Volume 7 Issue 7, July 2011

John W. Griffin, internationally renowned expert on peripheral nerve disorders, founding Director of the Johns Hopkins Brain Science Institute and launch Editor-in-Chief of Nature Reviews Neurology, died on 16^th April 2011. He will be remembered for his leadership in academic medicine, strong advocacy for young neurologists, and many original research contributions.

The clinical presentation of Guillain–Barré syndrome (GBS) is heterogeneous and, despite effective treatments, some patients die or sustain severe disabilities. An improved clinical prognostic score for GBS facilitates early identification of patients expected to have poor outcomes. These individuals might benefit from modified treatment or participation in therapeutic trials.

Accumulating evidence suggests that prestroke statin therapy could improve functional recovery following acute ischemic stroke. A recent meta-analysis of previous findings and a new study support this suggestion. These findings should, however, be interpreted with caution owing to possible publication bias favoring small studies with positive results.

Human leukocyte antigen (HLA) screening could identify patients at risk of adverse reactions to carbamazepine, one of the most commonly used anticonvulsants. A European genome-wide association study revealed strong correlations between HLA-A^*3101 and carbamazepine hypersensitivity. In Taiwan, HLA-B^*1502 screening led to significant reductions in the incidence of carbamazepine-induced adverse events.

Increased blood pressure is associated with poor outcomes in acute stroke, but studies assessing the use of antihypertensive agents have been inconclusive. The recent SCAST trial has found that the angiotensin receptor blocker candesartan does not benefit patients with acute stroke and elevated blood pressure, and might even be harmful.

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.

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.

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.

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 considerable need for research on Alzheimer disease (AD) is complicated by the impaired capacity of patients with AD to provide informed consent to participate in research and the lack of well-defined policy guidelines on this issue. Scott Kim highlights the importance of distinguishing between capacity and authenticity when assessing the ability of a patient with AD to provide informed consent, and discusses how the role of surrogate decision-makers could be enhanced in this context.