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Neurology publishes well-designed clinical trials—positive or negative. Noseworthy et al. (p. 1726) report the results of a large (27-center) study of linomide for relapsing-remitting MS and secondary progressive MS. Linomide is an immunomodulating agent with major benefits in experimental autoimmune diseases and in phase 2 trials in patients with MS. The trial was terminated before completion because of toxicity—myocardial infarction and pericarditis as well as pleural effusions and pancreatitis. The companion article by Wolinsky et al. (p. 1734) reports important data on MRI outcome measures that were developed with the trial. The accompanying editorial by Schwid and Trotter (p. 1716) considers the many important implications of this large trial—most importantly, the likelihood that a smaller, less careful trial might not have detected the uncommon but severe toxicity.
Interferon ß-1a: Effect on CNS injury in MS
Kita et al. (p. 1741) followed serial (monthly) MRIs in patients with relapsing-remitting MS both before and after treatment with IFNß-1a 6.0 MIU/week. The measure examined—magnetization transfer ratio (MTR)—has been proposed as a marker for irreversible CNS damage. Therefore, more rapid resolution of MTR abnormalities could indicate that tissue injury resolution was improved. The authors report that MTR abnormalities resolved more rapidly with IFNß-1a treatment.
Torsion dystonia: Genetic testing
Bressman et al. (p. 1746) screened a large population of patients and family members for dystonia type 1, recognized by the GAG deletion in the torsinA gene, and for clinical signs of dystonia, assessing the predictive value of testing at different ages. They conclude that in early-onset dystonia cases (< age 26) or later-onset cases with early-onset dystonia in family members genetic testing is war-ranted. They emphasize the importance of genetic counseling in conjunction with testing. In the accompanying editorial, Lichter and Lang (p. 1718) review the genetics of dystonia and the many questions that need to be addressed. Why does one family member have severe disease, another writer’s cramp, while another remains asymptomatic? What is the function of torsinA? What is the significance of the PET abnormalities found in patients with dystonia?
White matter lesions and atrial fibrillation
In a prospective population-based study, de Leeuw et al. (p. 1795) found the prevalence of atrial fibrillation (AF) to be 1.9% in subjects younger than 75 years and 5.5% in those older than 75 years. Subjects with AF had more periventricular white matter lesions than controls. Those with AF of longer duration had a six-fold greater risk of such lesions.
Putaminal hemorrhage with ipsilateral hemiparesis
Terakawa et al. (p. 1801) found convincing evidence for an uncrossed pyramidal tract and medial lemniscus in a patient who experienced right hemiparesis and sensory findings after an acute right putaminal hemorrhage.
Measuring quality of life in epilepsy
Quality of life (QOL) measurements are now essential for demonstrating that interventions—surgical or medical—merit approval by third party payors, and increasingly, by the Food and Drug Administration. Developing and validating QOL measures are of great importance for each specialty area in neurology. Birbeck et al. (p. 1822) compare the standard SF–36 with two epilepsy-targeted measures during an antiepileptic drug trial. The specialty-targeted device was more responsive to change.
Treatment of acute cluster headache
Bahra et al. (p. 1832) studied zolmitriptan for cluster headache in a randomized, placebo-controlled crossover study. They assessed the responses of both acute episodes as well as chronic cluster headache. Treatment with zolmitriptan 10 mg had significant benefit for acute episodes but did not improve chronic headache.
Creatine for muscular dystrophy
Last year, Neurology published an article on the benefit for muscular dystrophy (MD) with a trial of creatine. Here, Walter et al. (p. 1848) report a placebo-controlled, crossover trial of creatine (widely available as a health food) in 36 patients with MD: 12 with FSH, 10 with Becker, 8 with Duchenne, and 6 with sarcoglycan-deficient dystrophies. Slight but significant improvement in muscle strength was found. Creatine had no major side effects.
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