Lipoprotein Receptor–Related Protein and Alzheimer Disease
Hyman and colleaguesArticle provide their views of the lipoprotein receptor–related protein and the pathogenesis of Alzheimer disease. This article provides a focal point of integration that interrelates potential alterations in processing of the amyloid precursor protein and τ phosphorylation. Their hypothesis is imaginative, interesting, and important.
Neurotrophins and Neurologic Disease
Kernie and ParadaArticle review neurotrophins, including neurotrophin receptors, physiology, signaling, and relevance to neurologic disease. Neurotrophin therapy for neurologic disease is rapidly approaching clinical application, and the rationale for this approach is presented by experts in the field in a user-friendly manner.
National Institutes of Health Consensus Conference on Tuberous Sclerosis
Hyman and WhittemoreArticle summarize the 1998 consensus conference on tuberous sclerosis sponsored by the National Institutes of Health. Diagnostic criteria, genetic update, screening, and areas of future research are discussed.
Hereditary Motor and Sensory Neuropathy Type 2C
Nagamatsu and colleaguesArticle provide important linkage data in describing patients with hereditary motor sensory neuropathy type 2 phenotype as distinct from other phenotypic and genotypic forms of this syndrome. This is an important advance and again supports the rule that a genotype classification of genetic neurological disease is required.
Predicting Conversion to Alzheimer Disease
Daly and colleaguesArticle provide a clinical assessment that can be used to identify the subgroup of individuals within the category of questionable Alzheimer disease who have a high likelihood of converting to Alzheimer disease over time. It is a rigorous and practical approach with great clinical utility.
IgG From Patients With Alzheimer Disease Injures Cholinergic Neurons
Engelhardt and colleaguesArticle provide rigorous data that IgG from patients with Alzheimer disease can target a stereotaxically immune/inflammatory injury to cholinergic neurons in rat basal forebrain in vivo. Their study advances our insights into immunological mechanisms involved in the Alzheimer disease process.
Prediction of Spinal Metastases
Kienstra and colleaguesArticle describe a prospective series of 170 consecutive patients with cancer with recently developed back pain and the occurrence of possible spinal metastatic disease. They conclude that magnetic resonance imaging of the whole spine is a necessary step in the evaluation and diagnosis of spinal epidural metastases. Lessons learned in this clinical study have a clear and direct value.
Presenilin 1 Polymorphism and Alzheimer Disease
Romas and colleaguesArticle find no association between the presenilin 1 intron-8 polymorphism and sporadic late-onset Alzheimer disease. This study clarifies and negates a potential risk factor for sporadic Alzheimer disease.
Electroencephalography, Magnetic Resonance Imaging, and Neuropsychological Indicators of Seizure Lateralization
Moser and colleaguesArticle find that electroencephalography and magnetic resonance imaging were of high value for lateralization, while neuropsychological data were of limited utility in determining lateralization of temporal lobe epilepsy. The statistics provided are of considerable value in designing clinical studies for determining temporal lobe epilepsy lateralization, especially in planning temporal lobectomy.
Increasing Frequency of Dementia of Unknown Etiology With Age
Crystal and colleaguesArticle find that a significant percentage of patients with dementia aged 80 years or older do not meet the pathological criteria for Alzheimer disease or diffuse Lewy body disease. They find that hippocampal sclerosis and leukoencephalopathy are common in these patients but rare in clinically nondemented subjects in the same age group. These findings are of considerable clinical value and add a new dimension in evaluating patients in this age group.
Ethnic Differences in Essential Tremor
Louis et alArticle describe ethnic differences in the expression of essential tremor, suggesting that essential tremor is not a homogeneous disorder. Patients with essential tremor who were white, black, and Hispanic were studied, and phenotypic variability was found in these different groups. The differences found are important and need to be further analyzed and explained with regard to varying environmental factors and genotypes.
Pramipexole-Induced Peripheral Edema
Tan and OndoArticle report the clinical features and investigate the predisposing factors and eventual outcomes in patients who developed peripheral edema following treatment with pramipexole for Parkinson disease. Clearly, edema should be included among the potential adverse effects associated with this drug.
Phenotypic Manifestations of Machado-Joseph Disease
Landau and colleaguesArticle establish the diagnosis of Machado-Joseph disease (SCA3 genotype) from autopsy tissue from 1 patient and blood specimens from 6 others in a 7-generation family of German origin, first reported in 1951. It is of interest to apply current molecular genetic techniques and immunocytochemical studies establishing Machado-Joseph disease in a family 50 years after their original description.