MUNE评估ALS.pdfVIP

NIH Public Access Author Manuscript Muscle Nerve . Author manuscript; available in PMC 2010 November 1. N Published in final edited form as: I H Muscle Nerve. 2009 November ; 40(5): 809–814. doi:10.1002/mus.21399. - P A A u Standard and Modified Statistical MUNE Evaluations in Spinal- t h o Bulbar Muscular Atrophy r M a 1 2 2 n Tanya. J. Lehky, M.D. , CheunJu. Chen, M.D. , Nicholas A. Di Prospero, M.D., PhD. , u 2 2 1 s Lindsay E. Rhodes, B.S. , Kenneth Fischbeck, M.D. , and Mary Kay Floeter, M.D., Ph.D. c 1 r EMG Section, NINDS, NIH, Bethesda, MD i p t 2 Neurogenetics Branch, NINDS, NIH, Bethesda, MD Abstract MUNE, a technique used in ALS clinical trials to quantitatively assess motor neuron loss, should also be valuable in assessing progression in spinal bulbar muscular atrophy (SBMA), an x-linked neuronopathy. In ALS, instability of single motor units (SMUP) prompted Shefner9 to modify the N I statistical MUNE method to exclude SMUPs ≤ 40μV. It is unknown if there is a similar SMUP H - instability in the more chronic degenerative disease of SBMA. P A In this study, the standard parameter of excluding SMUP 10 μV was compared with the A u exclusion of SMUP 40 μV in the calculation of the statistical MUNE. The mean statistical t h MUNE, using the standard method and the Shefner method, was 60±21 to 47±23, respectively. o r Similar to ALS, SBMA s