Arch Neurol Chicago
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Arch Neurol Chicago · Aug 2007
Multicenter StudyPhenotypic study in 40 patients with dysferlin gene mutations: high frequency of atypical phenotypes.
To describe the phenotypic spectrum of dysferlin (DYSF) gene mutations (which cause dysferlinopathies, autosomal recessive muscular dystrophies) in patients with a dysferlin protein deficiency. ⋯ In addition to typical Miyoshi myopathy and limb-girdle muscular dystrophy type 2B, dysferlinopathies are a clinically heterogeneous group of disorders ranging from asymptomatism to severe functional disability.
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Arch Neurol Chicago · Jul 2007
Randomized Controlled Trial Multicenter Study Clinical TrialRandomized, double-blind, placebo-controlled trial on symptomatic effects of coenzyme Q(10) in Parkinson disease.
Major hallmarks in the pathophysiology of Parkinson disease are cellular energy depletion and oxidative stress leading to cellular dysfunction and death. Coenzyme Q(10) (CoQ(10)) is an electron acceptor bridging mitochondrial complexes I and II/III and a potent antioxidant that consistently partially recovers the function of dopaminergic neurons. ⋯ Nanoparticular CoQ(10) at a dosage of 300 mg/d is safe and well tolerated and leads to plasma levels similar to 1200 mg/d of standard formulations. Add-on CoQ(10) does not display symptomatic effects in midstage Parkinson disease.
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Arch Neurol Chicago · Sep 2005
Multicenter Study Comparative StudyPrediction of longitudinal brain atrophy in multiple sclerosis by gray matter magnetic resonance imaging T2 hypointensity.
Gray matter magnetic resonance imaging T2 hypointensity, a marker of iron deposition, is associated with clinical impairment and brain atrophy in cross-sectional studies of multiple sclerosis. Treatment with intramuscular interferon beta-1a limits brain atrophy in the second year of treatment. ⋯ Gray matter T2 hypointensity predicts the progression of brain atrophy in placebo- but not interferon beta-1a-treated patients. This predictive effect is seen as early as the first year. We hypothesize that interferon beta may exert its effect on brain atrophy in part by reducing a cascade of events that involve iron deposition as a mediator of neurotoxicity or as a disease epiphenomenon.
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Arch Neurol Chicago · Aug 2005
Multicenter StudyManagement of referred deep brain stimulation failures: a retrospective analysis from 2 movement disorders centers.
Since the Food and Drug Administration approved DBS, there has been a surge in the number of centers providing the procedure. There is currently no consensus regarding appropriate screening procedures, necessary training of individuals providing the therapy, the need for an interdisciplinary team, or guidelines for the management of complications. An increasing number of patients come to experienced DBS centers after unsatisfactory results from DBS surgery. An attempt is made herein to evaluate the reasons for DBS failure in a series of such patients and to make recommendations to improve overall DBS outcomes. ⋯ With appropriate intervention, 51% of patients who complained of "failed" DBS procedures ultimately had good outcomes. Thirty-four percent of these patients had persistently poor outcomes despite maximal intervention. This case series provides important insights into reasons for "DBS failure" and proposes strategies to manage patients with DBS more effectively.
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Arch Neurol Chicago · Jul 2004
Multicenter StudyPrediction of hospital disposition after thrombolysis for acute ischemic stroke using the National Institutes of Health Stroke Scale.
Early determination of discharge destination after acute stroke may promote earlier rehabilitation and reduce costs by shortening the duration of hospitalization. ⋯ Stroke severity as determined by the admission NIHSS score is the major independent predictor of disposition after hospitalization and treatment with rt-PA for acute stroke in a broad-based population. However, symptomatic ICH after rt-PA is a catastrophic event that may preclude discharge to home.