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排序方式: 共有10000条查询结果,搜索用时 46 毫秒
981.
Robert C. Griggs MD Barbara E. Herr MS Allen Reha PhD Gary Elfring Leone Atkinson MD Valerie Cwik MD Elaine Mccoll PhD Rabi Tawil MD Shree Pandya RPT MS Michael P. McDermott PhD Kate Bushby MD 《Muscle & nerve》2013,48(1):27-31
Introduction: In 2004, a Cochrane Review and AAN practice parameter concluded that prednisone 0.75 mg/kg/day is of short‐term efficacy in Duchenne muscular dystrophy (DMD). Subsequent efforts to standardize care for DMD indicated wide variation in corticosteroid use. Methods: We surveyed physicians who follow patients with DMD, including: (1) clinics in the TREAT‐NMD (Translational Research in Europe—Assessment and Treatment of Neuromuscular Diseases) network (predominantly Europe) and (2) U.S. MDA clinic directors. We also documented the co‐administered corticosteroids in a trial of a putative treatment (ataluren) for DMD. Results: Of 105 Treat‐NMD clinicians, corticosteroids were not used in 10 clinics, and 29 different regimens were used—the most frequent 0.75 mg/kg/day prednisone (61 centers); 10 days on/10 days off (36 centers); 0.9 mg/kg/day deflazacort (32 centers); and 5 mg/kg/day on weekends (10 centers). Similar diversity was identified in MDA clinics and in the ataluren trial. Conclusions: Variability in corticosteroid use suggests uncertainty about risks/benefits of corticosteroid regimens for DMD. Muscle Nerve, 2013 相似文献
982.
983.
984.
985.
Michael S. Cartwright MD Samantha Demar BS Leah P. Griffin MS Nikhil Balakrishnan MD Jessica M. Harris AAS Francis O. Walker MD 《Muscle & nerve》2013,47(4):515-521
Introduction: Nerve and muscle ultrasound has been studied in several conditions, but validity and reliability have not been assessed systematically. Methods: Nerve cross‐sectional area and muscle thickness were measured ultrasonographically at several sites in 4 cadavers, which were then dissected, and actual measurements were obtained. To assess intrarater and interrater reliability, between 3 and 5 ultrasonographers, with varying experience levels, made repeated measurements on healthy volunteers. Results: Correlation coefficients for nerve and muscle validity were >0.968 (P < 0.001), and for intrarater reliability were >0.901 (P < 0.001) for still and real‐time images. Correlation coefficients for interrater reliability were more varied, but for still images they were all significant at the P < 0.001 (0.542–0.998) level, and for real‐time images they were significant at the P < 0.05 level for half the sites (0.243–0.981). Conclusion: Overall, nerve and muscle ultrasound is a valid and reliable diagnostic imaging technique. Muscle Nerve, 2013 相似文献
986.
Anna‐Karin Persson PhD Shujun Liu MS Catharina G. Faber MD PhD Ingemar S. J. Merkies MD PhD Joel A. Black PhD Stephen G. Waxman MD PhD 《Annals of neurology》2013,73(1):140-145
Small‐fiber neuropathy (SFN) is characterized by injury to small‐diameter peripheral nerve axons and intraepidermal nerve fibers (IENF). Although mechanisms underlying loss of IENF in SFN are poorly understood, available data suggest that it results from axonal degeneration and reduced regenerative capacity. Gain‐of‐function variants in sodium channel NaV1.7 that increase firing frequency and spontaneous firing of dorsal root ganglion (DRG) neurons have recently been identified in ~30% of patients with idiopathic SFN. In the present study, to determine whether these channel variants can impair axonal integrity, we developed an in vitro assay of DRG neurite length, and examined the effect of 3 SFN‐associated variant NaV1.7 channels, I228M, M932L/V991L (ML/VL), and I720K, on DRG neurites in vitro. At 3 days after culturing, DRG neurons transfected with I228M channels exhibited ~20% reduced neurite length compared to wild‐type channels; DRG neurons transfected with ML/VL and I720K variants displayed a trend toward reduced neurite length. I228M‐induced reduction in neurite length was ameliorated by the use‐dependent sodium channel blocker carbamazepine and by a blocker of reverse Na‐Ca exchange. These in vitro observations provide evidence supporting a contribution of the I228M variant NaV1.7 channel to impaired regeneration and/or degeneration of sensory axons in idiopathic SFN, and suggest that enhanced sodium channel activity and reverse Na‐Ca exchange can contribute to a decrease in length of peripheral sensory axons. Ann Neurol 2012 相似文献
987.
Peter J. Darlington PhD Tarik Touil PhD Jean‐Sebastien Doucet BSc Denis Gaucher PhD Joumana Zeidan PhD Dominique Gauchat PhD Rachel Corsini MSc Ho Jin Kim MD Martin Duddy MD Farzaneh Jalili BSc Nathalie Arbour PhD Hania Kebir PhD Jacqueline Chen PhD Douglas L. Arnold MD Marjorie Bowman BScN Jack Antel MD Alexandre Prat MD PhD Mark S. Freedman MD Harold Atkins MD Rafick Sekaly PhD Remi Cheynier PhD Amit Bar‐Or MD for the Canadian MS/BMT Study Group 《Annals of neurology》2013,73(3):341-354
988.
989.
Masayuki Nakamori MD PhD Krzysztof Sobczak PhD Araya Puwanant MD Steve Welle PhD Katy Eichinger DPT Shree Pandya DPT Jeannne Dekdebrun MS Chad R. Heatwole MD Michael P. McDermott PhD Tian Chen MA Melissa Cline PhD Rabi Tawil MD Robert J. Osborne PhD Thurman M. Wheeler MD Maurice S. Swanson PhD Richard T. Moxley III MD Charles A. Thornton MD 《Annals of neurology》2013,74(6):862-872
990.