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This Month in Archives of Neurology
November 2008

This Month in Archives of Neurology

Arch Neurol. 2008;65(11):1415-1416. doi:10.1001/archneur.65.11.1415

Medulloblastoma 2008

Packer and VezinaArticle cite medulloblastoma as the most common malignant childhood brain tumor. Children have a high likelihood of long-term survival, as 80% or more will be alive 5 years after diagnosis and treatment, with many being cured of the disease. In their comprehensive review, the authors point out that molecular targeted therapies are being studied in patients and hold great promise.

Tissue Plasminogen Activator, Ischemic Stroke, and Potential Legal Issues

Liang and colleaguesArticle point out that the use of tissue plasminogen activator (tPA) is efficacious and can result in highly improved outcomes for most eligible patients. However, they state that these findings may implicate important potential legal issues. Informed consent concerns and, potentially, medical malpractice claims may result, particularly in the contexts of evidence-based practice, pay for performance, and the currently limited use of tPA for eligible patients.

Interferon Beta–Induced Restoration of Regulatory T-Cell Function in Multiple Sclerosis

Korporal et alArticle evaluated the effect of interferon beta (IFN-β) on the suppressive activity and homeostasis of circulating regulatory T (Treg) cells in patients with multiple sclerosis. They find that the increase in Treg-cell inhibitory capacities mediated by IFN-β can be explained by its effect on the homeostatic balance within the Treg compartment. Editorial perspective is provided by Harald H. Hofstetter, MD, Olaf Stuve, MD, PhD, and Hans-Peter Hartung, MD.Article

Treatment of Neuromyelitis Optica With Rituximab

Jacob and colleaguesArticle evaluated the use and efficacy of rituximab for neuromyelitis optica (NMO). They present clear evidence that, in NMO, treatment with rituximab appears to result in reduced attack frequency with subsequent stabilization or improvement in disability.

Relapses in patients with neuromyelitis optica before and after treatment with rituximab. The vertical lines indicate start of new treatments; horizontal line, patient.

Relapses in patients with neuromyelitis optica before and after treatment with rituximab. The vertical lines indicate start of new treatments; horizontal line, patient.

Magnetic Resonance Disease Severity Scale Predicts Progression in Multiple Sclerosis

Bakshi et alArticle have combined brain magnetic resonance imaging (MRI) lesion and atrophy measures to show that these measures can predict clinical progression in patients with multiple sclerosis and provide the basis to develop an MRI-based continuous scale as a marker of multiple sclerosis severity.

Gray Matter Magnetization Transfer Imaging: A Marker of Progression in Multiple Sclerosis

Khaleeli and colleaguesArticle provide convincing data that gray matter magnetization transfer ratio, normalized magnetization transfer ratio, meets many of the criteria for a surrogate marker of progression in early primary progressive multiple sclerosis.

Pulsed Oral Steroids for Chronic Inflammatory Demyelinating Polyneuropathy

Muley et alArticle show that pulsed oral methylprednisone is efficacious in the long-term treatment of chronic idiopathic demyelinating polyneuropathy and is relatively well tolerated.

Alzheimer and Cognitive Reserve: Education Effect Varies With Carbon 11–Labeled Pittsburgh Compound B Uptake

Roe and colleaguesArticle provide data supporting the view that cognitive reserve influences the association between Alzheimer disease pathology and cognition.

The Tau Gene Region Chromosome Inversion in Progressive Supranuclear Palsy, Corticobasal Degeneration, and Related Disorders

Webb et alArticle performed a genome-wide association scan to search for variants that confer susceptibility to 4 tauopathies and clinically related disorders. This article focuses on the results from an inverted region of chromosome 17 that contains the MAPT gene. They conclude that the association found in the tauopathies across this interval further supports the theory that 1 or more susceptibility loci in this region is affecting susceptibility specifically to progressive supranuclear palsy and corticobasal degeneration.

TDP-43 in the Cerebrospinal Fluid of Patients With Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis

Steinacker and colleaguesArticle find that patients with frontotemporal lobar degeneration (FTLD) and amyotropic lateral sclerosis (ALS) had higher transactivation response DNA-binding protein 43 (TDP-43) levels compared with control subjects, but with an overlap of values. Future determination of TDP-43 in the cerebrospinal fluid might aid in characterizing subgroups of patients across the ALS/FTLD disease spectrum.

Seven-Tesla Proton Magnetic Resonance Imaging in X-Linked Adrenoleukodystrophy

Ratai et alArticle report that 7-Tesla proton magnetic resonance spectroscopic imaging shows differences in the neurochemistry of patients with adult cerebral adrenoleukodystrophy but is unable to distinguish adrenomyeloneuropathy from female heterozygotes. Myo-inositol/creatine correlates with the severity of symptoms and may be a meaningful biomarker in adult X-linked adrenoleukodystrophy.

A Novel Duplication of 5q23.2 in Autosomal Dominant Leukodystrophy

Meijer and colleaguesArticle identified a novel duplication on chromosomal band 5q23.2 in a French Canadian family with autosomal dominant leukodystrophy, supporting duplication of LMNB1 as the disease-causing mutation.

Young-Onset Dementia

Kelley et alArticle find that young-onset dementia (onset prior to 45 years of age) encompasses a broad variety of etiologies, with a sizable minority having a potentially treatable disorder.

Amyloid Deposition Is Frequent and Often Is Not Associated With Significant Cognitive Impairment in the Elderly

Aizenstein et alArticle show that amyloid deposition can be identified in cognitively unimpaired elderly persons during life and the prevalence of asymptomatic amyloid deposition may be similar to that of symptomatic amyloid deposition. They find that one can remain cognitively unimpaired in the presence of significant amyloid burden.

Analyses of the National Institute on Aging Late-Onset Alzheimer Disease Family Study Implicate Additional Loci

Lee et alArticle conducted a linkage analysis and family-based and case-control association analyses as part of the National Institute on Aging Genetics Initiative for Late-Onset Alzheimer Disease (NIA-LOAD). They report that there are potentially several additional loci that may harbor genetic variants associated with LOAD. This data set provides a wealth of phenotypic and genotypic information for use as a resource in discovery and confirmatory research.