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Blood Protein Signature for Early Alzheimer Disease
Britschgi and Wyss-CorayArticle describe a blood-based panel of secreted signaling proteins that distinguishes between blinded samples from patients with Alzheimer disease and those of control subjects with high accuracy. A clinically useful and reliable biomarker blood test is now able to be implemented on a broad basis to diagnose the disease in its earliest phase.
Extranigral Parkinson Disease
Lim and colleaguesArticle address selected clinical, anatomical, pathological, and biochemical correlates of the early premotor symptoms of Parkinson disease, later nonmotor fluctuations, and advanced dopa-unresponsive motor and nonmotor features. The recognition of early features that predate classic motor symptoms will be important as effective neuroprotective therapy becomes available.
Contribution of White Matter Lesions to Gray Matter Atrophy in Multiple Sclerosis
Sepulcre et alArticle in an elegant study, show that focal white matter damage is associated with upstream gray matter atrophy, suggesting that retrograde damage of the pericaria from axonal injury in multiple sclerosis plaques is a significant factor in the genesis of gray matter atrophy.Article
The Continuum of Multisystem TDP-43 Proteinopathies
Geser and colleaguesArticle show in a comprehensive study that amyotrophic lateral sclerosis, frontotemporal lobar degeneration with motor neuron disease, and frontotemporal lobar degeneration with ubiquitinated inclusions are different manifestations of a multiple-system transactivation response DNA-binding protein 43 (TDP-43) proteinopathy linked to similar mechanisms of neurodegeneration.
Measuring β-Amyloid Protein Oligomers in Human Plasma and the Brains of Patients With Alzheimer Disease
Xia et alArticle report that an oligomeric specific enzyme-linked immunosorbent assay shows a tight link between oligomeric assemblies of the β-amyloid protein (oAβ) and Aβ42 monomer levels in the plasma and brain. This new assay provides the opportunity to study levels of oAβ in plasma in patients with Alzheimer disease to diagnose the presence and amount of an oligomeric form of Aβ that can potentially correlate with cognitive loss.
Rapid Progressive Neurodegenerative Dementias
Josephs and colleaguesArticle report that in addition to Creutzfeldt-Jakob disease (CJD), other causes of a rapidly progressive neurodegenerative dementia also include frontotemporal lobar degeneration with motor neuron disease, diffuse Lewy body disease, tauopathies, and Alzheimer disease. If the illness duration is more than 12 months, a non-CJD neurodegenerative disease may be a more likely diagnosis than CJD.
Creutzfeldt-Jakob Disease Variants
Appleby et alArticle report in a detailed and interesting study that classic Creutzfeldt-Jakob disease and the Heidenhain, Oppenheimer-Brownell, cognitive, and affective sporadic variants of Creutzfeldt-Jakob disease differ by age at disease onset, progression, and diagnostic test results.
Survival analysis of sporadic Creutzfeldt-Jakob disease (sCJD) variants (log-rank test, χ25 = 25.3, P < .001). CJD indicates Creutzfeldt-Jakob disease.
Mediterranean Diet and Mild Cognitive Impairment
Scarmeas et alArticle find that adherence to the Mediterranean diet is associated with a trend for reduced risk for developing mild cognitive impairment and with a reduced risk for mild cognitive impairment converting to Alzheimer disease.
African Ancestry at HLA Modifies Multiple Sclerosis Phenotypes
Cree and colleaguesArticle show that the role of HLA in multiple sclerosis is not limited to disease susceptibility, but that genes embedded in this locus also influence clinical outcomes.
Benign Multiple Sclerosis Is Characterized by a Lower Rate of Brain Atrophy as Compared With Early Multiple Sclerosis
Gauthier et alArticle report that serial magnetic resonance imaging showed a low 2-year rate of brain atrophy in clinically benign multiple sclerosis, suggesting a less prominent degenerative component in its pathogenesis than in patients with typical early multiple sclerosis. Identification of patients with a low rate of brain atrophy may indicate a benign course.
Sporadic and Hereditary Brachial Plexus Neuropathy
Klein et alArticle find that patients with rare sporadic brachial plexus neuropathy (S-BPN) may have the same conserved 17q25 sequence found in many American hereditary BPN kindreds. Patients with BPN with this conserved sequence do not appear to have SEPT9 mutations or alterations of its messenger RNA expression levels in lymphoblast cultures. Patients with BPN with this conserved sequence are predicted to have the most common genetic cause in the Americas by a founder effect mutation.
FMR1 Premutation Alleles in Women With Parkinsonism
Cilia and colleaguesArticle show that screening of women with the parkinsonism clinical spectrum is unlikely to be productive in the absence of additional medical or family history suggesting involvement of the FMR1 gene.
Genomewide Association Results Suggest GAB2 as an Alzheimer Disease Susceptibility Gene
Schjeide et alArticle report that GAB2 contains genetic variants that may lead to a modest change in the risk for Alzheimer disease.
This Month in Archives of Neurology. Arch Neurol. 2009;66(2):151–152. doi:10.1001/archneurol.2008.572
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