy, Wernicke encephalopathy, Korsakoff syndrome, Marchaifava–Bignami disease and central pontine myelinosis. Here, Zahr et al. focus on Wernicke encephalopathy and Korsakoff syndrome, exploring their position within a proposed spectrum of neurological disruption that begins with alcohol-related brain damage.

Evidence collected over the past decade has challenged the idea that essential tremor is a monosymptomatic tremorogenic disorder. In this Review, Bermejo-Pareja describes the nonmotor symptoms that are most commonly associated with essential tremor, focusing particularly on cognitive deficits.

A number of established therapies are available for multiple sclerosis (MS), but the advent of numerous novel therapies has meant that treatment decisions for MS are becoming increasingly complex. In this Review, Kieseier and Stüve discuss the challenges that both neurologists and patients face in terms of treatment decision-making, and highlight the risks, benefits and treatment paradigms associated with MS therapy.

Stroke is an important cause of long-term disability in children. In this Review, Jordan and Hillis examine the etiology of and risk factors associated with pediatric stroke, before exploring the notable diagnostic challenges associated with this condition. The authors also examine the management of stroke in children, highlighting the need for further clinical trials of potential stroke therapies in this patient group.

Psychogenic nonepileptic seizures (PNES) and epileptic seizures share many similar features, as a result PNES are often misdiagnosed and mistreated as epilepsy. In this article, Devinsky et al. provide an overview of the latest findings relating to risk factors and pathogenesis of PNES, and highlight the clinical features that can help clinicians differentiate between this condition and epileptic seizures.

Many patients with multiple sclerosis relapse or experience continued disease progression despite disease-modifying therapies, such as interferon β. This Review explores responses to interferon β treatment in patients with multiple sclerosis, discusses early identification of nonresponders, and describes a practical approach for incorporating clinical data, biological markers and MRI measures of disease activity into the management of these patients.