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This Month in Archives of Neurology
October 2009

This Month in Archives of Neurology

Author Affiliations

Copyright 2009 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.2009

Arch Neurol. 2009;66(10):1190-1191. doi:10.1001/archneurol.2009.222
Ataxia: Channeling Changes Into Novel Therapies

Shakkottai and PaulsonArticle provide a clear and direct discussion suggesting that neuronal dysfunction stemming from perturbed channel activity likely explains some motor deficits in episodic and degenerative ataxias. Importantly, they point out that understanding these physiologic changes may reveal novel therapeutic targets for symptomatic treatment of ataxia. New ideas, new strategies, and potential new therapeutic approaches are presented in this important review.

Essential Tremors

LouisArticle reviews the facts related to the present understanding of essential tremor, the most common pathological tremor in humans. He provides evidence that essential tremor is a family of diseases rather than a single entity.

Strategies for the Prevention of Dementia

Middleton and YaffeArticle emphasize that, for the foreseeable future, risk factor modification remains the cornerstone of dementia prevention including vascular risk factor control, cognitive activity, physical activity, social engagement, diet, and recognition of depression.

Interferon-Beta Therapy Enhances Cytokine Expression in Multiple Sclerosis

Cepok and colleaguesArticle demonstrate that interferon-beta strongly upregulates a set of cytokines and CCR1 in peripheral immune cells. The upregulation of these chemokines may reduce chemoattraction of immune cells into the central nervous system and add to the therapeutic effects of interferon-beta. Editorial perspective is provided by Olaf Stüve, MD, PhD, and Richard M. Ransohoff, MD.Article

Modeling Predicts That Epilepsy May Be Caused by Very Small Functional Changes in Ion Channels

Thomas et alArticle describe how statistical power calculations indicate that small changes in ion channels are effectively undetectable with current experimental practices, thus posing new challenges for the functional analysis and validation of epilepsy genes. This article provides an important paradigm shift in our thinking regarding detection of genetically basic channelopathies that cause epilepsy.

Seizure Relapse and Drug Resistance Following Long-term Seizure Remission

SchillerArticle reports that about half of patients who entered long-term seizure remissions experienced seizure relapse, and more than a quarter of patients eventually developed drug resistance. Treatment history served as a significant independent predictive risk factor for seizure relapse and development of drug resistance.

Image not available

Seizure relapse and development of drug-resistant epilepsy in patients who entered long-term (>1 year) seizure remissions. The average percentages of patients who were seizure free and drug responsive are plotted as a function of time from the onset of the antiepileptic drug–induced seizure remission. The experimental points were fitted with monoexponential functions (lines), with minimal values of 56.4% for seizure relapses and 72.6% for development of drug resistance and a half-decay time constant of 21.5 months for both curves.

Codistribution of Amyloid β Plaques and Spongiform Degeneration in Familial Creutzfeldt-Jakob Disease

Ghoshal and colleaguesArticle provide the first description of amyloid β (Aβ) pathology in familial Creutzfeldt-Jakob disease with the E200K mutation. This observation suggests that the prion protein plays a central role in Aβ formation and that Aβ pathology and prion disease likely influence each other.

Ten-Year Change in Plasma Amyloid β Levels and Late-Life Cognitive Decline

Okereke et alArticle suggest that higher plasma amyloid β (Aβ)–40 to Aβ42 ratios in late-midlife and increases in Aβ40 to Aβ42 10 years later were significantly associated with greater decline in global cognition in late life.

Longitudinal Study of the Transition From Healthy Aging to Alzheimer Disease

Johnson and colleaguesArticle describe that there is a sharp inflection point followed by accelerating decline in multiple domains of cognition, not just memory, in the preclinical period of Alzheimer disease, when there is insufficient cognitive decline to warrant a clinical diagnosis of Alzheimer disease using conventional criteria. These significant findings emphasize that research into early detection of cognitive disorders using only episodic memory tasks may not be sensitive to all early manifestations of the disease.

Sex and SORL1 Variants in Alzheimer Disease

Cellini et alArticle confirm the association of SORL1 with Alzheimer disease, showing a possible effect of sex, and suggest that this gene may be a promising susceptibility factor in late-onset Alzheimer disease.

Intronic Variants of BTBD9 Gene and Tourette Syndrome

Rivière and colleaguesArticle provide evidence that variants in BTBD9 that predispose patients to restless legs syndrome and periodic limb movements during sleep are also associated with Tourette syndrome, particularly Tourette syndrome without obsessive-compulsive disorder.

Ideomotor Apraxia and Corticobasal Syndrome

Huey et alArticle report that in corticobasal syndrome, patients' ideomotor apraxia is associated with left posterior frontal cortical and subcortical volume loss.