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This Month in Archives of Neurology
Arch Neurol. 2008;65(1):13-14.
Aging Face of the Fragile X Gene
Amiri and colleagues (SEE ARTICLE) 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.
Neuroprotection in Multiple Sclerosis: Modeling Axonal Degeneration in the Anterior Visual System
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 al (SEE ARTICLE) in this review underscore the potential utility of the anterior visual system for the purpose of modeling neuroprotection in response to novel therapies.
Undernutrition and Outcome in Acute Ischemic Stroke
Acute ischemic stroke patients with baseline malnutrition are undernourished during hospitalization. Yoo and colleagues (SEE ARTICLE) show that nutritional support, particularly in patients with baseline undernutrition, improves clinical outcome. Editorial perspective for this critically important and underappreciated subject is provided by Neeraj Badjatia, MD, and Mitchell S. V. Elkind, MD, MS. (SEE ARTICLE)
Genomewide Association of Alzheimer Disease
A genomewide association analysis of Alzheimer disease (AD) presented by Li et al (SEE ARTICLE) 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.
Increased Risk for Alzheimer Disease in Type 2 Diabetes With APOE  4
Irie and colleagues (SEE ARTICLE) found that having both diabetes and the APOE  4 allele increases the risk of dementia, especially for Alzheimer disease.
Noninvasive Ventilation in Myasthenic Crisis
Bilevel positive airway pressure (BiPAP) noninvasive ventilation is an effective treatment in patients in myasthenic crisis as reported by Seneviratne and colleagues (SEE ARTICLE). 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.
Validating CNS Penetration-Effectiveness Rank for Antiretroviral Agents Into Brain
Letendre and colleagues (SEE ARTICLE) 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.
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Figure. 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.
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Mesial Frontal Epilepsy and Body Turning
Leung et al (SEE ARTICLE) 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.
Neuromyelitis Optica and Non–Organ-Specific Autoimmunity
Pittock and colleagues (SEE ARTICLE) 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.
Cognitive Functions in Neuromyelitis Optica
This study by Blanc et al (SEE ARTICLE) 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.
Magnetic Resonance Imaging White Matter Hyperintensities and Brain Volume in Mild Cognitive Impairment and Dementia
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 al (SEE ARTICLE).
Reduced Purkinje Cell Number and Tremor
Axelrad et al (SEE ARTICLE) 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.
Education and Reported Age at Onset for Alzheimer Disease
Roe and colleagues (SEE ARTICLE) 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.
Brain Volume Decline in Aging
Fotenos et al (SEE ARTICLE) found that privileged, nondemented older adults harbor more preclinical brain atrophy, consistent with their having greater reserve against the expression of Alzheimer disease.
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