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Columbia University Medical Center/NY-Presbyterian experts at AAN
Date:3/18/2013

uromuscular Diseases

Authors: Michelle Blumenschine, Jacqueline Montes, Sally Dunaway, Ashwini Rao, Claudia Chiriboga, Douglas Sproule, Darryl De Vivo

Background/Significance: The 6-minute walk test (6MWT) is a primary end-point in many clinical trials involving neuromuscular disease. Weakness and fatigue are captured by the 6MWT, but it is unclear whether fatigue accompanies weakness in these conditions. Comparison across neuromuscular diseases has not been made. In this study, all patients with neuromuscular disease demonstrated weakness. Only the spinal muscular atrophy (SMA) group demonstrated fatigue, inversely related to walking ability. In contrast, weakness progressed in Duchenne muscular dystrophy without fatigue. Previous studies showed that functional worsening in SMA is absent over 12 months and subtle over longer periods. Conversely, fatigue increased significantly over 12 months. These findings suggest independent mechanisms underlying weakness and fatigue in SMA.

Session Information: N1-1.008, Integrated Neuroscience Session: Pediatric Neuromuscular Diseases, Poster Rounds

Presentation Time: Sunday, March 17, 10:30 am:30 am

Session Information: P03.049, Anterior Horn: Spinal Muscular Atrophy and Other Motor Neuropathies

Presentation Time: Tuesday, March 19, 2:00 pm:30 pm

Case Series: Eosinophilic Vasculitis Isolated to the Central Nervous System

Authors: Brenton Wright, Linda Lewis, James Noble, Jean Paul Vonsattel, Alexander Khandji, R. Sommerville, Clinton Wright

Background/Significance: Eosinophilic vasculitis has been described as part of the Churg-Strauss syndrome, but affects the CNS in only 6 to 7 percent of cases. Presentation in an isolated CNS distribution is rare, with only one case previously reported in the literature. No etiology for the vasculitic process has yet been identified. Here, the researchers present a new case of isolated CNS eosinophilic vascul
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Contact: Karin Eskenazi
ket2116@columbia.edu
212-342-0508
Columbia University Medical Center
Source:Eurekalert

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