Adrenoleukodystrophy (ALD) Revisited
Moser and colleagues, in a focused and important editorial critique (SEE ARTICLE) , review their extensive experience with ALD and review the work ofTakano et al (SEE ARTICLE) describing a mutational analysis of 29 unrelated Japanese patients with X-linked ALD. The frequency and types of mutations in the Japanese population are similar to other national groups. The detailed data provided by Takano et al and those in other ethnic groups provide the opportunity to determine whether the nature of the mutations determines the phenotype. The role of very long–chain fatty acids in the pathogenesis of X-linked ALD is discussed. The pathogenesis of the brain inflammatory response is also reviewed and placed into clinical context. Moser et al and Takano et al provide a synopsis of present molecular-clinical relationships.
Treating Multiple Sclerosis (MS)
Tselis and Lisak (SEE ARTICLE) provide a well-focused discussion of current therapies for MS, emphasizing the interferons and glatiramer acetate (formerly copolymer-1). Their comments provide useful pointers on the effectiveness of new agents on the market.
Biomarkers of Alzheimer Disease (AD)
Growdon (SEE ARTICLE) provides a thorough, consensus view of currently available diagnostic tests for AD. Accurate and dependable biomarkers would clearly provide increased sensitivity and specificity in diagnosing AD. As Growdon points out, preliminary biomarkers are of limited diagnostic value. The clinician needs to be aware of the value and limitations of current biomarkers in diagnosing AD and predicting the natural history of disease in specific patients.
Gene Therapy in Neurologic Disease
Suhr and Gage (SEE ARTICLE) are pioneers and leaders in the use of gene therapy with viral vectors. They point out that we may be on the verge of a renaissance in the use of viral vectors to treat specific targeted neurologic disease. The current status in our success in this regard is critically and positively reviewed.
Mild Cognitive Impairment (MCI)
Petersen and colleagues (SEE ARTICLE) contrast MCI and mild AD. Their careful and comprehensive study points out that patients who meet the criteria for MCI can be differentiated from normal control subjects and those with mild AD. It is important to identify patients with MCI as they constitute a clinical entity that can be characterized for treatment interventions.
Myelin Antibody in Multiple Sclerosis
Karni et al (SEE ARTICLE) describe finding in patients with MS an increase in antimyelin antibody that is not specific for MS. They raise the important point that myelin antibody may not be pathogenic in MS. On the contrary, it may have a protective role against further immune-mediated damage after myelin breakdown.
Cognitive Function With Glatiramer Therapy
Weinstein and colleagues (SEE ARTICLE) have studied patients with relapsing-remitting MS for their cognitive function after glatiramer acetate therapy. They could demonstrate no effect of this agent on cognitive function as compared with placebo-controlled patients. Their findings provide the basis for future studies in evaluating this vital area of neurologic function.
Magnetic Resonance (MR) Spectroscopy in Myotonic Dystrophy (MD)
Akiguchi and colleagues (SEE ARTICLE) describe MR spectroscopy in patients with myotonic dystrophy. The ratio of N-acetyl aspartic acid to creatine (NAA/Cr) and phosphocreatine in patients with MD was significantly lower than control subjects. Also the ratio of NAA to choline-containing compounds in patients with MD was significantly lower than controls. Thus, MR spectroscopy provides important new data on cerebral biochemical alterations in patients with MD.
Progressive Parkinson Disease (PD)
Louis and colleagues (SEE ARTICLE) describe the progression of extrapyramidal signs in patients with PD. The type and degree of progression is measured and discussed in this clinically useful review.
Gray and White Matter Atrophy in Aging and AD
Salat et al (SEE ARTICLE) measure the decline of white and gray matter volumes in normal aging and in AD. Their findings suggest that AD affects gray and white matter loss differently than accelerated and normal aging.
T1 Spin-Echo MRI in MS
The degree of inflammatory activity present in patients with MS only partially predicts an increase in T1 lesion load at long-term follow-up; so conclude van Walderveen et al (SEE ARTICLE) . Initial T1 lesion load provides some insight into the underlying biological activity in patients with MS.
