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Lovett-Racke and colleagues review our current knowledge of RNA interference, an evolutionarily conserved mechanism to degrade messenger RNA that protects host cells from RNA viruses and transposons, which can jeopardize the genome. Small interfering RNA has been used to study the function and significance of a vast number of genes in a variety of cell types. In the future, small interfering RNA will have tremendous potential as gene-specific therapeutic agents for the treatment of many central nervous system diseases.
Swift and Ausman offer their views as to what’s ahead for neurologists, neurosurgeons, and neuroscientists in the new millennium. The scientific and economic future is rapidly changing with respect to therapeutic and practice management issues. Their crystal ball insights into future events facing our specialty are insightful and even clairvoyant!
Snider and colleagues describe a novel presenilin 1 mutation causal of very early-onset autosomal dominant Alzheimer disease with associated Lewy bodies. This kindred has the earliest reported onset of pathologically confirmed familial Alzheimer disease and dementia with Lewy bodies. Editorial perspective is provided by Roger N. Rosenberg, MD.
Stover et al describe an open-label pilot study to evaluate the safety and efficacy of implantation of human retinal pigment epithelial cells attached to gelatin microcarriers into the putamen of patients with advanced Parkinson disease. This highly innovative therapeutic approach appears to be safe and well tolerated. These implants improved motor symptoms and justify further therapeutic trials.
Massacesi and colleagues evaluated azathioprine efficacy on suppressing new brain lesions in multiple sclerosis as judged by magnetic resonance imaging. This study indicates for the first time that azathioprine is effective in reducing new brain inflammatory lesions evaluated during administration of well-tolerated lymphocyte-suppressing doses.
Morris et al examined the effect of dietary fish on age-related cognitive decline. Of note, they report that cognitive scores declined on average at a rate of 0.04 standardized units per year, and fish intake was associated with a significantly slower rate of cognitive decline in mixed models adjusted for age, sex, race, education, cognitive activity, physical activity, alcohol consumption, and total energy intake.
Gilmore and colleagues assessed the relative contributions of white matter and gray matter volume loss to spinal cord atrophy in multiple sclerosis. They find that the white matter area was significantly reduced in multiple sclerosis cases in the upper but not the lower cord levels. Thus, spinal cord atrophy in multiple sclerosis is due to white matter, rather than gray matter, volume loss.
Bhidayasiri et al describe and further delineate the clinical and magnetic resonance imaging findings in patients with late-onset Friedreich ataxia and review the pertinent literature. Gait and limb ataxia and also dysarthria, loss of vibratory sense, and abnormal eye movements were the common clinical features. Patients with late-onset Friedreich ataxia also expressed lower limb spasticity and retained reflexes. In contrast to previous reports, patients with late-onset Friedreich ataxia also showed cerebellar atrophy on magnetic resonance imaging.
Burns and colleagues assessed the relation of age-related white matter lesions with cognition in nondemented aging and early-onset Alzheimer disease. They report that age-related white matter lesions are prevalent in nondemented aging and early-onset Alzheimer disease and their presence influences the cognitive impairment in the earliest stages of clinical Alzheimer disease. Further, persons with early-onset Alzheimer disease may be more vulnerable to the clinically relevant cognitive influence of white matter lesions than nondemented individuals with similar white matter lesion burden.
Fleisher et al determined whether functional magnetic resonance imaging can reliably distinguish risk groups for Alzheimer disease among cognitively normal middle-aged adults. They find that when compared with low-risk persons, the high-risk group for developing Alzheimer disease showed many areas of differential blood oxygen level–dependent response in regions commonly associated with Alzheimer disease pathologic abnormalities. These results demonstrate that functional magnetic resonance imaging is a viable technique to identify persons at risk for Alzheimer disease.
Boito and colleagues studied patients with limb-girdle muscular dystrophy type 2I to derive genotype/phenotype correlations. Limb-girdle muscular dystrophy type 2I is due to mutations in the fukutin-related protein gene (FKRP). Their data indicate that FKRP gene mutations are a relatively frequent cause of limb-girdle muscular dystrophy. They report that the degree of cardiac and respiratory insufficiency did not correlate with muscle severity. Two asymptomatic FKRP gene mutation cases suggest that modulating factors may ameliorate clinical phenotype.
Greenwood et al report that children with neurofibromatosis do not have the normal relationship between gray matter volume and IQ as do control subjects. They further find that larger gray matter volume predominately in the posterior brain regions and white matter volume predominately in the frontal regions of the brain contribute to the larger brain volume seen in children with neurofibromatosis.
This Month in The Archives of Neurology. Arch Neurol. 2005;62(12):1803–1804. doi:10.1001/archneur.62.12.1803
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