Nygaard and StrittmatterArticle describe their extraordinary observation that cellular prion protein (PrPc) is a high-affinity receptor for β-amyloid oligomers, mediating their toxicity on synaptic plasticity. They discuss the relationship between Alzheimer disease and PrPc and the potential clinical implications. Importantly, based on this new finding, PrPc may provide a novel target for therapeutic intervention in Alzheimer disease.
Significant glottic narrowing due to vocal cord dysfunction in patients with amyotrophic lateral sclerosis is described by van der Graaff and colleaguesArticle. Both nuclear and supranuclear mechanisms may play a role. They point out that vocal cord dysfunction can occur at any stage of the disease, may account for sudden death in amyotrophic lateral sclerosis, and requires vigilant airway management.
Suzuki et alArticle report that heart and skeletal muscles are autoimmune targets in patients with myasthenia gravis. This autoimmunity has a broad clinical spectrum with antistriational autoantibodies. The involvement of the heart in myasthenia gravis needs to be defined early and treated with aggressive immunotherapy, which has had a good clinical outcome. Editorial perspective is provided by Johan A. Aarli, MD.Article
Boyle and colleaguesArticle show that an increase in muscle strength is associated with a significant decrease in the risk of Alzheimer disease, as found in community-based older persons without dementia at baseline. Further, muscle strength was associated with a decreased risk of mild cognitive impairment. These observations support the view that exercise has a beneficial effect of reducing the risk of developing significant cognitive loss and dementia.
Moraal et alArticle find that at least 9 T2 lesions and 3 periventricular lesions show strong prognostic value for conversion to clinically definite multiple sclerosis, though all patients in the study received interferon beta-1b for a least 1 year. These findings are important in providing an accurate assessment and grading of patient prognosis and response to therapy.
Lo and colleaguesArticle describe specific motor and nonmotor features in early Parkinson disease that predict increased mortality risk, particularly postural instability gait difficulty, cognitive impairment, and hallucinations. Clearly, these predictors will be useful in clinical practice and when designing clinical trials.
Kaplan-Meier plot of time to death for clinical subtype. PIGD, postural instability gait difficulty.
Kaplan-Meier plot of time to death for cognitive impairment.
Hu et alArticle provide clarity and focus in describing the distinct patterns of survival profiles in patients with frontotemporal dementia and motor neuron disease. Overall survival may depend on the relative timing of emergence of secondary symptoms.
Burkholder and colleaguesArticle show, using ocular coherence tomography, that reduced macular volumes were associated with peripapillary retinal nerve fiber layer thinning (axon loss). This diagnostic method offers a means to quantitate the degree of neuronal loss for grading disease activity and responsiveness to therapy.
Outteryck et alArticle indicate that optical coherence tomography does not show retinal axonal loss at the earliest clinical stage of multiple sclerosis and does not predict conversion to multiple sclerosis at 6 months.
Hayden and colleaguesArticle report that much of the association between family history of Alzheimer disease, apolipoprotein E ε4, and cognitive decline is attributed to undetected incipient or latent disease.
Reitz et alArticle find that in early Alzheimer disease, or in nondemented persons with impaired memory ability, functional and metabolic hippocampal hypofunction contribute to memory impairment.
He and colleaguesArticle report that amnestic forms of mild cognitive impairment appear to have demographic, genetic, and magnetic resonance imaging findings suggestive of Alzheimer disease pathology, where as the nonamnestic forms have findings suggestive of vascular disease. These distinctions have considerable value in appreciating the neurobiology of disease for these different syndromes and potential therapies.
Willey et alArticle find that baseline lipid panel components were not associated with an increased stroke risk in the cohort. Treatment with cholesterol-lowering medications and changes in low-density lipoprotein cholesterol over time may have attenuated risks in this population, and lipid measurements at several points over time are a better marker of stroke risk.
This Month in Archives of Neurology. Arch Neurol. 2009;66(11):1320-1321. doi:10.1001/archneurol.2009.251