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Standard and modified statistical mune evaluations in spinal‐bulbar muscular atrophy
Author(s) -
Lehky Tanya J.,
Chen Cheun Ju,
di Prospero Nicholas A.,
Rhodes Lindsay E.,
Fischbeck Kenneth,
Floeter Mary Kay
Publication year - 2009
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.21399
Subject(s) - amyotrophic lateral sclerosis , spinal and bulbar muscular atrophy , medicine , spinal muscular atrophy , atrophy , motor unit , disease , anatomy , androgen receptor , prostate cancer , cancer
Motor unit number estimation (MUNE), a technique used in amyotrophic lateral sclerosis (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 Shefner et al.6 6 to modify the statistical MUNE method to exclude SMUPs ≤ 40 μV. It is unknown if there is similar SMUP instability in the more chronic degenerative disease of SBMA. In this study the standard parameter of excluding SMUP < 10 μV was compared with the exclusion of SMUP < 40 μV in the calculation of the statistical MUNE. The mean statistical MUNE, using the standard method and the Shefner et al. method, was 60 ± 21 to 47 ± 23, respectively. Similar to ALS, SBMA showed an increased proportion (17%) of individual SMUPs ≤ 40 μV compared to normal controls. In conclusion, excluding SMUPs ≤ 40 μV from the statistical MUNE calculations is appropriate for SBMA subjects because their SMUP, characteristics are similar to ALS. Exclusion of the low‐amplitude SMUPs reduces the calculated MUNE. Muscle Nerve, 2009