The profile of motor unit number estimation (MUNE) in spinal and bulbar muscular atrophy
The profile of motor unit number estimation (MUNE) in spinal and bulbar muscular atrophy
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DOI：
10.1136/jnnp.2009.190462
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发表时间：
2009-12
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通讯作者：
Keisuke Suzuki;M. Katsuno;H. Banno;Y. Takeuchi;Motoshi Kawashima;Noriaki Suga;A. Hashizume;T. Hama;K. Uchida;Fumitada Yamashita;Tomohiko Nakamura;M. Hirayama;F. Tanaka;G. Sobue
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作者：
Keisuke Suzuki;M. Katsuno;H. Banno;Y. Takeuchi;Motoshi Kawashima;Noriaki Suga;A. Hashizume;T. Hama;K. Uchida;Fumitada Yamashita;Tomohiko Nakamura;M. Hirayama;F. Tanaka;G. Sobue
目的 脊髓和延髓性肌萎缩症 (SBMA) 是一种下运动神经元疾病，由雄激素受体 (AR) 基因中三核苷酸 CAG 重复序列的扩增引起。这种疾病的基本组织病理学发现是脊髓和脑干中下运动神经元的广泛损失。然而，临床上很难评估运动神经元变性的程度，这强调需要生物标志物来检测剩余的神经元功能。方法 作者对 52 名 SBMA 患者进行了运动单位数估计 (MUNE)，以研究该方法是否可以作为 SBMA 的潜在生物标志物，并在一年后在患者亚组中重新评估 MUNE。结果 与对照组相比，SBMA 患者的功能运动单位数量显着减少，并且与同侧握力和疾病持续时间相关。纵向分析表明，运动单位在一年内进一步减少。结论 结果提示MUNE是反映SBMA患者运动神经元变性严重程度和进展的电生理参数。
Objective Spinal and bulbar muscular atrophy (SBMA) is a lower motor neuron disease caused by the expansion of a trinucleotide CAG repeat in the androgen receptor (AR) gene. The fundamental histopathological finding of this disease is an extensive loss of lower motor neurons in the spinal cord and brainstem. It is, however, difficult to evaluate clinically the degree of motor neuron degeneration, which stresses the need for biomarkers to detect the remaining neuronal function. Methods The authors performed motor unit number estimation (MUNE) in 52 patients with SBMA, to investigate whether this method could be a potential biomarker of SBMA, and re-evaluated MUNE 1 year later in a subgroup of the patients. Results The number of functioning motor units was remarkably reduced in patients with SBMA compared with controls, and was correlated with both ipsilateral grip power and disease duration. A longitudinal analysis demonstrated a further reduction in motor units within 1 year. Conclusions The results suggest that MUNE is an electrophysiological parameter that reflects the severity and progression of motor neuron degeneration in patients with SBMA.