Department of Neurology, Kawasaki Medical School, Okayama, Japan.
Eur J Neurol. 2009 Jan;16(1):76-80. doi: 10.1111/j.1468-1331.2008.02349.x.
We report decremental responses to repetitive nerve stimulation (RNS) in 11 patients diagnosed with X-linked spinobulbar muscular atrophy (X-SBMA).
The compound muscle action potential (CMAP) of the right abductor digiti minimi (ADM) and trapezius (TZ) in response to a 3-Hz stimulation of the ulnar nerve at the wrist and accessory nerve at the neck were recorded by surface electrodes.
A decremental response to RNS was observed in 90.9% of the TZ muscle and 27.2% in the ADM muscle of patients with X-SBMA.
These electrophysiological features of X-SBMA are considered to be useful for diagnosis of X-SBMA. Furthermore, the waning phenomena that mostly appeared in the TZ muscle and increment of CMAP in RNS after the exercise also suggest a unique manifestation in X-SBMA.
我们报告了 11 例诊断为 X 连锁脊髓延髓肌萎缩症（X-SBMA）患者对重复神经刺激（RNS）的递减反应。
通过表面电极记录右侧小指展肌（ADM）和斜方肌（TZ）对腕部尺神经和颈部副神经 3Hz 刺激的复合肌肉动作电位（CMAP）。
X-SBMA 患者的 TZ 肌肉中 90.9%和 ADM 肌肉中 27.2%观察到 RNS 的递减反应。
X-SBMA 的这些电生理特征被认为有助于 X-SBMA 的诊断。此外，在 TZ 肌肉中主要出现的衰减现象以及运动后 RNS 中 CMAP 的增加也提示 X-SBMA 具有独特的表现。
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