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Arch Neurol. 1998;55:775.

Russian Dystonia
Illarioshkin et al (SEE ARTICLE) describe 6 families with a mutation in the GTP cyclohydrolase 1 (GCH-1) gene in a set of Russian families with dopa-responsive dystonia (DRD). Three new heterozygote point mutations in exons 1, 2, and 4 of the GCH-1 gene were identified in 3 families with autosomal dominant inheritance. The molecular genetics of DRD is continuing to unfold.

Tumor Necrosis Factor Predicts Active Multiple Sclerosis
The production of tumor necrosis factor , and not interferon , predicts exacerbation in patients with stable multiple sclerosis, as reported by van Oosten and colleagues (SEE ARTICLE) . This reported association is by no means a conclusive one and does not explain all the fluctuations of disease activity, but it is a step forward.

Being Aware of Huntington Disease
Snowden et al (SEE ARTICLE) provide an interesting analysis of patients with Huntington disease who have an impaired subjective experience of chorea. Their study provides good evidence that the denial of the symptoms in this disease has a physiological basis and is not a secondary consequence of cognitive impairment or other features. It is an insightful and useful observation.

Apolipoprotein E e4 Enhances AD
Martinez and colleagues (SEE ARTICLE) have found a relationship among the risk for Alzheimer disease (AD), familial aggregation of AD, and the apolipoprotein E e4 allele in first-degree relatives of patients with AD. Although the lifetime risk among first-degree relatives increases significantly with the number of e4 alleles present in the proband, clearly other factors enhancing the risk for AD remain to be found.

Anticardiolipin Antibodies and Intracranial Pressure
Leker and Steiner (SEE ARTICLE) describe patients with intracranial hypertension and show that there is a statistically significant association for the presence of anticardiolipin antibodies in these patients. Examining patients for this class of antibody is important in establishing causality for benign increased intracranial pressure.

Diagnosing Essential Tremor
Essential tremor falls under several terms and descriptions. Louis et al (SEE ARTICLE) provide criteria that have practical clinical value.

Cognitive Functions in Juvenile and Late Onset HD
Evaluating the severity of cognitive loss in Huntington disease (HD) depending on the onset of disease at a juvenile or adult age was measured by Gomez-Tortosa et al (SEE ARTICLE) . It is interesting and somewhat paradoxical that cognitive status is slightly better preserved in patients with juvenile-onset HD compared with adult onset. There were more subtly qualitative differences in the 2 groups as well, which adds to the complexity in our understanding of CAG number and selective neurologic functions.

Carbon Monoxide and Memory
Acute low-level exposure to carbon monoxide and cognitive functions were measured by Amitai and colleagues (SEE ARTICLE) . Low-level exposure to carbon monoxide results in impairment of higher cognitive functions. Harmful effects of low-level carbon monoxide can add up over time.

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