Amiri and colleaguesArticle review the 2 opposing faces of the fragile X mental retardation 1 (FMR1) gene: a neurodegenerative syndrome (fragile X–associated tremor/ataxia syndrome) in older adults, caused by excess gene activity and production of a toxic RNA, and a childhood-onset disorder (fragile X syndrome), caused by absence of gene activity.
A major objective in multiple sclerosis therapeutics is to develop strategic targeting of specific injury pathways to provide neuroprotection and potentially even restoration. Frohman et alArticle in this review underscore the potential utility of the anterior visual system for the purpose of modeling neuroprotection in response to novel therapies.
Acute ischemic stroke patients with baseline malnutrition are undernourished during hospitalization. Yoo and colleaguesArticle show that nutritional support, particularly in patients with baseline undernutrition, improves clinical outcome. Article
A genomewide association analysis of Alzheimer disease (AD) presented by Li et alArticle identified the APOE linkage disequilibrium region as the strongest genetic risk factor for AD. This could be a consequence of the co-evolution of more than 1 susceptibility allele, such as APOC1, in this region. They also provide new evidence for additional candidate genetic risk factors for AD that can be tested in further studies.
Irie and colleaguesArticle found that having both diabetes and the APOE ε4 allele increases the risk of dementia, especially for Alzheimer disease.
Bilevel positive airway pressure (BiPAP) noninvasive ventilation is an effective treatment in patients in myasthenic crisis as reported by Seneviratne and colleaguesArticle. They show that a BiPAP trial before the development of hypercapnia can prevent intubation and prolonged ventilation, thus reducing pulmonary complications and length of intensive care unit and hospital stay.
Letendre and colleaguesArticle show that poorer penetration of antiretroviral (ARV) drugs into brain appears to allow continued human immunodeficiency virus (HIV) replication in the central nervous system as indicated by higher cerebrospinal HIV viral load (Figure). It is important, they point out, that ARV treatment strategies that account for brain penetration should be considered in consensus treatment guidelines and validated in clinical studies.
Proportion of subjects with detectable cerebrospinal fluid (CSF) viral load decreases with central nervous system Penetration-Effectiveness (CPE) rank. The proportions (black circles) and 95% confidence interval (vertical bars) were calculated from observations at each CPE rank level (1 subject with a CPE rank of 0 and 2 with a CPE rank of 4 were grouped with the adjacent groups). The solid curve represents predicted proportions of CSF viral suppression from the univariate logistic regression.
Leung et alArticle show that ictal body turning along the horizontal body axis is a feature of mesial frontal epilepsy and distinguishes it from lateral frontal and orbitofrontal seizures.
Pittock and colleaguesArticle in an elegant study show that neuromyelitis optica (NMO)-IgG–seropositive NMO spectrum disorders occurring with Sjögren syndrome/systemic lupus erythematosus (SS/SLE) or non–organ-specific autoantibodies is an indication of coexisting NMO rather than a vasculopathic or other complication of SS/SLE.
This study by Blanc et alArticle confirmed recent findings of brain involvement in neuromyelitis optica (NMO). They report that cognitive performance was significantly lower in NMO and multiple sclerosis groups compared with healthy subjects in a battery of neuropsychological tests.
White matter hyperintensities are associated with the risk of progressing from normal to mild cognitive impairment. In persons whose cognitive abilities are already impaired, brain parenchymal fraction predicts the conversion to dementia as reported by Smith et alArticle.
Axelrad et alArticle demonstrated a reduction in Purkinje cell number in the brains of patients with essential tremor who do not have Lewy bodies. Their data further support the view that the cerebellum is anatomically, as well as functionally, abnormal in these essential tremor patients.
Roe and colleaguesArticle found that the reported age at onset of dementia symptoms is earlier for participants with more education. Participants with fewer years of education show greater clinical severity of Alzheimer disease at first assessment. Symptoms of Alzheimer disease are recognized later among those with less education.
Fotenos et alArticle found that privileged, nondemented older adults harbor more preclinical brain atrophy, consistent with their having greater reserve against the expression of Alzheimer disease.
This Month in Archives of Neurology. Arch Neurol. 2008;65(1):13-14. doi:10.1001/archneurol.2007.28